Rheumatology Flashcards
(215 cards)
1
Q
Patient complains of pain on abduction of shoulder, mainly anterior tenderness over the bicipital groove.
A
Bicipital Tendinopathy
Tx NSAIDs —> PT —> Steroid injection (close to the tendon in the Bicipital groove, NOT into the tendon)
2
Q
Patient complains of pain in the shoulder. He started playing tennis after a long time. New shoulder pain while trying to comb hair or raising pants or lifting weights above head, washing the back while showering, or pain in the lateral shoulder upon laying down especially at night time.
A
Rotator cuff tendinopathy
3
Q
A 75-year-old man falls from a height with an outstretched hand. Patient complains of shoulder pain and on exam he can shrug shoulder, cannot abduct his arm and cannot keep that arm up after passive intervention to 90° (drop arm test). X-ray reveals no fracture, only mild narrowing of the subacromial space. Next step after x-ray:
A
Rotator cuff tear
Do MRI next
4
Q
Patient complains of pain in the shoulder, increased on abduction, extremes of movement painless. Pain more on active than passive abduction. Swinging arm back and forth without pain.
A
Subacromial bursitis
Tx Steroids into bursa
5
Q
Patient with pain and grinding or popping sensation in the anterior shoulder while reaching to put seatbelt on. Pain on abduction and beyond 120°.
A
Acromioclavicular joint arthritis
6
Q
A 66-year-old woman presents with gradual onset and progressive right shoulder pain for the past year. Difficulty combing her hair and washing face and head while showering. She had a minor motor vehicle accident a couple of years ago. Exam reveals difficulty in abduction and external rotation of the right shoulder with crepitus and tenderness over the shoulder. X-ray shows a narrowing of the glenohumeral joint space.
A
Glenohumeral osteoarthritis
Tx NSAIDs and stretching exercises. Persistent symptoms —> intra-articular steroids x2 —> No response over 6-12 months period —> surgery
7
Q
A 72-year-old woman with right shoulder pain for the past year with gradual onset of symptoms with movement of the shoulder and at night time. Exam reveals difficulty abducting shoulder. X-ray shows calcification of the ligaments with some effusion which on tap reveals RBCs, WBC’s 2000. Alizarin stain shows basic calcium phosphate crystals and occasional hydroxyapatite crystals.
A
Milwaukee shoulder aka calcium phosphate shoulder disease
Tx NSAIDs and repeated arthrocentesis
Persistent symptoms —> intra-articular steroids —> Degenerative changes —> arthroplasty
8
Q
A 60 year old woman had a cast for her arm injury. Post removal of cast complains of stiffness, inability to move her shoulder. Exam reveals pain and tenderness around shoulder, loss of both active and passive range of movement. X-ray of shoulder appears normal. Injecting steroid into the shoulder joint with resistance.
A
Adhesive capsulitis
Tx early mobilization
9
Q
Patient presents with a complaint of pain in the right shoulder, arm and neck. Exam shows pain in the shoulder and arm area when extending the neck, looking to the affected side and applying pressure downwards on the head (Spurling test positive).
A
Cervical impingement syndrome
10
Q
Patient with right shoulder pain. On forward flexing the right arm and internal rotation with pain.
A
Shoulder impingement syndrome involving supraspinatus tendon.
11
Q
Pain upon lying on the side: (name 3 conditions)
A
1) Shoulder —> rotator cuff tendinopathy
2) Left precordial —> costochondritis
3) Lateral hip pain —> greater trochanteric pain syndrome
12
Q
A student presents with pain and swelling of elbow nearing his exams (or a carpet layer, roofer, etc). Can pronate and supinate arm, but can’t flex.
A
Olecranon bursitis
Etiology: trauma, gout, sepsis
Tx: NSAIDs, local steroids
13
Q
A student presents with pain and swelling of elbow nearing his exams (or a carpet layer, roofer, etc). Can pronate and supinate arm, but can’t flex.
If above patient presents with fever and chills. Exam reveals increased warmth with tenderness. Range of movements painless. Tap reveals 9000 WBCs (could be <20,000).
A
Septic olecranon bursitis
Tx Aspiration, drainage, IV antibiotics + NSAIDs
14
Q
Patient presents with pain in lateral elbow and anterior to the lateral epicondyle. Pain on extension of wrist and fingers and supination of the forearm. While at the airport, he lifted a suitcase (or during handshake) and pain came back again.
A
Lateral epicondylitis aka tennis elbow
Mainly due to lifting heavy objects.
Extensor carpi radialis brevis most commonly affected.
15
Q
Patient presents with pain in lateral elbow and anterior to the lateral epicondyle. Pain on extension of wrist and fingers and supination of the forearm. While at the airport, he lifted a suitcase (or during handshake) and pain came back again.
The best way to reduce recurrence in the future is:
A
Six weeks of physical therapy with eccentric exercise.
NOT steroid injection
16
Q
Carpal tunnel syndrome
A
Weakness in abductor pollicis brevis (most commonly affected).
Phalen’s sign can be +. Tinel’s sign less sensitive than Phalen’s sign.
Management: Do not use NSAIDs.
Next step —> use neutral splint at night.
If no response to above Tx or there is thenar atrophy —> nerve conduction studies —> surgical release
17
Q
In a patient with median nerve involvement, you will most likely see:
A
Inability to oppose little finger with thumb.
Not inability to flex wrist.
18
Q
A 55-year-old woman with numbness in both thumbs and index fingers upon holding anything for a few minutes. Heart rate 52. Fatigue positive. Most appropriate next diagnostic step?
A
TSH
Bilateral carpal tunnel syndrome
19
Q
Pregnant woman complains of pain and paresthesias in both hands in the thumb and index finger, especially at night. What to do?
A
Neutral splinting of wrists
20
Q
A 45-year-old with long-standing rheumatoid arthritis with bilateral tingling sensations in both hands, worsened during night. Thenar muscle wasting present. What is the next best diagnostic step?
A
Nerve conduction studies —> surgery next
Carpal tunnel syndrome
21
Q
What is the best treatment for a patient who fails splinting and has thenar atrophy?
A
Surgical release
Carpal tunnel syndrome
22
Q
Numbness of thumb, index finger and middle finger. Early morning stiffness for an hour. Difficulty opening bottles. Dx?
A
Rheumatoid arthritis
Carpal tunnel syndrome
23
Q
Long distance cyclist presents with tingling and numbness in the little and fourth finger, and ulnar aspect of palm. On exam abduction and adduction of interossei is decreased. Can’t hold paper between little finger and ring finger. Upon holding paper between thumb and index finger, positive flexion and weakness at IP joint of thumb forming a pinch. Severe cases with claw hand.
A
Ulnar nerve entrapment at forearm/wrist
24
Q
Patient presents with pain on radial (lateral) aspect of wrist especially when she lifts her children. Or a young man who plays video games. Point tenderness over radial styloid process. Pain on resisted abduction and extension of thumb. Making a fist with fully flexed thumb and ulnar deviation is painful.
A
DeQuervain’s tenosynovitis —> abductor pollicis longus and extensor pollicis brevis
Tx Rest the tendon (no gripping or grasping), splinting —> local steroids
If disability is severe —> surgery
25
Patient presents with wrist drop. Decreased sensation on the radial and dorsal aspect of his hand.
Compression of radial nerve at spiral groove in the middle of the upper arm a.k.a. Saturday night palsy
26
Patient presents with finger stuck at flexion at PIP. It is straightened with effort of the other hand. Tenderness at the base of finger.
Digital tenosynovitis stenosans (swelling of flexor tendon a.k.a. trigger finger)
Tx activity modification and splinting —> steroids next
27
Patient with stiffness of ulnar aspect of hand. Unable to extend third and fourth fingers. Thickening and contraction of palmar fascia. (DM and EtOH liver disease).
Dupuytren’s contracture
| Tx collagenase injections —> surgery next
28
Patient fell on outstretched hand. Tenderness over anatomical snuffbox. X-ray report negative for fracture. What do you do next?
Thumb spica splint and then bone scan or MRI
29
Diabetic patient with inability to completely extend fingers, can bring both hands together with tips of fingers and wrists of both hands, but can’t bring both MCPs together (i.e. can’t extend fingers). No erythema or swelling.
Diabetic cheiropathy due to collagen deposition
30
Meralgia paresthetica
Diabetic patient with burning sensation or numbness in anterior and lateral thigh.
Pain worsens on abduction of thigh and with exercise. Palpation of right lower quadrant in the inguinal region elicits pain in the thigh.
Etiology: Compression of cutaneous femoral nerve.
Tx weight loss/anticonvulsants/local steroids
31
Patient complains of hip pain. X-ray reveals fracture of ramus of pubis. What to do?
Early ambulation and PT
32
Patient presents with pain in the patellar region. Exam shows no skin breakdown. Erythema and tenderness over the patellar region.
Prepatellar bursitis aka housemaid’s knee
| Tx NSAIDs/local steroids
33
Diabetic patient with pelvic girdle and thigh pain. No burning sensation. Exam reveals atrophy and weakness of thigh muscles.
Diabetic amyotrophy
Treat with tighter glucose control (like you would for all other diabetic microvascular complications, aim for A1c <6.5; for microvascular disease you do LDL control)
34
Elderly patient presents with pain in the knee, mainly on the medial aspect about 2 inches below joint line, which gets worse on climbing stairs (semiflexion). X-rays show no linear calcification, mild osteoarthritic changes.
Pes anserine pain syndrome (sartorius bursa)
| Tx Weight loss, quadriceps strengthening —> NDSAIDs —> local steroids
35
A patient presents with a complaint of pain in the side of the knee while jogging or cycling. The pain radiates upwards towards the thigh. Exam reveals focal tenderness in the lateral aspect of the knee joint just above the midline while abducting and extending the hip. A snap is heard on flexion. On resisted internal rotation of tibia there is no pain.
Iliotibial band syndrome
| Tx Correct training errors, proper footwear, stretching the hip abductors
36
A 28 year old long distance runner complains of pain in the knee. The pain is described as a burning sensation on the inner and outer aspect of the patella and also behind the patella exacerbated by physical activity (running, climbing stairs, doing squats, ascending/descending stairs or hills). He also has knee stiffness especially after sitting for a long time (the “moviegoer” sign). What will help establish the diagnosis?
Patellar compression
Dx chondromalacia patella (patellofemoral syndrome)
Tx Decrease running/quadriceps strengthening/analgesics. Surgical procedure NOT required.
37
Teenager presents with anterior knee pain just below the knee joint. Exam reveals point tenderness just below the knee joint especially on extending knee against resistance.
Osgood-Schlatter disease
38
A 20-year-old presents to ER with acute pain in the knee with swelling. He was playing football about an hour ago and heard a popping sound, followed by pain and swelling. Anterior Drawer sign and Lachman sign positive.
ACL tear
39
A patient presents several hours after injury with swelling. Knee locks or gives away. During injury heard a popping sound. 24 hours later, with pain and swelling. Palpation of the medial joint line with pain and a clicking sound is heard on flexion of the knee with the ankle in external rotation. McMurray’s test positive.
Meniscal tear
40
Patient presents with pain in the knee on the medial aspect after an injury several hours ago. No history of popping sound. He can ambulate but cannot pivot or twist. Exam shows Drawers test negative, Lachman’s test negative, Varus test negative, Valgus test positive with pain along the medial joint.
Medial collateral ligament injury
41
A patient presents with pain and swelling of posterior leg. The pain began abruptly >24 hours ago after he played a game of tennis for a long time. Exam shows ecchymosis, swelling and tenderness in the mid calf region. No history of rheumatoid arthritis. What to do next?
Ultrasound
Dx gastrocnemius tear
Tx Rest/NSAIDs/crepe bandage
42
Patient presents with pain in the ankle on ambulation. He twisted his ankle while stepping on an uneven surface. Can walk 4 steps without support. Compression of the posterior malleolus (medial and lateral) with NO pain.
```
Ankle sprain (anterior talofibular ligament)
No need for x-ray
Tx NSAIDs, splint
```
43
A 35-year-old woman preparing for marathon for the past two months presents with pain in her leg for the past four weeks. It has gradually increased over time. Exam reveals focal tenderness over the tibia.
Stress fracture
Check x-ray and if negative MRI next
Tx with rest
44
A long distance runner complains of pain in the lower medial aspect of leg. He has noticed the pain over the past two days. Pain is worsened when he jogs. Exam reveals a diffuse tenderness of the medial aspect of his leg. X-ray negative for fracture.
Medial tibial stress syndrome a.k.a. Shin splints
| Tx ice packs, orthotic soles
45
Patient presents with pain and burning sensation in between third and fourth toes. It gets worse while walking with high heels. Also complains of pain while walking on a hard surface. The pain radiates to the front of the toes along with paresthesias along the plantar aspect. The pain gets better when the shoes are removed.
Morton’s neuroma (interdigital plantar neuroma)
46
Patient presents with pain in the heel, stiffness during early morning stride. It’s better as the day goes along.
Plantar fasciitis
Tx ice packs after activity/arch support/NSAIDs
Correct training errors/steroid injections/surgery
Stretching exercise with dorsiflexion of foot
47
Patient presents with pain in the heel, stiffness during early morning stride. It’s better as the day goes along.
Above patient’s x-ray shows a heel spur. Because of the pain is:
Plantar fasciitis, not the calcaneal spur
48
Foot pain, more in the morning, difficulty with dorsiflexion. On exam tenderness at the base of the calcaneus, with increased tenderness on squeezing the heel.
Plantar fasciitis, not calcaneal fracture
49
Patient with numbness and burning sensation in the toes. Gets worse on walking and at the end of the day. The symptoms are aggravated at night time. Radiates to the front of the toes. Taking off shoes makes it feel better. On percussion, posterior and inferior to medial malleolus produces pain.
Tarsal tunnel syndrome (tibial nerve compression)
| Tx arch support (shoe modification), NSAIDs, surgical decompression for severe cases
50
A 20-year-old man presents with complaints of difficulty walking and running, has a history of falls for the past several months. Exam reveals atrophy of the calf muscles bilaterally and hammer toe with a high plantar arch of the foot.
Charcot-Marie-Tooth disease
51
Patient presents with complaints of painful feet, ankles and knees after prolonged standing, which has happened for the past couple of years. Exam reveals normal feet. Upon standing, the arches of feet collapse and there is valgus of heel.
```
Pes planus (flat feet)
Tx orthotics shoes
```
52
A 45-year-old woman presents with extreme fatigue, doesn’t want to get out of bed. Also complains of diffuse muscle aches. Exam reveals tenderness in most muscle groups. Non-restorative sleep. No swelling of joints or erythema. No fever. ANA 1:64, ESR 30, CPK 98.
Fibromyalgia
Start amitriptyline or tai chi or aerobic exercise
Does not need anti-DNA/Sm antibody testing
53
An elderly man presents with generalized bodyaches and fatigue. It started a couple of days ago with pain in the upper arms and neck. It is associated with morning stiffness. Exam reveals no focal deficit. X-rays show minimal osteoarthritic changes. ESR 52. AlkPhos elevated.
Polymyalgia rheumatica
| Tx dramatic response to low dose prednisone
54
Patient with episodic swelling of ears. Exam with cartilaginous portion of ear swollen. How to establish diagnosis?
Relapsing polychondritis
Biopsy of cartilage of ear
Tx immunosuppressive
Complications —> Laryngeal involvement with inflammation, aortic inflammation and regurgitation
55
Conditions involving DIP, PIP, MCP, and 1st CMP
DIP: Psoriasis, OA (Heberden’s)
PIP OA (Bouchard’s), RA, Scleroderma
MCP: Hemochromatosis (wrist too), RA, Scleroderma (tendon friction rub)
1st CMP: OA
Reactive arthritis can be DIP+PIP+MCP
56
Rheumatoid Arthritis diagnostic criteria
Diagnosis with 6 or more points:
```
*Joint involvement:
2-10 large joints (1 point)
1-3 small joints (2 points)
4-10 small joints (3 points)
>10 joints (5 points)
```
```
Serological abnormality (RF or CCP):
Low positive (2 points)
High positive (3 points)
```
Other:
ESR or CRP elevation (1 point)
Duration >6wks (1 point)
X-ray changes (marginal bony erosions, periarticular osteopenia; zero points)
57
Rheumatoid arthritis poor prognostic factors
Progressive synovitis
Joint space loss/erosions
Increased RF
Increased ESR
58
70 year old woman with early morning stiffness >45 minutes and symmetrical small joint pain in her second, third, fourth MCP in right hand and second, third, fourth and fifth MCP and PIP in left hand. Right knee joint over the past six weeks. ESR 48. Exam reveals swelling of MCPs and PIPs of both hands. RF is negative. What to do?
RA score is >6 so meets criteria:
Total joints = 3+4+4 = >10 joints => 5 points
ESR elevated => 1 point
Duration >6wks => 1 point
Start treatment
Consider checking anti-CCP
59
Articular and musculoskeletal RA manifestations
Atlanta-odontoid subluxation (often requires chronic RA >30yrs)
—> compress vertebrobasilar artery —>lightheaded ness, syncope —> check lateral neck x-ray; if present, Anesthesia does retrograde intubation
—> compress cord —> sensory/motor symptoms/quadriplegia
Carpal tunnel and tarsal tunnel syndromes common too
60
Extra-articular RA manifestations
Spill over of active disease from synovium
```
Skin:
RA nodules (usually a/w increased RF, but RF neg in 15%)
Vasculitis/DR4 (eg- necrosis of fingertips, ulcers on shins; DDx polyarthritis nodosa, Hep C with cryoglobulinemia)
```
Heart:
CAD, AFib, pericardial effusion, constrictive pericarditis, myocarditis
```
Lung:
RA nodules (Caplan’s syndrome), pleural effusion (low glucose, high LDH, exudative), BO, interstitial fibrosis, hypersensitivity pneumonia is (BAL with increased Lymph’s; type III/IV so Eos neg and IgE wnl; seen with methotrexate usually within 3mos of use, nitrofurantoin, sirolimus)
```
Blood:
Anemia of inflammation, DVT/PE
Vasculitis:
Necrosis, ulceration at tips of fingers
Nerve:
Mononeuritis multiplex —> foot drop/hand drop
Renal:
Usually late-stage disease with amyloid neuropathy, drug induced (NSAIDs, penicillin INR —> nephrotic syndrome)
Eyes:
Scleritis (painful, photophobia, diffuse redness of sclera all layers, can cause globe rupture; Tx steroids and Tx underlying Dz more aggressively)
Episcleritis (eye discomfort, scattered superficial redness; Tx is self-limited or symptomatic)
61
Rheumatoid arthritis treatment in Mild-Moderate disease
First: MTX +/- NSAID +/- hydroxychloroquine/sulfasalazine
Then: +/- low-dose steroids +/- Leflunomide (pyrimidine inhibitor, takes up to 1yr to come out of body so Tx cholestyramine x11 days to wash out all metabolites sooner such as if pt just got pregnant)
Increase methotrexate to >/= 25mg/wk or goal methotrexate polyglutamate level >/= 60 (the active metabolite)
If persistent synovitis at 8-12wks despite good level then treat as “Severe”
62
Rheumatoid arthritis treatment in severe/refractory disease
Add-on TNF alpha inhibitors:
Make sure PPD neg (>/=5mm is positive if about to give these meds); don’t give with active viral infections (eg- LRTI, bronchitis). These all increase infection risk (including histoplasma, cocci reactivation) so make sure pneumonia vaccines given, Hep B/C negative (else need concurrent treatment with tenofovir, plus continued treatment for 1yr after off these meds)
Etanercep, infliximab, adalimumab, or anakinra
Or golimumab or certolizumab
If still not better then: abatacept, rituximab, tocilizumab, or tofacinib
63
Tocilizumab side effects
```
Works as an IL-6 inhibitor
Diverticular rupture (LLQ pain, fever), OCP failure, lymphopenia, hepatitis B/C reactivation, pneumonia, PMLE (progressive multifocal leukoencephalopathy, also seen with JC virus, HIV; white matter lesions)
```
64
The fastest acting DMARD for rheumatoid arthritis?
Methotrexate
65
The only med shown to decrease mortality in RA?
MTX
66
You diagnosed patient with rheumatoid arthritis and started methotrexate with hydroxychloroquine and low-dose steroids. What else will you start in this patient?
Folic acid; NOT the same thing as folinic acid
| Found to reduce the mucosal, hematologic, hepatic, and gastrointestinal side effects of methotrexate
67
NSAID side effects
Nephrotic syndrome, peptic ulcer disease, interstitial nephritis
68
Hydroxychloroquine side effects
Macular damage (after 5yrs of use), loss of accommodation (aka presbyopia)
69
Methotrexate side effects
Hepatotoxicity, hypersensitivity pneumonitis, aphthous ulcers
70
Leflunomide side effects
Teratogenic
71
Corticosteroid side effects
Osteoporosis, hypertension, cataracts, avascular necrosis
72
Infliximab side effects
Reactivation of tuberculosis, demyelinating disease, fungal infection, psoriasis, drug-induced lupus
73
Etanercept side effects
Reactivation of tuberculosis, demyelinating disease, fungal infection, psoriasis, drug-induced lupus.
PLUS:
ANA positivity, anti-DNA positivity (not anti-histone like other drug-induced lupus), flu-like symptoms
74
Cyclosporine side effects
ATN, renal insufficiency, hirsuitism, HTN
Avoid taking with grapefruit juice (increases levels)
75
Patient with rheumatoid arthritis presents with sudden onset of pain behind the knee and in the calf. Ecchymotic area below the malleolus (crescent sign). Ultrasound is negative for DVT. What to do?
Dx Baker cyst rupture
Tx intra-articular steroids (immediate pain relief)
Not NSAIDs or anticoagulation
76
Patient with long-standing history of rheumatoid arthritis undergoes an elective surgery under general anesthesia. Postoperatively patient is quadriplegic. Most likely ideology is?
Atlanta-odontological subluxation C1-C2
| Not cerebral stroke
77
Patient with long-standing rheumatoid arthritis presents with hoarseness of voice for several weeks. Most likely diagnosis?
Cricoarytenoid joint involvement
78
Hey 65-year-old with rheumatoid arthritis >30 years with persistent paresthesias in both hands. Bilateral carpal tunnel release surgery is done with little change in the paresthesias three months later. He also has occasional dizzy spells. Past history is significant for total knee arthroplasty and severe joint deformities. On exam there is decreased power and hyperactive reflexes. The patient is going for hip replacement surgery, what would you do next?
X-ray neck to look for subluxation
| Not MRI brain or EMG to look for residual CTS
79
A patient with history of rheumatoid arthritis on methotrexate for about a year, presents with a few days history of pain and swelling of the knee joint. Fever with leukocytosis. What to do next?
Tap the joint to rule out septic arthritis
80
A patient with history of rheumatoid arthritis on methotrexate for about a year, presents with a few days history of pain and swelling of the knee joint. Fever with leukocytosis.
Joint tap reveals turbid exudate, labs are pending. What to do?
Start IV antibiotics
Fluid should be thin and not turbid with rheumatoid arthritis/gout/pseudogout
Not intra-articular steroids or MTX
81
Patient with rheumatoid arthritis on hydroxychloroquine. What sort of medication monitoring is required?
Retinal eye exam for baseline now, and then repeat in five years and yearly thereafter
82
Leading cause of death in RA? For Lupus? For HIV?
Heart disease
83
Patient was being treated with steroids for temporal arteritis or polymyalgia rheumatica or SLE. A couple of months later, steroids were being tapered off because of improvement in his symptoms. Now patient returns with bilateral symmetrical joint pain and early morning stiffness involving MCP, PIP. No shoulder ache or headaches. Exam reveals positive nodule on left olecranon area. Most likely diagnosis?
Rheumatoid arthritis
| Not OA or poly myositis
84
Patient on methotrexate for rheumatoid arthritis. What medication monitoring is required?
CBC, creatinine, AST (a marker of liver fibrosis)
| At 2wks then at 4wks and then Q12wks long-term
85
A patient with rheumatoid arthritis with minimal response to methotrexate 25 mg per week. What will you do next?
Place a PPD (not start anti-TNF-alpha agent until after)
| If PPD positive, then CXR. If CXR negative, then INH x1 month and can start TNF agent while finishing INH
86
DMARD precautions
Give pneumococcal and influenza vaccines before biological DMARDs (anti-TNF alpha therapy) and non-biologic DMARDs such as methotrexate or leflunomide
Don’t give live vaccines for biologic DMARDs
Don’t give DMARDS with active infections
87
A 35yo presents with recurrent pain which starts in PIP, then MCP and then in the knee. A few hours later, the joints are swollen. Within 24 hours, the pain resolved starting with knee, MCP and then PIP. In between the attacks the patient is absolutely fine. Diagnosis is?
Palindromic rheumatism
Onset PIP—>MCP—>knee and Resolution in reverse knee—>MCP—>PIP
1/2 will go on to develop rheumatoid arthritis
Tx DMARDs
88
Patient with rheumatoid arthritis with necrotic ulceration of tips of fingers and foot drop. Diagnosis?
Rheumatoid vasculitis
89
Less than 1% of patients with long-standing rheumatoid arthritis present with splenomegaly and leukopenia. Recurrent skin and lung infections. Skin ulcers positive. Diagnosis?
Felty syndrome
Tx DMARDs, steroids, G-CSF and maybe splenectomy
Compare to total opposite with Juvenile onset RA = Still’s Dx = leukocytosis, sore throat, increased ferritin
90
A 32-year-old woman presents with polyarthritis and fever. She had a sore throat several weeks ago. She continues to have recurrent fever which is accompanied by an evanescent salmon colored/faint pink rash on the trunk and upper extremities. Throat culture negative. ESR 110, ferritin 600. WBC 24,000 with 85% PMNs. Patient started on a course of antibiotics without success. AST/ALT increase. RF negative. ANA negative. Most likely diagnosis?
Juvenile idiopathic rheumatoid arthritis aka Still’s disease
| Not sepsis, Felty Sn, or poststreptococcal GN
91
What disease is more likely related to rheumatoid arthritis?
Which virus is most likely associated with rheumatoid arthritis?
Periodontal disease, not lactose intolerance
EBV
92
Sjogren Syndrome
```
RF positive
ANA positive
SSA/SSB positive
Increased ESR
Increased risk of pneumonia and celiac disease
Associated with distal RTA type 1
```
Enlarged salivary glands, dry mouth, dry eyes
Dx:
Shirmer (blotting paper) test positive if <5mm wetting in tearing of eyes in 5min (normal is 15mm in 5min)
Only if antibodies negative —> Do lip biopsy of minor salivary glands
Tx:
Symptomatic = hydration, pilocarpine (stimulates muscarinic cholinergic receptors), steroids
93
Seronegative spondyloarthropathies (RF negative, HLA-B27 positive)
Involve spine
Asymmetric poly/oligoarthritis (<4 joints affected)
Enthesitis (inflammation of ligaments, tendons, joint capsule, etc)
Dactylitis (swelling of entire digit)
Eg- Ankylosing spondylitis, Reactive Arthritis (mucosal inflammation of GI or GU tract)
94
Ankylosing Spondylitis
Starts after resting, gets better with exercise
Symptomatic sacroiliitis
Uveitis: eye pain, photophobia, lacrimation
HLA B27 positive, apical fibrosis
X-ray shows bamboo spine (specific)
Associated with aortitis (check screening TTE)
Diminished chest expansion (can’t take a deep breath, decreased NIP/negative inspiration pressure)
95
Patient who loves to do outdoor work with on and off back pain. He presents with complaints of pain and redness of right eye. Exam reveals he has some loss of forward spinal mobility. Exam of the eye shows injection around the cornea. Fluorescein eye stain is negative. What is most suggestive of ankylosing spondylitis in the above patient?
Loss of spinal mobility (Normal is >/= 5 cm spine stretching with movement = Schober’s test)
Not uveitis or HLA B27 positivity
96
Patient who loves to do outdoor work with on and off back pain. He presents with complaints of pain and redness of right eye. Exam reveals he has some loss of forward spinal mobility. Exam of the eye shows injection around the cornea. Fluorescein eye stain is negative.
What diagnostic test will you do next for this patient?
What is the most sensitive test?
What is the eye manifestation?
X-ray of sacroiliac joint (not iliac spine) next; not HLA-B27 or urine culture
MRI of sacroiliac joint is most sensitive and very specific; not CT scan or MRI of lumbar spine
Uveitis; not conjunctivitis or corneal abrasion
97
Patient presents with low back pain. X-ray shows fusion of sacroiliac joints and ankylosis of the spine (bamboo spine). HLA B27 positive. This is most likely associated with what condition?
Management?
Dx ankylosing spondylitis
a/wAortitis (so check echo next)
Not OA or RA
Tx:
Stiffness —> exercise first —> PT next
Pain —> NSAIDs. If pt comes back in 6wks with minimal improvement in pain, fatigue and morning stiffness —> anti-TNF-alpha (not MTX and not hydroxychloroquine)
The best way to manage ROM in akylosing spondylitis = exercise
Follow disease activity with ESR
98
Uveitis associations
Reiter’s syndrome (Reactive Arthritis)
Behçet’s disease
Ankylosing spondylitis
99
Patient with history of ankylosing spondylitis complains of back pain. What to do?
X-ray (not PT)
| ?aortitis
100
Reiter’s syndrome (Reactive Arthritis)
Conjunctivitis, urethritis, asymmetric arthritis
Mouth ulcers
Keratoderma blenorrhagicum (wrinkly and shiny hand)
Check HIV test next (might be a manifestation)
101
A 35-year-old with left ankle arthritis and left Achilles tendon area pain. She has had redness of the eye, abdominal pain and diarrhea. Exam reveals pain at the base of the calcaneus. Diagnosis? Treatment?
Reactive arthritis; not parvovirus
Recall can be GI or GU inflammation
Tx cipro + NSAID
102
How to treat patient with remote history of diarrhea and now enthesitis?
NSAIDs; not Cipro
103
Patient with reactive arthritis with ongoing arthritic pain for more than six months not relieved with NSAIDs or steroids. What to do?
Sulfasalazine/methotrexate
| Not antibiotics
104
How to treat patient with remote history of urethritis and now enthesitis?
Treat chlamydia (don’t need to check, just treat) and give NSAIDs too
105
Patient presents with lower back pain and pain in the distal inter-phalangeal joints. HLA B27 positive. On exam pitting nail changes present. Diagnosis?
Psoriatic arthritis
Tx:
Mild —> NSAIDs; if skin and nail changes —> MTX
Next —> TNF inhibitors, IL-17/23 inhibitors
NOT hydroxychloroquine (makes psoriatic arthritis worse)
Beta-blockers and infection make psoriasis worse too
106
Patient of Middle Eastern or Japanese descent with recurrent painful aphthous stomatitis, genital aphthous ulcers, joint pain and erythema nodosum on legs. HLA B25 positive. On exam uveitis present (posterior —> blurry vision), pathergy positive (hyperreactive to needlesticks). Dx/Tx?
Behçet’s disease
Tx mucocutaneous diease with colchicine
Moderate to severe disease —> steroids —> azathioprine —> TNF inhibitors
107
A 24 year old man presents with complaints of multiple oral aphthous ulcers and redness of the eye for the past month. Tender nodules on the shins. A chest x-ray reveals a prominent pulmonary artery which is found to be an aneurysm on CT scan. What is the most likely diagnosis?
Behcet’s Disease, not sarcoidosis
108
Oral aphthous ulcer and genital ulcer. Refuses needle sticks because area swells up for a long time afterwards.
Behçet’s disease
109
Painful shin nodules and genital ulcer. Refuses needle sticks.
Behçet’s disease
110
Painful shin nodules and oral aphthous ulcers; aortic aneurysm; painful red eye with blurry vision; occasional knee and ankle pain.
Behçet’s disease
111
Oral aphthous ulcers, history of urethral discharge in the past, red eye with knee and ankle pain.
Reiter’s Syndrome
112
Erythema nodosum and hilar adenopathy.
Sarcoidosis
113
Patient returns from the Caribbean with high fever and pain in small joints of the hand, wrist and ankle, with or without macular papular rash. Most likely diagnosis?
Chikungunya
114
A 25 year old presents with pain and swelling of his right knee. He has diarrhea intermittently for about a week. Exam reveals swelling of entire second toe (sausage digit) and severe pain on palpation of the Achilles tendon. Also a painless ulcer on the tongue. What is the most likely diagnosis?
Reactive arthritis
| Not disseminated gonococcal infection, gout, or ankylosing spondylitis
115
A 25 year old presents with pain and swelling of his right knee. He has diarrhea intermittently for about a week. Exam reveals swelling of entire second toe (sausage digit) and severe pain on palpation of the Achilles tendon. Also a painless ulcer on the tongue.
What is most likely positive?
Stool culture
| Not knee tap, HLA B27, or monosodium urate crystals
116
A 70-year-old diabetic man presents with pain in the mid-back area. There is early morning stiffness of the spine. Exam reveals decreased thoracic lateral flexion (can’t bend sideways). X-ray of the thoracic spine reveals flowing ossification (calcifications) of the anterior longitudinal ligaments. Most likely diagnosis?
Diffuse idiopathic skeletal hyperostosis (DISH)
| Not ankylosing spondylitis
117
Osteoarthritis Clinical Features
Joint pain: 1st CMC, knees, PIP, DIP, hip, cervical and lumbar spine
X-ray with osteophytes, central erosions in DIPs
If no osteophytes, then morning stiffness (few min with OA, >30min with RA), crepitus on movement of joint, joint fluid WBCs <2000, Labs with low-titer RF (<1:40) and ANA (<1:160)
Recall joint deformities:
PIP (Bouchard’s nodes)
DIP (Heberden’s nodes)
118
Osteoarthritis Treatment
Lose weight (esp if BMI >30) —> orthoses (insoles, braces, knee taping, assistive devices)
—> topical agents (hand and knee) —> NSAIDs/non-acetylated salicylates —> celecoxib with PPI —> intra-articular steroids —> replace joint
119
Osteophyte formation
Central bony erosions in PIP and DIP
Subchondral sclerosis
Osteoarthritis
| a/w joint space deformity too
120
Periarticular osteopenia
| Marginal bony erosions in PIP and MCP
Rheumatoid arthritis
| a/w joint space deformity too
121
Patient presents with fatigue and hemoglobin 9. MCV 75. History reveals patient is taking NSAIDs for osteoarthritis of the knee. Stool for occult blood is positive. What to do?
d/c oral NSAIDs and start topical NSAIDs
| Not transfuse PRBC
122
Most important risk factor for osteoarthritis?
Obesity
| Not sedentary lifestyle
123
Elderly patient presents with pain in the thumb while turning keys and opening car doors. Exam reveals pain at the base of the thumb on flexion and internal rotation, crepitus positive. Most likely diagnosis?
Osteoarthritis of 1st CMC joint
| Not DeQuervain’s tenosynovitis
124
Patient with osteoarthritis wants to try glucosamine or chondroitin sulfate. What to do?
No difference from placebo.
125
Patient with long-standing history of osteoarthritis and takes acetaminophen for pain. He plays tennis occasionally. Exam reveals mild crepitus and swelling of his right knee. No fever, WBC 8000. Right thigh smaller than left. What do you do?
Tap knee and send pt for PT to strengthen quadriceps
126
Randomized clinical trials have shown benefit of acupuncture in:
OA of knee and hip
Topical capsaicin also shown to benefit OA of knee and hand
127
Patient is a bricklayer, presents with pain and swelling of both PIP and DIPs. X-rays show loss of cartilage and narrowing of joint space on central erosions. Most likely diagnosis?
Erosive osteoarthritis
| Not erosive RA or psoriatic arthritis
128
A laborer or farmer presents with URI. He has had rheumatoid arthritis for more than 15 years. Exam reveals swollen PIP and MCP with a boggy feeling. Patient does not complain of pain. Strength of hand is normal. X-ray of hand reveals erosions of the PIP and MCP. Most likely diagnosis?
```
Rheumatoid arthritis (arthritis robustus)
Not psoriatic arthritis, OA, gout
```
129
Most likely location of pain in OA of hip?
Groin
130
Right groin pain with RA and osteophytes on x-ray?
Secondary OA
131
Right groin pain with rheumatoid arthritis, x-ray negative, MRI double line sign on T2.
Avascular necrosis (recall occurrence with sickle cell disease too)
132
Pain over lateral aspect of hip. Can’t sit in car, can’t sleep on that side.
Greater trochanteric pain syndrome (a.k.a. Trochanteric bursitis)
Tx: inject local steroid
133
Patient less than 40 years old with bilateral groin pain, more on left. Worse with activity and internal rotation.
Acetabular impingement (by osteophyte)
134
Patient with SLE on steroids for >2 years presents with pain in the hip and walks with a limp. The best diagnostic test?
MRI
| FYI: duration of steroids may be more important than dose for avascular necrosis risk
135
A 45-year-old man complains of pain in the right buttock that shoots down the back of the thigh for the past three days. Tenderness over the right sciatic notch when pressure applied by thumb and pain on abduction while lying down. Most likely diagnosis?
Piriformis syndrome
136
Patient with pain in the groin. X-ray reveals osteophytes. You prescribe analgesia and a cane. What are your directions for the use of the cane?
Use cane on the opposite side of the affected joint
137
A patient with one knee osteoarthritis walks with a cane. What directions when ascending and descending stairs?
Ascend stair with good leg first, followed by affected leg and cane. Descend stair with affected leg first, followed by the cane and good leg.
“Up to heaven and down to hell”
138
Gout vs Pseudogout
Gout —> monosodium urate (negative birefringence) —> bony tophus with erosion (big toe)
Pseudogout —> calcium pyrophosphate dihydrate —> chondrocalcinosis (calcified ligaments on x-ray)
Don’t treat asymptomatic hyperuricemia
Uris acid levels may fall to a normal range in 30% during a gout attack
139
A 50 year old man with past medical history of DM and PUD woke up this morning with severe toe pain. Exam reveals erythema of the big toe with severe tenderness. He had a similar episode two years ago which resolved by itself in a few days Serum urate is 5.5. Pt refuses tap of the affected joint. Next step?
```
Colchicine (not as good if past 12-24hrs from disease onset —> use NSAID instead)
Not indomethacin (Hx PUD) or oral glucocorticoid (Hx DM)
```
140
Patient with history of DM presents with sudden onset of pain in the toe since yesterday. Three days ago he stumbled barefoot on cobblestone. Exam reveals big toe red and exquisite tenderness. What to do?
Dx traumatic gout
Tx NSAIDs
Not steroids, colchicine, or allopurinol
141
Patient with history of CKD presents with sudden onset of pain in the toe since yesterday. Exam reveals big toe red and exquisite tenderness. What do you do?
Steroids
| Other options not great with CKD (NSAIDs, colchicine, allopurinol)
142
Patient with history of CKD, CAD with MI had CABG 2 days ago, develops sudden onset of pain in the toe since this morning. Exam reveals big toe and ankle joint red and exquisite tenderness. What to you?
```
Intra-articular steroids
Not NSAIDs (Hx CAD/CABG), steroids (Hx recent surgery/wound), colchicine (Hx CKD)
```
143
Patient with history of recurrent gout presents with pain in his toe, ankle and knee or one joint with severe disease. What to do?
Systemic steroids + colchicine
Or NSAID + colchicine
NOT NSAIDs + steroids (higher bleed and PUD risk)
And not intra-articular steroids (too many joints)
144
Patient with second gouty attack OR CKD with gout OR tophaceous gout OR goout with bony erosion. What is the best management after acute flare?
Colchicine + allopurinol
Not colchicine + NSAID (Hx CKD)
Not observation
145
Patient with second gouty attack OR CKD with gout OR tophaceous gout OR goout with bony erosion.
Above patient with history of gout attacks in the past on colchicine and allopurinol 100 mg daily prophylaxis, now presents with acute gout attack. What to do?
Continue allopurinol, start NSAID
Not d/c allopurinol and start NSAID
Only 1st attack then don’t give allopurinol, 2nd and after you should though
146
Patient with second gouty attack OR CKD with gout OR tophaceous gout OR goout with bony erosion.
Above young patient with gouty attacks on allopurinol 300 mg daily and NSAID comes back with recurrent attacks. Your gas is level seven. What to do?
Increase allopurinol to 800mg daily
3 weeks later, uric acid level still 7 (normal <6), then —> assess pt adherence to allopurinol
147
Patient with history of hypertension takes ACE inhibitor. Presents with a complaint of generalized rash. Exam reveals fever, necrolytic kind of rash. WBC 15,000 and eosinophils 10%, BUN/creatinine 40/3.2. AST/ALT increased. This is most likely due to?
Allopurinol
Consider checking HLA-B5801 before starting in Asians (esp- Koreans, Han Chinese, Thai) and maybe African Americans
Recall allopurinol increases level of azathioprine
148
Patient with history of gout presents with swelling of the knee joint. A tap reveals negatively birefrigerant crystals and WBC count of 40,000. Patient started on NSAIDs. He is also taking allopurinol. A week later, the patient presents with reaccumulation of fluid in the joint and still with pain. A re-tap again reveals negatively birefringent crystals and WBC of 46,000. The fluid has been yellow, turbid, viscous or thick just like during the previous top. What is the best management?
IV antibiotics
| Not PO steroids or intra-articular steroids
149
Patient with hypertension on chlorthalidone with uric acid elevation.
If asymptomatic what to do?
If has a gouty attack what to do?
Continue chlorthalidone if asymptomatic hyperuricemia
If symptomatic then d/c chlorthalidone (after an attack, not during)
150
Elderly patients especially women on chlorthalidone/HCTZ can present with pain in the PIP and DIP with nodules and swelling distal to the nodules. Dx?
Most likely will have which deposits?
This represents gouty arthritis
```
Monosodium urate (chocolate colored negative birefringent crystals)
Not calcium oxalate (dumbbell shaped crystals, knee tap in CKD, variable birefringence)
```
151
Pseudogout
Calcium pyrophosphate dihydrate disease
Causes: hyperparathyroidism, hemochromatosis
Dx: writs, knee, and shoulder most commonly affected
X-ray: chrondrocalcinosis
Joint fluid: rhomboid crystals, weakly positive birefringence
Tx: similar to gout, less responsive to colchicine (give NSAIDs more often)
152
Patient presents with arthritis, x-rays reveal multiple areas of joint calcification in multiple joints. Also complains of fatigue. FBS 158. Dx? Next test?
Chondrocalcinosis due to calcium pyrophosphate dihydrate deposition (pseudogout)
```
Check TIBC (r/o hemochromatosis; transferrin sats maybe better, or ferritin an option too)
Not ANA or RF
```
153
A patient with hyperparathyroidism undergoes parathyroidectomy. Post surgery the patient develops acute onset of pain and swelling of the right knee. Tap is done. It would reveal:
55,000 WBCs with positive birefringence
| Not bacteria and not negative birefringence
154
Patient post surgery three days later with wrist, MCP, and knee pains. Knee joint swollen. Serum uric acid is seven. What do you expect in fluid?
Calcium pyrophosphate dihydrate crystals (pseudogout despite the elevated uric acid level)
Not monosodium urate crystals
155
Complement levels in vasculitis
Normal with Large and Medium vessel vasculitis
Low with pure Small vessel vasculitis (no medium involvement)
156
Large vessel vasculitis
Temporal arteritis
Takayasu arteritis
Aortitis
157
Medium vessel vasculitis
Polyarteritis nodosa
Granulomatosis with polyangiitis (Wegener’s)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
158
Small vessel vasculitis
Henoch-Schonlein
Microscopic polyangiitis (MPA)
Leukocytoclastic angiitis (hypersensitivity vasculitis, eg- due to penicillins)
EGPA
Goodpasture’s syndrome
Cryoglobulinemia (eg- Hep C; recall decrease C4 more than decrease C3)
SLE (recall decrease C3 more than decrease C4)
RA
Subacute bacterial endocarditis (a/w p-ANCA)
159
A 65-year-old woman complains of frontal headache which is moderately severe and throbbing. Scalp hurts when she combs her hair. She says it hurts while chewing. She had an episode of blurry vision this morning, which made her come to the office. Review of systems includes low-grade fevers over the past month. Exam reveals no focal deficits. Dx? Tx? Eval?
ESR (elevated in 85-90%); not MRI or CT brain, not carotid ultrasound.
Then give high-dose steroids (biopsy isn’t urgent but is next).
If ESR 85 (elevated) and temporal artery biopsy is negative, most likely diagnosis is still temporal arteritis (not migraines or tension headaches).
160
A 65-year-old man with headaches, ESR 85, steroids were started and temporal artery biopsy was negative. Blood pressure in both extremities normal. What to do next?
Do ultrasound-guided biopsy of contralateral temporal artery next (take a bigger piece to eval too).
Not MRA of carotid, aorta, and neck vessels
161
A patient presents with pain and weakness of the left arm after exercise. Recently some dizziness and visual disturbances/TIA as well. BP right arm 140/80 and left arm 155/95.
If pt age 25 also with history of malaise and low grade fevers?
If pt elderly with normal ESR?
If pt elderly with high ESR?
What next?
Takayasu arteritis
Temporal arteritis (normal ESR in 5-15%)
Temporal arteritis
—> steroids —> temporal artery biopsy —> MRA/CTA of chest to r/o aneurysm
162
How to diagnose Takayasu arteritis? Tx?
Aortography (shows stenosis)
Tx steroids, calcium channel blockers
163
Ankylosing spondylitis is associated with?
Aortitis and Uveitis
164
Aortitis can be associated with?
Ankylosing spondylitis
Uveitis
Syphilis
165
Wrist drop and:
| Mesenteric artery aneurysm (pain with meals, proven with mesenteric angiography), increased creatinine, rash
Polyarteritis nodosa
| Tx steroids and cyclophosphamide
166
Wrist drop and:
| Sinus disease, lung infiltrate, lung nodule, increased creatinine
GPA
| Tx steroids and cyclophosphamide
167
Wrist drop and:
| Pulmonary opacity, wheezing
EGPA
| Tx steroids only
168
A 50-year-old man presents to the ER with complaints of abdominal pain which worsens with eating, mainly in the periumbilical area. It gets better when the stomach is empty. The pain has worsened over the past several weeks. He also complains of joint pains in his hands and feet ulcers on the lower extremities. Breath sounds clear. Purpuric rash on the lower extremities. ESR 100. BUN/creatinine 20/1.0. Chest x-ray with no infiltrates. The best test to determine diagnosis?
Dx polyarteritis nodosa
Check abdominal angiogram (will show saccular aneurysms)
Usually spares lungs
169
A 40-year-old man presents with complaints of weakness of left-hand and abdominal pain. Exam reveals decreased power in the left hand. ESR 96. Urinalysis with 1+ protein, RBCs >50/hpf. KUB and abdominal x-ray shows no obstruction or perforation. Most likely diagnosis?
Polyarteritis nodosa
Not GPA, EGPA, or SLE
Tx steroids and cyclophosphamide
170
A 35-year-old patient presents with abdominal pain. Labs reveal renal insufficiency. Hepatitis B sAg positive. This patient most likely has?
PAN
| About 30% of PAN have HBV SAg positive
171
A 24-year-old woman of Italian/Jewish/Arab descent with recurrent abdominal pain every two months lasting 1 to 2 days. Patient had appendectomy in childhood for abdominal pain. During the attacks of periumbilical pain which spreads all over the abdomen, she gets high fever. Swollen knee. Power normal, no ulcers. Abdominal imaging studies normal. Father had similar symptoms during childhood. Diagnosis?
Familial Mediterranean Fever (serositis and arthritis)
All the “linings” (joints, gut, etc) can be affected
Tx colchicine for prophylaxis
Complication of FMF is AA amyloidosis —> leads to renal failure
172
Causes of amyloidosis (chronic inflammation) and diagnosis:
FMF
RA
MTB
Multiple myeloma (recall K>L)
Biopsy —> Congo red staining will show fibrils of apple green birefringence.
First do an abdominal fat biopsy; if negative, then biopsy affected organ.
173
A 50-year-old woman presents with cough, shortness of breath, hemoptysis and arthritis. Nasal septum is flattened. Chest x-ray with multiple pulmonary nodules and one cavitary lesion (thick-walled). AFB smear and cultures negative. BUN/creatinine 40/3.4. Biopsy shows vasculitis with necrotizing granuloma’s. Urinalysis shows RBCs >20/hpf, 1+ protein, c-ANCA (proteinase 3) positive, RF positive. The most likely diagnosis is? Tx?
Granulomatosis with polyangiitis (Wegener’s)
Not PAN, microPAN, or EGPA
```
Tx:
Non-severe —> steroids + MTX
Severe —> steroids + cyclophosphamide or rituximab
In remission —> rituximab
Relapse —> steroids + rituximab
```
This patient was severe
174
Thin and Thick-walled lung cavities
Thick: blasts, histo, GPA
Thin: cocci, nocardia, NTM
175
A 24-year-old man presents with ongoing sinusitis for the past couple of months, associated with cough. He was treated with amoxicillin for seven days. Exam reveals temperature of 100.5 F and boggy turbinates with purulent secretions. Rhonchi on auscultation. CT of sinuses shows total opacification and chest x-ray shows nodular infiltrates. ANA positive. Proteinase-3 antibodies positive (c-ANCA). Myeloperoxidase antibody positive (p-ANCA). Most likely diagnosis?
GPA
Not polyarteritis nodosa
FYI: c-ANCA is specific, p-ANCA is not specific
176
A 40-year-old woman with history of asthma has several allergies. She has no pets. She presents with shortness of breath, wheezing and complains of weakness of the left foot. She uses albuterol, salmeterol inhaler and is being weaned off steroids. Exam reveals rales at right upper lobe. Decreased power in the left foot with hypoactive reflexes. CBC with eosinophilia. Chest x-ray with right upper lobe density. Most likely diagnosis?
EGPA
| Not GPA, PAN, or SLE
177
In a patient with arthralgias and malar rash you are suspecting lupus. What do you check next?
ANA only
| Not ANA, anti-dsDNA, anti-Smith
178
What antibody is most specific for SLE?
Anti-Smith or anti-dsDNA titer
Not ANA, anti-ssDNA, or anti-histone
Recall larger decrease in C3 than decreased in C4; CH50 is also decreased
179
SLE Treatment
Follow SLE disease activity with anti-dsDNA levels
Arthritis: ASA, NSAID —> hydroxychloroquine —> belimumab
Photosensitivity, rash: avoid sun/use sunscreen —> steroid —> hydroxychloroquine —> +/- quinacrine
Thrombocytopenia —> steroids —> IVIG
Hemolytic anemia —> steroids
Nephritis —> steroids —> add cyclophosphamide —> MMF —> cyclosporine
In AA and Hispanics nephritis —> steroids —> MMF
180
Patient with SLE on steroids, still with symptoms.
What to do for musculoskeletal symptoms?
For nephritis, CNS symptoms, systemic vasculitis, alveolar hemorrhage?
Hydroxychloroquine
Cyclophosphamide
181
Patient is on minocycline for acne or rheumatoid arthritis for >2 years. Or patient is on procainamide for >1 year for an arrhythmia. Or hydralazine for CHF for past 2 years. Presents with malaise, low-grade temperature, arthralgias involving MCP, PIP joints, ESR 65 and ANA positive 1:320. C2 and C4 normal. All of the following favor a drug-induced lupus diagnosis except?
Drug-induced lupus NOT favored if there is high anti-dsDNA titer (that’s with etancercept only)
Drug-induced lupus IS favored by normal complement, no psychosis or seizures, urinalysis with no RBCs/casts/proteinuria, high ANA titer
182
A 24-year-old with history of lupus for the past four years was found to have lupus anticoagulant syndrome and history of spontaneous abortions twice. She is on cyclophosphamide and steroids. She asks you about best long-term contraceptive method. What would be your recommendation?
Progesterone (levonorgestrel) only intrauterine device
| Not medroxyprogesterone intramuscularly every 3 months
183
A 35-year-old woman was diagnosed with SLE and lupus nephritis. Patient was treated with steroids and IV cyclophosphamide. About two years ago her medications were tapered off and she remained asymptomatic and healthy. For the past month she has noted low-grade fevers and arthralgias. What would best suggest she has a flare of SLE?
Increased anti-dsDNA and decreased complement
184
A patient with lupus nephritis is started on cyclophosphamide and steroids. A week later sudden onset of headache, confusion, visual disturbance. BP 160/100. MRI brain shows enhancement in occipital lobes.
Reversible posterior leukoencephalopathy syndrome
| Tx —> control blood pressure
185
A patient with lupus who is in remission presents for a follow up in six months. She has no symptoms. Anti-dsDNA titer is elevated.
Serological active clinically quiescent disease
Tx: don’t treat (treat the patient not the numbers)
186
Leading cause of death in patient with SLE >10yrs is?
Cardiovascular disease
| Not nephritis
187
SLE increases risk for what?
```
Stroke
Myocardial infarction
DVT
Recurrent spontaneous abortions
Avascular necrosis
```
188
Patient with recently diagnosed SLE, presents with complaints of decreased urination and weakness of legs. Pain in the back as well. Hyperactive reflexes. Exam reveals NO spinal tenderness. What to do?
MRI spine to r/o transverse myelitis (MRI would show edema of spinal cord with inflammation)
If confirmed, Tx IV steroids
189
Pt with SLE on hydroxychloroquine and prednisone ~20 mg/day presents with psychosis. Most likely etiology?
>20mg of steroids —> often steroid-induced; auditory hallucinations
<20mg of steroids —> often SLE-itself (lupus cerebritis); visual and tactile disturbances
190
A 20 year old woman delivers a baby who has a complete heart block. The mother has a scaly papular rash. ANA negative. The woman is most likely to have what antibodies?
```
Anti SSA (anti-Ro)
Not anti-dsDNA, anti-RNP, or anti-Smith
```
191
Spontaneous abortions and checking for anti phospholipid antibody
1st trimester spontaneous abortion for the 1st time —> DON’T check
1st trimester spontaneous abortion for the 3rd time —> DO check
3rd trimester spontaneous abortion for the 1st time —> DO check
Huge positive means give prophylaxis with lovenox and aspirin with the next pregnancy
192
Sclerosis if generalized
Generalized = systemic sclerosis (scleroderma)
ANA positive
Anti-topoisomerase positive (Scl-70)
Anti-RNA polymerase III positive
193
Sclerosis if limited
CREST
Anticentromere positive
Higher risk for pulmonary HTN than scleroderma
194
Sclerosis if involves muscles
Dermatomyositis
Anti-Mi-2 positive
Anti-Jo-1 positive
Anti-PM-1 positive
195
Systemic sclerosis (Scleroderma; anti-Scl-70 positive) findings and Tx
Skin: diffuse fibrous thickening, sclerodacyly, tight face and small mouth (Tx methotrexate), abnormal nail fold capillaries, Raynaud phenomenon
Joints: symmetric arthritis, MCP (Tx NSAIDs 1st), tendon friction rub (Tx MTX 2nd)
Lungs: interstitial pneumonia is, interstitial fibrosis (check HRCT, Tx with MMF and steroids), pulmonary HTN
Renal: renal crisis (often precipitated by steroids; see HTN, proteinuria, edema; Tx with ACE-inhibitors irrespective of serum creatinine level)
GI: dysmotility (Tx erythromycin), GERD (Tx PPI), wide mouth diverticula, bacterial overgrowth syndrome (low B12, high folate; Tx rifaximin)
Heart: restrictive pericardial disease
196
Raynaud phenomenon, primary vs secondary
Secondary has dilated nailfold capillaries
Primary is idiopathic
Tx gloves then nifedipine
197
Patient with systemic sclerosis (scleroderma) can present with what lung findings?
```
Interstitial pneumonitis (fibrosis alveolitis)
Not alveolar pneumonitis, lobular pneumonitis, or obstructive PFTs
```
198
Anti-polymerase III antibodies
```
Systemic sclerosis (scleroderma)
These antibodies are a/w increased risk for diffuse thickening of skin, cancer, renal crises
```
199
CREST (anticentromere positive)
```
Calcinosis cutis (calcification of soft tissue)
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasias
```
Pulmonary HTN too more common than in scleroderma
200
A 35 year old gardener presents with complaints of painful fingers during cold weather. Fingers turn white when exposed to cold. Management?
Wear warm gloves
If that doesn’t work, then nifedipine
201
How will you recognize a rheumatological (secondary vs primary) disease in a patient with Raynaud phenomenon?
Abnormal nailfold capillaries
Age >40
Digital ulcerations
B/L hand involvement can be primary or secondary
202
A 40 year old with short stature presents with back pain. Exam reveals blue sclera and scoliosis. Excessive joint flexibility. Diagnosis?
Osteogenesis imperfecta
203
Patient’s exam reveals upper body to lower body ratio is decreased and arm span greater than the height. Thumb sign, wrist sign and pectus carinatum. Some scoliosis and heel valgus.
Marfan syndrome
| Check TTE next
204
Patient with joint laxity and hypermobility, translucent skin, easy bruising, atrophic scars, scoliosis and pes planus.
Ehlers-Danlos
205
A 45-year-old played basketball for four hours at a family reunion and now presents with swelling of the arms and legs sparing the hands and feet (fingers and toes). Exam reveals an orange peel-like quality of skin. CBC shows eosinophils positive.
Eosinophilic fasciitis
| Tx: observe —> steroids
206
Polymyositis
Anti-Jo-1 positive
HLA-DR3 positive, Women > Men
Proximal muscle weakness: can’t get up from a chair, can’t get up from a squatting position, can’t comb hair, mechanic’s hands (scaly dry, darkened, cracked horizontal lines on the palmar area, finger pads and lateral aspect).
Abnormal CPK >10x normal, increased ANA titers.
Biopsy —> myonecrosis with cellular infiltrate
207
Conditions mimicking polymyositis
Colchicine polymyositis —> vacuoles WITHOUT inflammation on muscle biopsy
Inclusion body myositis —> vacuoles WITH inflammation on muscle biopsy
208
A 66-year-old woman presents with complaints of difficulty getting up from a chair and difficulty combing her hair. Exam reveals rash on her cheeks, forehead and upper eyelids. This patient is at risk for developing which disorder?
Malignancy: ovary, breast or GI
Not amyotrophic lateral sclerosis or multiple sclerosis
209
Dermatomyositis
Dermatomyositis (anti-Mi-2 positive) = Polymyositis + skin changes
Gottron’s papules (on knuckles; more specific)
Not heliotropic rash (on eyelids; not specific)
Weakness
Next check CPK. If elevated, Tx steroids NOT methotrexate
210
Patient with dermatomyositis and mechanic’s hands with interstitial lung disease and Raynaud’s phenomenon. Dx and Tx?
Anti-synthetase syndrome
| Tx azathioprine or tacrolimus
211
A 55-year-old woman presents with weakness. Past history significant for polymyositis. Labs reveal a CPK of 950. Patient started on prednisone 50 mg daily, she feels better and the CPK decreases to less than 190. Three months later, CPK levels within normal limits, but the patient complains of new onset difficulty getting cereal from shelf in the supermarket. Dx?
Steroid myopathy
| Tx by tapering the steroids
212
A 65-year-old man with progressive weakness of legs, arms, forearms, wrists and fingers (distal > proximal). CPK high (<10x normal). Not responding to steroids —> MTX added, CPK still high. Biopsy reveals endomysial inflammation and basophilic rimmed vacuoles. What to do?
Inclusion body myositis
| Taper the prednisone and discontinue methotrexate
213
Patient with gouty tophus on colchicine and allopurinol. History of asthma and on inhaled steroids as well, presents with complaints of weakness while trying to get up from chair. Lower extremity proximal muscle weakness positive. DTRs decreased. CPK 350. Muscle biopsy reveals vacuoles (no information). Most likely diagnosis?
Colchicine polymyositis
Tx by stopping the colchicine
Not polymyositis, inclusion body myositis, or steroid myopathy
214
A 35-year-old intubated asthmatic, four days later, on steroids and albuterol, difficulty extubating. CPK 925. Most likely diagnosis?
Critical illness myopathy
| Not steroid induced myopathy
215
What autoantibodies for:
1) SLE (2)
2) Drug-induced SLE
3) Subacute cutaneous SLE
4) Scleroderma
5) CREST
6) MCTD
7) Poly/dermatomyositis (2)
8) Sjogren Sn (2)
9) Wegener’s
10) Microscopic polyarteritis angiitis
1) dsDNA, Smith
2) histone
3) SSA (Ro)
4) Scl-70
5) centromere
6) RNP
7) Jo-1, Mi-2
8) SSA (Ro)/SSB (La)
9) c-ANCA (proteinase-3)
10) p-ANCA (myeloperoxidase)
