Rheumatology Part 2- Paulson (exam 2) Flashcards
(151 cards)
1
Q
Fibromyalgia: Describe.
A
A chronic clinical syndrome of generalized musculoskeletal pain.
-Controversial condition
“real disease” vs. psychological disease vs. medicalization of socially constructed entity?
2
Q
Fibromyalgia: Associated symptoms?
A
- Fatigue
- Disordered sleep
- Multiple somatic symptoms
- Cognitive problems
- Psychiatric symptoms.
3
Q
Fibromyalgia: Epidemiology?
A
- Very common
- Especially among those visiting rheumatology and pain management providers.
- Much more common in women
- Especially 20-50
- Estimates of percentage affected range from 2-10%
4
Q
Fibromyalgia: Etiology?
A
- Unknown
- Genetic basis
- Psychosocial factors
- Neuroendocrine dysfunction
- ANS dysfunction
- STRESSFUL EVENTS
- Viruses/Infections
5
Q
Fibromyalgia: Pathophysiology? dont memorize
A
- Unknown cause
- Disorder of altered pain processing & regulation: –“Central sensitization”
- -Allodynia & hyperalgesia
- Functional MRI and PET studies
- Genetic basis
- Psychosocial factors
- Neuroendocrine dysfunction
- Autonomic nervous system dysfunction
- Stressful events
- Viruses/infections
6
Q
Fibromyalgia: Describe allodynia.
A
Allodynia refers to central pain sensitization (increased response of neurons) following normally non-painful, often repetitive, stimulation. Allodynia can lead to the triggering of a pain response from stimuli which do not normally provoke pain.
7
Q
Fibromyalgia: Describe hyperalgesia.
A
Hyperalgesia is an enhanced pain response. It can result from either injury to part of the body or from use of opioid painkillers. When a person becomes more sensitive to pain as a result of taking opioid medication, it’s called opioid-induced hyperalgesia (OIH).
8
Q
Fibromyalgia: What imaging studies have supported the little we understand about fibromyalgia?
A
- Functional MRI and PET studies.
9
Q
Fibromyalgia: Presentation?
A
- Chronic pain/stiffness (generalized): *Involves all 4 quadrants of the body
- *Pain often described as worst around neck, shoulders, low back, and hips
- Common associated complaints
- Onset often after acute injury, infection, childbirth, persistent stress, exposure to toxins.
10
Q
Fibromyalgia: Common associated complaints?
A
- Sleep disturbance
- Fatigue
- Muscle weakness
- Paresthesias
- Cognitive disturbance
- HA
- Depression
- Anxiety
- IBS
- Dry mouth
- Pelvic pain
- Bladder symptoms
- Tinnitus
- Multiple chemical hypersensitivities
- TMJ issues
11
Q
Fibromyalgia: What is a unique physical exam associated with fibromyalgia?
A
- Exam of tender points.
12
Q
Fibromyalgia: Describe the exam of tender points.
A
- Exam is Normal apart from pain at tender points.
- Apply 4 kg/cm^2 to the points (apply enough pressure to whiten the nail bed of the fingertip of the examiner)
13
Q
Fibromyalgia: What is the ACR( american college of rheum) Classification Criteria for tender points?
A
- 9 pairs (18 total) of tender points.
- 1190 Dx criteria: 11/18 tender points and symptoms of widespread pain (above and below the waist, and both sides of the body)
- Note control locations.
14
Q
Fibromyalgia: When performing an exam of tender points, where are the control locations?
A
- Thumb
- Mid-forearm
- Forehead
15
Q
Fibromyalgia: Where are the anterior tender points for fibromyalgia?
A
- Under sternomastoid m.
- Near 2nd costochondral junction
- 2 cm distal to lateral epicondyle
- Prominence of the greater trochanter.
- Medial fat pad of the knee.
16
Q
Fibromyalgia: What are the posterior tender points for fibromyalgia?
A
- Insertion of the suboccipital m.
- Mid-upper trapezius m.
- Origin of the supraspinatus m.
- Upper outer quadrant of the buttock.
17
Q
Fibromyalgia: Labs/Imaging?
A
- Generally of little benefit to making the dx.
- Fibromyalgia itself doesn’t cause any abnormalities.
- CBC, ESR, CRP, TSH would be reasonable initial lab tests
18
Q
Fibromyalgia: DDx
A
RA SLE Polymyositis PMR OSA Hypothyroidism Many, many more
19
Q
Fibromyalgia Initial approach/initial tx:
A
Initial approach: Pt education Good sleep hygiene (poor sleep often causes worsening pain) Exercise (low-impact aerobic activity) CBT?? Meds (next slide)
20
Q
Fibromyalgia Tx: What medications are not helpful for these patients?
A
**Not helpful:
Opioids
Corticosteroids
NSAIDs
21
Q
Fibromyalgia Tx: What medications are associated with helping these patients?
A
- TCAs
- Cyclobenzaprine (Flexeril)
- SNRIs
- SSRIs
- Anticonvulsants
22
Q
Fibromyalgia Tx: Examples of TCAs?
A
- **Amitriptyline (Elavil)
- nortriptyline (Pamelor)
- desipramine (Norpramin)
23
Q
Fibromyalgia Tx: Examples of SNRIs?
A
- **duloxetine (Cymbalta)
- minacipran (Savella)
24
Q
Fibromyalgia Tx: Examples of SSRIs?
A
- fluoxetine (Prozac)
25
Fibromyalgia Tx: Examples of anticonvulsants?
- **pregabalin (Lyrica)
| - gabapentin (Neurontin)
26
Fibromyalgia Tx: What are appropriate referrals for these patients?
-Studies show patients seem to do well with close PCP follow up
-If needed, can refer to:
Psychiatry
Physical Therapy
Rheumatology
Pain Management
Alternative: massage, acupuncture, etc
27
Fibromyalgia: Prognosis?
- Chronic, but not considered progressive.
- Minimal or no improvement in symptoms despite variety of treatment modalities.
- Pts do well with close PCP follow up
- Most patients are able to work
- Female gender, low socioeconomic status, unemployment, depression, and obesity associated with worse outcomes
28
PMR: What does PMR stand for?
- Polymyalgia rheumatica
29
GCA: What does GCA stand for?
giant cell arteritis
30
Describe PMR
Inflammatory condition associated with pain and stiffness of the hips and shoulders
31
Describe GCA
aka Temporal Arteritis: headache, jaw claudication, and visual symptoms associated with elevated ESR that can cause blindness
32
Frequently GCA and PMR ____
coexist
| -Thought to represent a spectrum of the same disease
33
Epidemiology/Risk factors for GCA and PMR:
- Almost always patients >50 years
- Increases with increasing age
- Women >>men
- Highest in Scandinavian populations & northern Europeans
- Less common in Japanese, Black, and northern Indians
* *PMR much more common than GCA
34
_____ increases GCA risk
smoking
35
______ decreases risk of GCA
Diabetes
36
Pathophysiology of GCA/PMR:
- Both PMR and GCA are assoc. with _____
- affected joints have..?
- Unknown cause
- Both PMR and GCA are associated with polymorphisms of HLA-DR alleles.
- Affected joints have lymphocytes and monocytes causing inflammation in PMR
37
In GCA, there is infiltration of inflammatory cells into the vessels causing a ________
-**vasculitis
38
MC sites of GCA?
MC in the thoracic aorta, large cervical arteries, and branches of the external carotid arteries
39
In GCA, areas of active inflammation can ______
thrombose
| -as well as fragmentation of the elastic lamina
40
Clinical Manifestations- PMR
- **Pain and stiffness in the shoulder and pelvic areas
- -Usually shoulders come first
- -Morning stiffness & stiffness after activity. “gel phenomenon”
- -Movement worsens pain
- -Impaired range of motion
- -Should have normal muscle strength
- Nonspecific systemic symptoms: low-grade fever, malaise, weight loss
- -Can be the first symptoms
- Bursal inflammation/synovitis
- -Wrists, knees, sternoclavicular joints can have swelling and/or pitting edema
41
Classic Sx of GCA:
**headache, scalp tenderness, jaw claudication, visual changes (esp. amaurosis fugax or diplopia)
42
amaurosis fugax=
transient vision loss (NEVER normal!!)
43
Vision loss in GCA usually stems from?
- anterior ischemic optic neuropathy
- -Occlusive arteritis of the posterior ciliary artery (branch of ophthalmic artery)
- Chief blood supply to the optic nerve
44
About half of GCA Pts have ____
PMR
45
Constitutional Sx of GCA
fever, fatigue, weight loss, malaise
46
Atypical Sx of GCA
respiratory symptoms (dry cough), neurologic symptoms, otolaryngeal symptoms
47
Physical Exam-GCA
- Might look ill overall
- Temporal artery can be thickened, tender, prominent, or normal appearing
-Funduscopic exam:
Can see pallor and edema of the optic disc, with scattered cotton-wool patches and small hemorrhages, or can have a normal funduscopic exam
-Cardiovascular exam:
Asymmetry of pulses in arms, aortic regurgitation murmur, bruits near clavicle if GCA has affected the aorta or major branches
48
Labs: GCA
```
-**Elevated ESR/CRP
Anemia
-WBC usually normal
-May have reactive thrombocytosis
-May have abnormal LFTs (esp ↑ALP)
-Albumin can be low
```
49
Define elevated Sed rate
normal sed rate is less than 20.
50
DDx PMR
Rheumatoid arthritis
Fibromyalgia
Polymyositis
Infections ie: endocarditis, osteomyelitis
Drug-induced myopathy/myalgia (esp. statins)
-RS3PE syndrome (Remitting Seronegative Symmetrical Synovitis with pitting Edema)
-hypothyroidism
51
DDx- GCA
```
Takayasu Arteritis
Migraine headache
Meningitis
Retinal Vein or Artery occlusion
Amyloidosis
Small and medium vessel vasculidities
Wegener’s granulomatosis, polyarteritis nodosa, microscopic polyangitis
Malignancy
Sinus infection
```
52
Diagnosis for PMR
- Clinical Diagnosis
| - Multiple scoring/classification systems exist
53
GCA dx (what is the gold standard test**?)
- **Temporal artery biopsy is gold standard
- If GCA suspected, but a unilateral biopsy is negative --> contralateral biopsy
- Classification criteria does not replace biopsy for definitive diagnosis
54
Tx PMR
-Glucocorticoid therapy:
**Prednisone 15 mg PO daily
(Normal starting doses might range from 10-20 mg)
-If no improvement after 7 days, could ↑ to 30 mg
-If still no improvement, consider a different Dx.
(**Rapid improvement generally seen-- alerts you that you have the correct dx)
- After stable and Sx controlled, start tapering dose
* *Flares are common--> increase dose and taper slower
55
Tx GCA
-***Don’t wait for biopsy results to start treatment!!
-**Goal is to prevent permanent blindness
-Glucocorticoids: **Prednisone 40-60 mg PO daily
--If no response, increase dose
Once controlled (usually 2-4 weeks), start tapering
-No faster than reducing by 10% every 1-2 weeks
-Will need to be on prednisone for months
-Think about osteoporosis prevention!
-***Flares common- increase prednisone by 10 mg. Inflammatory markers are helpful (CRP is the first to increase)
56
Prognosis: PMR
- Good, with steroid therapy
- Many patients have a self-limiting course
- Does not cause chronic damage
- Most morbidity is related to long-term steroid use
57
Prognosis: GCA
- **If not treated, can have a poor prognosis and lead to permanent blindness
- Fair number have a chronic course and relapses
- Association with increased cardiovascular events (MI, CVA, & PVD)
- Long-term steroid risk has consequences
-Referral for either: rheumatology
58
Takayasu Arteritis (TA): describe this condition
=**Chronic vasculitis mostly affecting the aorta and main branches
--Speculated to be on the spectrum of PMR/GCA with similar pathophysiology
59
Takayasu Arteritis (TA): demographic
Most common in women, Asians
| --Onset usually between 10 and 40
60
Takayasu Arteritis (TA): early sx and later sx
- Constitutional symptoms common early in disease
- Later, symptoms of vascular insufficiency: Claudication, cool extremities, subclavian steal syndrome can lead to syncope, BP differential, arthralgias, skin lesions, pulmonary manifestations, abdominal pain/diarrhea/GI hemorrhage, angina pectoris
61
TA PE findings
-BP differential (usually at least 10 mmHg), diminished, asymmetrical arterial pulses in arms/legs, bruits, may have synovitis in large joints, renovascular hypertension
62
TA: labs
mild anemia, elevated ESR/CRP, low albumin
63
TA: dx
Suspected with clinical features, then imaging showing arterial luminal narrowing or occlusion with wall thickening
64
TA: tx
Glucocorticoids: **Prednisone 45-60 mg PO Qam initially
- Taper when symptoms controlled/labs improved.
- Many need chronic steroids.
- Other immunosuppresants are second line.
- Surgical intervention with PCTA (percutaneous angioplasty), bypass grafting, or aortic repair may be needed.
65
TA: prognosis
chronic disease that’s relapsing-remitting. 20% with self-limited disease. Vascular involvement generally progressive.
66
Reactive Arthritis (ReA): describe this condition (KNOW)
AKA Reiter’s Syndrome or seronegative spondyloarthropathy
| =Asymmetric polyarthritis that develops after a GI or GU infection, typically of large lower extremity joints
67
Reactive Arthritis Epidemiology/risks:
- Relatively rare (about 4/100,000)
- Male >female
- Typically young adults (most common in 3rd decade)
- Strong association with HLA-B27 gene (60-80% of patients)
68
Reactive arthritis Pathophysiology: what kind of infection and MC pathogens associated
- GI infection (Shigella, Salmonella, Yersinia, Campylobacter, Escherichia coli, Clostridium difficile)
- -->Inflammation in gut compromises wall integrity and activate proinflammatory cytokines --> microlesions--> cytokines migrate to joint/entheses
-Sexually transmitted infection (Chlamydia, Ureaplasma urealyticum)
69
Those with ______ are predisposed to developing inflammatory enthesopathy, which is triggered by exposure to certain infectious agents
HLA-B27
70
Enthesopathy=
refers to a problem with the attachment of tendons, ligaments or components of a joint onto the bone. People with enthesopathy typically experience pain and may have stiffness or difficulty moving the affected joint or area of the body.
71
Clinical Sx/s reactive arthritis
- Sx of diarrhea or urethritis (1-4 weeks earlier)
- ->May have asymptomatic GU infection
- **Asymmetric arthritis usually involving large weight bearing joints (knee, ankle) which may persist
- (+/-) axial arthritis- spine or SI joint (less common)
- (+/-) enthesitis--> Often seen as swelling at heel
- (+/-) dactylitis
72
enthesitis=
inflammation of the entheses, the sites where tendons or ligaments insert into the bone.
73
Dactylitis=
severe inflammation of the finger and toe joints
74
Clinical Manifestations-extraarticular for Reactive Arthritis
- General: Fever, fatigue, weight loss
- Eye: Conjunctivitis, anterior uveitis
- Mucocutaneous: Balanitis, stomatitis, keratoderma blennorrhagicum
- Nails: Mimics psoriasis
75
Balanitis=
inflammation of the skin at the end of the penis
76
keratoderma blennorrhagicum=
skin lesions commonly found on the palms and soles but which may spread to the scrotum, scalp and trunk
77
DDx Reactive arthritis
-Disseminated gonococcal infection
-Rheumatoid arthritis
-Ankylosing spondylitis
-Psoriatic arthritis
-Behҫet disease
-Gout/pseudogout
-Viral gastroenteritis
-Rheumatic fever
Septic arthritis
78
Labs: Reactive Arthritis
- (+/-) elevated CRP/ESR
- (+/-) positive stool culture or urethral swab
- Synovial fluid analysis: elevated WBC, mostly neutrophils
- -Won’t recover causative organism
79
Dx: ReA
- Clinical dx
- -No single diagnostic test
- Musculoskeletal findings
- Pt has a Preceding infection
- No more compelling explanation for the arthritis
80
Tx: reactive arthritis
- Treat the underlying infection
- -When pts are given antibiotics for STIs at time of dx, it reduces chance of ReA.
- Arthritis treatment:
* *NSAIDs--> Mainstay/initial therapy--Naproxen, diclofenac, or indomethacin for approx. 2 weeks
- If no response to NSAIDs:
- Intraarticular glucocorticoids (triamcinolone)
- PO glucocorticoids (prednisone)
- DMARD: sulfasalazine or methotrexate
81
Prognosis/Rehab/Referral: Reactive Arthritis
- Most patients have self-limiting disease
- Typically lasts 3-5 months
- Most patients remit within 6-12 months
- May require PT
- Refer to rheumatology if needed
82
Sjӧgren Syndrome: describe this disease (and 2 MC symptoms)
=Systemic autoimmune disease that commonly affects the lacrimal and salivary glands, resulting in mouth **(xerostomia) and eye dryness **(keratoconjunctivitis sicca)
83
Sjӧgren Syndrome: are other organs affected?
- Other organs may be involved as well
| - -***Can be primary or secondary to another autoimmune disease
84
Epidemiology/Risk Factors: Sjӧgren Syndrome
- Women>men 10:1
- Peak is in middle-aged females
- Seen in all races, Caucasian slightly more common
- Estimated to affect 0.1-0.6% of adult females
-Tricky to truly estimate, because prevalence depends on classification criteria used.
-Genetic component
HLA-DQ and HLA-DB
85
Clinical Manifestations-Ocular--> Sjӧgren Syndrome
keratoconjuntivitis sicca:
- Diminished aqueous tear production
- Eyes feel dry, gritty, sandy, foreign body sensation, burning, itching, intolerance to contacts, photophobia, eye fatigue
- Conjunctival or corneal damage seen on Rose Bengal, lissamine green, or fluorescein staining
- Schirmer test showing decreased tear production
- May see mucus filaments, dilation of bulbar conjunctival vessels, or lacrimal gland enlargement
86
What can keratoconjuntivitis sicca progress to?
Can progress to corneal ulceration,
bacterial keratitis, eyelid infection with
with destruction of corneal epithelium
87
What is the objective test used to assess for dry eyes?
schirmer test (stick a piece of paper in their eye and see how dry their eyes are)
88
What is the objective test used to assess dry mouth?
Saxon test or sialometry
89
Clinical Manifestations-Mouth: Sjӧgren Syndrome
* *Xerostomia:
- “cotton mouth” sensation, dysphagia (esp. dry foods), trouble with prolonged speaking, loss of taste
- Dental caries, (esp. at gum line), gingival recession, oral candidiasis common, laryngotracheal reflux, chronic esophagitis
- Salivary gland enlargement can occur
- --Parotid most obvious
- Saxon test or sialometry
90
Describe the Saxon test
Pt chews on the sponge for 2 minutes (weigh the sponge before and after)
91
Clinical Manifestations of Sjӧgren Syndrome (constitutional, Skin, MSK, Lungs)
Constitutional: Fatigue, low-grade fever
Skin: Xerosis, purpura, Raynaud phenomenon, cutaneous vasculitis, angular chelitis, annular erythema
Musculoskeletal: Arthralgia, symmetric, nondeforming, intermittent. Mild myopathy with weakness
Lungs:
- Dry cough, excessive throat clearing, rhinitis, sinusitis, hoarseness
- Non-specific interstitial pneumonitis (NSIP) , lymphocytic interstitial pneumonitis (LIP), usual interstitial pneumonitis (UIP), cryptogenic organizing pneumonia (COP)
92
Clinical Manifestations of Sjӧgren Syndrome (cardiac, GI, Renal)
Cardiac: Rare, but can have pericarditis, heart block
GI: Atrophic chronic gastritis, celiac disease more common than general population, abnormal LFTs, primary biliary cirrhosis, autoimmune hepatitis
```
Renal: Chronic interstitial nephritis most common.
Glomerular disease (less common)
Membranoproliferative glomerulonephritis (MPGN) and membranous nephropathy (MN)
```
93
Clinical Manifestations of Sjӧgren Syndrome (Urogenital, neurologic and psychiatric )
Urogenital: Vaginal dryness, dyspareunia, pruritis
Dysuria, urinary frequency, urinary urgency, nocturia in absence of UTI
Neurologic and Psychiatric:
Painful peripheral neuropathy, ataxia, trigeminal neuropathy, mononeuritis multiplex, multiple cranial neuropathies
CNS involvement with focal lesions, chorea, aseptic meningitis, diffuse brain lesions, spinal cord involvement, cognitive dysfunction “brain fog”
-Affective disorders common, especially depression
94
Labs Sjӧgren Syndrome
- **Most patients have positive ANA
- **Many have positive antibodies for SS-A (anti-Ro) and SS-B (anti-La)
- **Many positive for RF
-Others: centromere antibodies, anti-CCP, antimitochondrial antibodies
Mild anemia, cytopenia, hypergammaglobulinemia
-May have elevated ESR. CRP can be normal or elevated
-May have abnormal LFTs
If impaired renal function, may have ↑Cr and/or ↓ bicarb.
95
Dx test for Sjӧgren Syndrome
-**Salivary Gland Biopsy--> Long considered gold standard
In real life, often saved for patients when diagnosis is unclear
- ≥4 salivary gland lobules are removed from incision on inner lip
- Positive if focus score of ≥1 per 4 mm2
- --A focus is a cluster of ≥50 lymphocytes
96
DDx: Sjӧgren Syndrome
```
Age-related sicca syndrome
Medication side effects
Chronic Hepatitis C
Sarcoidosis
HIV patients
Sialadenitis
Head and neck radiation
```
97
How to make a dx of Sjӧgren Syndrome
1. ) The patient has an objective test of dry eye (Schirmer or abnormal surface staining) or xerostomia (Saxon or whole sialometry).
- Or imaging consistent with glandular abnormalities seen in SS
2. ) The patient has anti-Ro/SSA and/or anti-La/SSB antibodies, a positive lip biopsy, or an established rheumatic disease
- Or anticentromere antibodies
- Or ANA ≥1:320 + positive RF
98
Tx oral Sx: Sjӧgren Syndrome
- Symptomatic
- Artificial saliva
Mouth lubrication/oral care:
- Maintain good hydration
- Sugar free gum & hard candies
- Pilocarpine or cevimeline
- Fluoride treatment, regular dental visits, aggressive mouth care
Immunosuppresants-mixed results in studies
99
Tx Ocular Sx: Sjӧgren Syndrome
-Environmental management (if they can avoid dry, windy areas)
- Regular use of artificial tears:
- -Every 2-4 hours
- -At night, ocular ointments are helpful
- If no relief with above, ocular cyclosporine 0.05%-->Can use with artificial tears
- Topical steroids may be used if fail above, have ocular inflammation, and work with ophthalmologist
- Punctal occlusion/plugs (block tears from getting out-- last case scenario)
100
Tx-Systemic Manifestations: Sjӧgren Syndrome
Immunosuppressive therapy:
- Hydroxychloroquine
- Methotrexate
You will probably have referred to rheumatology at this point if the patient has systemic disease
101
Sjӧgren Syndrome- Prognosis/referral?
- Depends on extent of involvement
- Severe exocrine involvement--> higher lymphoma risk-->Usually B cell non-Hodgkin lymphomas
- At higher risk of other autoimmune diseases
- Those with extraglandular disease do have a higher M&M
- Refer if:
- -Systemic signs & symptoms
- -Ocular dryness not responding to artificial tears
102
Rheumatoid Arthritis (RA): describe this condition
Chronic, systemic inflammatory disorder with synovitis of the joints that can lead to joint destruction, deformity, and disability
103
RA risk factors/epidemiology
- Women>men 3:1
- About 0.5-1% of adults
-Peak onset:
Women: 4th-5th decade
Men: 6th-8th decade
- Significant genetic component
- Smoking significantly increases risk
104
RA Pathophysiology
- **Genetics: HLA-DR MHC genes are the most important.
- “shared epitope”
- Synovial tissues are the main target for the autoimmune process-->
- T cells, B cells, macrophages, synovial cells go to synovial tissues-->
- Synovial tissue proliferates (synovitis), there is excess synovial fluid, and a pannus forms -->
- Pannus invades nearby bone and cartilage, destroying them and stretching the joint capsule, causing destruction & deformity.
105
Clinical Manifestations-Joints RA
- Usually insidious onset
- ***Morning stiffness >30 minutes
- May recur after daytime inactivity
- Symmetric swelling of many joints
- Tender, painful joints
- **Joints most often affected: PIPs fingers, MCPs, wrists, ankles, knees, MTPs
- May have synovial cysts, tendon rupture, or entrapment syndrome
106
Clinical Manifestations-Joints of Hands RA
- Ulnar deviation of MCP joints is classic
- Swan neck deformity
- Boutonnière deformities
- Z deformity
107
Describe a swan neck deformity
Hyperextension of PIP, flexion of DIP
108
Describe Boutonnière deformities
Flexion of PIP, extension of DIP
109
Describe Z deformity
Hyperextended interphalangeal joint
110
Clinical Sx/s: RA
- General?
- skin?
General: Fatigue, weight loss, low-grade fever
```
Skin:
Rheumatoid nodules:
-Subcutaneous nodules
--**Almost only in those RF positive
-Often on extensor surfaces (esp. forearms), over joints, or pressure points
-Firm, not tender
-May also be in lungs, sclerae, other tissues
-Vasculitis
```
111
Clinical Sx/s: RA
| -ocular?
- Keratoconjunctivitis sicca (secondary Sjӧgren Syndrome)
| - Scleritis/episcleritis, scleromalacia
112
Clinical Sx/s: RA
| -pulmonary?
Pleural effusions
Rheumatoid nodules
Interstitial lung disease
113
Clinical Sx/s: RA
- Chronic inflammation increases risk for CV disease
- Pericardial effusions
- Pericarditis
114
Felty Syndrome:
| classic triad
- Splenomegaly
- Neutropenia--> Could be asymptomatic or have recurrent bacterial infections
- RA-->Typically seropositive, erosive, and severe
115
Labs: Felty Syndrome
-**Anti-CCP antibodies-->Most specific bloodwork for RA. Associated with aggressive disease.
- **Rheumatoid Factor (RF)--> Associated with more severe disease
- About 15% of patients are seronegative.
- **Elevated ESR/CRP
- May see mild anemia, thrombocytosis, WBC normal or mild leukocytosis
-Synovial fluid: inflammatory effusion, leukocytes usually 1500-25,000/cubic mm, PMNs predominate
116
Imaging studies: RA
- early on in the disease? vs
- Findings late in the disease?
-Radiographs are most specific
--If early in disease, likely normal
-Initial: soft tissue swelling, osteopenia around joint
Earliest changes show in wrists or feet
-Later: Joint space narrowing and erosions
117
What is the most sensitive imaging study for RA?
- MRI (More sensitive than radiography)-->May detect erosions earlier in the disease course
- Ultrasound: Also more sensitive than radiographs
118
DDx RA
```
Osteoarthritis
PMR
Psoriatic arthritis
Chronic tophaceous gout
CPPD deposition disease
SLE
Rheumatic fever
Reactive Arthritis
```
119
Dx: RA
- Inflammatory arthritis involving ≥ 3 joints
- Positive RF and/or anti-CCP
- ->If seronegative, can still diagnose RA if you have excluded other causes and all other characteristics met.
- Elevated ESR and/or CRP
- Duration of ≥ 6 weeks
- You have excluded other causes
120
RA treatment goals
- Control pain and inflammation
- Preserve function
- Prevent deformity
- ***Early diagnosis and initiation of DMARDs (Disease-modifying antirheumatic drugs) is important!!
- Target of treatment is remission or low disease activity
- Rheumatologist involvement
- Patients often need combinations of DMARDs
- ->**MTX + TNF inhibitor most common
121
RA: pretreatment screening
Baseline CBC, Cr, LFTs, ESR, CRP
Ophthalmic screening
Check for latent TB
Baseline radiographs
122
NSAIDs as tx for RA? y/n?
Help with symptoms, but don’t alter disease course
- -Not for monotherapy
- All are about equal
123
Corticosteroids for RA tx
- Very helpful for both Sx relief and slowing the rate of joint damage
- Not recommended for monotherapy or long-term use
- **Good as a bridge while starting a DMARD.
- Taper as soon as able.
- **Start with prednisone 5-20 mg/day, depending on how severe the Sx
124
Conventional DMARDs (list examples)
**Methotrexate
*Sulfasalazine
*Hydroxychloroquine
Leflunomide
Gold (intramuscular & oral)
Azathioprine
Minocycline
Cyclosporine
Penicillamine
125
Biologics (ex's)
Infliximab (Remicade)
Adalimumab (Humira)
Golimumab (Simponi)
Certolizumab (Cimzia)
Abatacept (Orencia)
Rituximab (Rituxan)
Tocilzumab (Actemra)
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What is the initial DMARD of choice for tx in RA pts?
Methotrexate
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- Starting dose of Methotrexate
- S/E?
- all Pts on methotrexate MUST take ____ ____
- Normal starting dose is 7.5 mg PO weekly
- Max dose 20-25 mg/wk
- Usually note improvement within 2-6 weeks.
- Contraindicated: pregnancy (teratogenic), liver disease, heavy alcohol use, severe renal impairment
-S/E: GI upset, stomatitis
Needs close monitoring:
- CBC to monitor for cytopenias
- LFTs for hepatotoxicity
-**All should take folic acid 1 mg PO daily or leucovorin calcium 2.5-5mg weekly 1 day after taking MTX to prevent hematologic & other s/e.
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TNF inhibitors (which one is 1st line, and when should they be prescribed?)
-SQ or IV
-Expensive!!
-Well tolerated
Have a much higher risk of serious bacterial infections, as well as granulomatous infections (esp. reactivation of TB)
-**MUST screen for latent TB prior to starting
-Can worsen CHF
-Work really well, esp. for those with severe disease
-**Etanercept generally 1st choice.
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Follow-up: RA pts
- Assess Sx and functional status at all visits
- Variety of scales that can be used. Pick one, and be consistent
- Monitor lab work for disease activity and potential toxicities of medication
- **Follow radiographs every 2 years
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Prognosis: RA
-Prior to use of MTX, very poor prognosis: morbidity, mortality, and financial loss
- With successful treatment options, less deformity, joint surgeries, morbidity & mortality
- Patients will have disease flares
- Higher mortality of those with RA attributed to cardiovascular disease from chronic inflammation
-**Poor prognostic factors: RF or anti-CCP positive, extraarticular disease, functional limitation, erosions on radiograph
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Polyarteritis nodosa (PAN): describe this condition
***Systemic necrotizing vasculitis that usually affects medium-sized or small muscular arteries
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PAN: which organs are affected? which are spared?
- Can affect any organ, but skin, muscle, peripheral nerves, kidneys, GI tract, heart are commonly affected.
- **Lungs usually spared
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Epidemiology/Risk Factors: PAN
- Slightly more common in males
- Can occur at any age, but more common in 40-60 year olds
- No racial group especially affected
- Incidence 1/100,000 people yearly
- *Associated with Hepatitis B
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Pathophysiology: PAN
- Most cases are idiopathic
- -Some associated with Hep B, Hep C, and hairy cell leukemia-->Called secondary PAN in these cases
- The inflamed wall of the vessel thickens and lumen narrows -->less blood flow, thrombosis more likely--> ischemia or infarction of tissue
- Inflammation can also weaken the vessel and result in aneurysm
- ***Veins are not involved
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Systemic Sx: PAN
- Fatigue, arthralgias, fever, weight loss, weakness
| - Pain in the extremities is often an early feature
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Classic symptoms of PAN: skin
- **Lower extremity ulcerations classic
- Subcutaneous nodules
- Livedo reticularis
- Bullous or vesicular eruption
- Palpable purpura
- May see infarction or digital gangrene
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Renal Sx: PAN
- **Kidney is the MOST commonly affected organ
- **Hypertension and renal insufficiency
- If severe, renal infarct
- Perirenal hematoma from rupture of renal arterial aneurysms
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GI Sx: PAN
-**Abdominal pain common
Esp. after meals. “intestinal angina”
-Nausea/vomiting/diarrhea
-Infarction can lead to acalculous cholecystitis, bowel infarction, perforation, necrotizing pancreatitis, etc.
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Cardiac Sx: PAN
- MI
| - CHF from uncontrolled HTN or from ICM
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Lungs Sx: PAN
not involved
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MSK Sx: PAN
- Pain in extremities, esp. early in disease process
- Arthralgia, myalgia
- Muscle weakness
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Eye Sx: PAN
Retinal hemorrhage
Visual impairment
Optic ischemia
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Nervous System Sx: PAN
- Mononeuritis multiplex
- Foot drop
- Usually sensory problems 1st, then motor deficits
- CNS involvement (less common): encephalopathy, seizure, stroke
Other: Ischemic orchitis
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Mononeuritis multiplex=
nerve damage to 2 different nerves in different parts of the body
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Ischemic orchitis:
Ischemic orchitis may result from damage to the blood vessels of the spermatic cord during inguinal herniorrhaphy, and may in the worst event lead to testicular atrophy.
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DDx: PAN
-Infectious diseases causing vascular inflammation or mimicking vasculitis--> Hep B/C, infectious endocarditis, HIV, mycotic aneurysm with embolization
-Systemic vasculidities:
Wegener’s, HSP, drug-induced vasculitis, Churg-Strauss, microscopic polyangitis
-Other diseases:
Atherosclerosis, thrombotic disorders, embolic disease, vasculitis from a connective tissue disease (ie: RA, SLE)
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Labs: PAN
```
-Elevated CRP/ESR
May have abnormal LFTs/Hepatitis panel
- (+/-) elevated Cr (due to renal fx impaired)
-Anemia
-CK may be elevated
```
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Dx PAN (KNOW!!)
- **Biopsy of an involved organ
- -> "Necrotizing inflammation of medium-sized arteries"**
Angiogram: Many small aneurysms. **“rosary sign”
-Irregular constrictions of larger vessels and occlusion of smaller vessels
-CT and MR angiography also helpful to show extent of organ involvement
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Tx: PAN
| -mild disease?
Does the patient have viral hepatitis?
If so, treat with antiviral, limit length of steroids.
Mild disease:
-Corticosteroids alone--> Prednisone 1 mg/kg daily (max 60-80 mg/day). Taper after 4 weeks if improved
If resistant/intolerant to steroids, methotrexate, azathioprine also used
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Tx PAN: Mod-severe disease
Initial: high-dose corticosteroids- Prednisone + immunosuppressant (cyclophosphamide) (IV or oral)
- If life- or organ-threatening, IV methylprednisone
- Switch off cyclophosphamide when possible to another immunosuppressant. Max 12 months.
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Prognosis: PAN
- Without treatment, poor prognosis (10%- 5 year survival)
- With treatment, 80%
- If successfully treated, relapses less common (20%)
Worse prognosis:
- CKD with Cr >1.6
- Proteinuria >1g/dL
- GI ischemia
- CNS disease
- Cardiac involvement
