Rheum part 1

Question 1

Pain and stiffness in shoulders and hips/pelvic girdle lasting several weeks with no other cause, worse with inactivity, resulting in nocturnal pain and morning predominance w/ morning stiffness >45 min
No blindness or cranial symptoms
Fever, malaise, weight loss, pain in lower back
Difficulty combing hair, putting on coat, rising from chair
NOT causing true muscle weakness

Answer 1

polymyalgia rheumatica

Question 2

What is polymyalgia rheumatica commonly associated with?

Answer 2

giant cell arteritis

Question 3

Is polymyalgia rheumatica or temporal arteritis associated with blindness?

Answer 3

temporal arteritis

Question 4

Clinical → labs = elevated acute phase reactants (ESR, CRP, anemia)

PE: strength is NORMAL

Answer 4

polymyalgia rheumatica

Question 5

how do you treat polymyalgia rheumatica?

Answer 5

None of the following”above head” symptoms (headache, jaw pain, scalp tenderness, visual symptoms): Prednisone daily with tapering after 2-4 weeks and low dose for one year
Methotrexate for more successful taper

Refractory = biologics

Question 6

Headache, jaw claudication, visual abnormalities, scalp tenderness, throat pain, jaw pain with chewing, throat pain
Temporal artery

CAN cause blindness (occlusion of posterior ciliary branch of ophthalmic artery) + large artery complications – amaurosis fugax, diplopia

Dry cough, FUO, rigor and sweats, painful paralysis, vague tongue, nose, ear pain

Answer 6

temporal arteritis

Question 7

Affects medium and large sized vessels

Nonclassical presentation = aortic regurgitation, arm claudication, dry cough, mononeuritis multiplex, FUO, vague pain

If older with FUO with elevated ESR/CRP and normal WBC, must be considered even w/o typical features

Answer 7

temporal arteritis

Question 8

PE: Temporal artery normal or nodular, enlarged, tender, pulseless
Fundoscopic exam may be normal for first 24-48 hours after blindness occurs
Asymmetry of pulses in arms, aortic regurgitation murmur, bruits near clavicle (subclavian artery stenosis)
→ aneurysm of thoracic aorta

LABS:
Normal WBC
ESR > 50
CRP slightly more sensitive, elevated but can be normal
Normochromic, normocytic anemia
Thrombocytosis
Alkaline phosphatase elevated
→ temporal artery biopsy for confirmation
US = “halo sign” on temporal artery

Answer 8

temporal arteritis

Question 9

when using an US on the temporal artery, a halo sign indicates

Answer 9

temporal arteritis

Question 10

how do you treat temporal arteritis

Answer 10

Prednisone started immediately for at least one month and then taper

If vision loss → IV methylprednisolone (usually vision loss is permanent :()

Tocilizumab - IL-6 antibody to reduce flares and steroid use

Question 11

How do you confirm temporal arteritis diagnosis?

Answer 11

MRI or CT angiography can help confirm diagnosis with narrowing, thickening or aneurysmal dilation

Question 12

those with temporal arteritis are also at higher risk for

Answer 12

TAAs, regurgitation, dissection

Question 13

Chronic and widespread aching pain and stiffness most commonly in neck, shoulders, low back and hips with fatigue, sleep problems, subjective numbness, chronic headaches

Minor exertion worsens pain and fatigue

Higher intramuscular pressure

Answer 13

fibromyalgia

Question 14

fibromyalgia is seen in

Answer 14

women 20-50 years, often correlated with IBS

Question 15

Syndrome of chronic, widespread MSK pain and stiffness w/ unknown cause - consider sleep disorders, depression, viral infections, rare complication of other illness (hypothyroidism, RA, OSA)

Answer 15

fibromyalgia

Question 16

PE: normal except for “trigger points” of pain produced with palpation of various areas (ex: trapezius, medial fat pad of knee, lateral epicondyle of elbow)

Dx of EXCLUSION – TSH helpful due to hypothyroidism risk
Rule out other diagnoses!

WPI and SS scales to dictate diagnosis

Answer 16

fibromyalgia

Question 17

whats the criteria for fibromyalgia

Answer 17

1) Widespread pain index (WPI) score of 7 or higher and symptom severity scale score of 5 or higher
(OR WPI 3-6 and SS 9 or higher)
2) experienced this at least 3 months
3) no other disorder could explain the symptoms

Question 18

How do you treat fibromyalgia?

Answer 18

Multidisciplinary:
Patient education, CBT, meditation
Exercise programs, massage, therapy
Cannabis, treat depression and anxiety

Amitriptyline, fluoxetine, duloxetine, chlorpromazine, milnacipran, cyclobenzaprine, pregabalin, gabapentin, low-dose naltrexone

FDA approved: pregabalin, duloxetine, milnacipran

Question 19

Insidious onset with fever, malaise, weight loss weeks-months

Pain in extremities - arthralgia, myalgia (calves), neuropathy (mononeuritis multiplex) w/ foot drop MC

Skin = livedo reticularis (could be from cold), subcutaneous nodules, ulcers, gangrene, commonly in lower extremity near malleoli

With abdominal involvement = N/V, diffuse abdominal pain from eating (postprandial)
Can cause perforation, HOTN

Answer 19

polyarteritis nodosa

Question 20

polyarteritis nodosa is ass with

Answer 20

vasculitis neuropathy (mononeuritis multiplex)

Question 21

Necrotizing arteritis/vasculitis of medium-sized vessels affecting skin, peripheral nerves, mesenteric vessels, heart, brain
Can be caused by hep B, rarely genetic (adenosine deaminase 2)

Does NOT affect the lungs, but can cause MI secondary to vasculitis/myocarditis

Answer 21

polyarteritis nodosa

Question 22

HTN due to renal artery dysfunction

LABS: Anemia, leukocytosis, ESR and CRP elevated
ANCA negative
Rh factor or ANA present but nonspecific
Hep B serologic tests
If childhood onset – genetic testing

Tissue biopsy or angiogram required for confirmation of diagnosis of symptomatic sites

Angiogram = aneurysmal dilations in renal, mesenteric, hepatic arteries (careful)

Answer 22

polyarteritis nodosa

Question 23

What is required for diagnosis of polyarteritis nodosa?

Answer 23

tissue biopsy or angiogram of symptomatic sites

Question 24

How do you treat polyarteritis nodosa?

Answer 24

Mild = NSAIDs or colchicine

Severe = IV methylprednisolone

Cyclophosphamide or other immunosuppressive agents lower risk of disease-related death (methotrexate or azathioprine)

Steroids in high doses to help control symptoms

Hep B = short course of prednisone + HBV therapy and plasmapheresis

Genetics = TNF inhibitors

Question 25

Progressive or abrupt muscle weakness weeks-months involving proximal and symmetric muscle groups of upper + lower extremities and neck Difficulty climbing stairs, rising from chair, generally preceding upper NO facial or ocular weakness Pain and tenderness of muscles Dysphagia Late = muscle atrophy and contractures 50-60 years, women>men

Answer 25

polymyositis

Question 26

LABS: serum muscle enzymes CK and aldolase are elevated LFTs elevated ESR and CRP, Hbg normal ANA elevated Autoantibodies present – anti-Jo-1 anti-Mi-2 anti-MDA5 anti-155/140 (cancer) Anti-140 (juvenile) anti-SAE (cancer) Biopsy of clinically involved muscle MRI helps to detect early involvement, EMG abnormalities

Answer 26

polymyositis

Question 27

How do you treat polymyositis?

Answer 27

Prednisone, often long term Monitor muscle strength and muscle enzymes, immunosuppressive drugs may reduce need for steroids (methotrexate, azathioprine, mycophenolate) Refer! Admit if signs of rhabdomyolysis, dysphagia, respiratory insufficiency

Question 28

polymyositis is infiltration of

Answer 28

endomysium

Question 29

polymyositis is a type of

Answer 29

idiopathic inflammatory myopathy

Question 30

Fever, anorexia, malaise, weight loss, skin lesions, fatigue “Butterfly” malar rash Panniculitis, discoid lupus, fingertip lesions, periungual erythema, nail fold infarcts and splinter hemorrhages Alopecia Lesions of mucous membrane lesions, Raynaud phenomenon Joint symptoms as earliest manifestation → swan neck deformity EYES: conjunctivitis, photophobia, transient/permanent blindness, blurry vision LUNGS: pleurisy, pleural effusion, pneumonia, pneumonitis is frequent HEART: HF, arrhythmia, endocarditis, pericarditis, mitral regurgitation Mesenteric vasculitis NEUROLOGIC: psychosis, cognitive impairment, seizures, peripheral/cranial neuropathies, stroke Glomerulonephritis and interstitial nephritis (kidney involvement!)

Answer 30

lupus

Question 31

common in women, after menarche and before menopause Black > white Genetically passed through maternal line Sunlight exposure Increased estrogen (reproductive age)

Answer 31

lupus

Question 32

Inflammatory autoimmune disorder - autoantibodies to nuclear antigens Suspect in any patient with multisystem disease and + ANA test (rule out drug induced!) Can be induced by hydralazine (if taken >1 year), isoniazid, sulfasalazine, quinidine, minocycline, procainamide – discontinue agent!

Answer 32

lupus

Question 33

What drugs can cause lupus

Answer 33

Can be induced by hydralazine (if taken >1 year), isoniazid, sulfasalazine, quinidine, minocycline, procainamide – discontinue agent!

Question 34

PE: cotton wool spots on retina LABS: ANA + – initial screening TOC anti-Sm antibodies (specific) Anti-double-stranded DNA antibody levels = disease activity/response to treatment SS-A/Ro, SS-B/La, ribonucleoprotein (RNP) phospholipid Lupus nephritis → abnormal urinary sediment (urinary RBCs, w or w/o casts, proteinuria frequent) Flare = high ESR Entry criteria: + ANA If yes → additive criteria, need >/= 10 points

Answer 34

lupus

Question 35

SOAP BRAIN MD

Answer 35

Serositis Oral or nasal ulcers Arthritis in >2 joints Photosensitivity Blood disorders Renal involvement Ana + Immunologic Neurologic symptoms Malar rash Discoid rash

Question 36

How do you treat lupus

Answer 36

Avoid sun exposure, use sunscreen Tailor to specific patient and severity! Skin lesions = topical steroids Joint pain = rest and NSAIDs Hydroxychloroquine for initial therapy of rash and joint symptoms Steroids for controlling complications (acute exacerbations), more severe Immunosuppression with mycophenolate, azathioprine, methotrexate Biologics Flu vaccine annually and pneumococcal vaccine every 5 years Refer Admit if rapidly progressing, severe complications, infection

Question 37

Initial: excessive vasoconstriction with digital pallor or cyanosis Recovery: vasodilation with hyperemia and rubor, intense throbbing, paresthesia, pain, slight swelling Fingers but can affect toes, nose, ears Asymptomatic between attacks Numbness, stiffness, diminished sensation, aching pain Young women 15-30y Rheumatic disease

Answer 37

Raynaud syndrome

Question 38

Paroxysmal digital ischemia, exaggerated response of digital arterioles to cold or emotional stress Scleroderma can cause secondary RP with digital pitting, ulceration, gangrene (CREST syndrome)

Answer 38

Raynaud syndrome

Question 39

How do you treat Raynaud syndrome

Answer 39

Keep warm: wearing warming clothing will help prevent vasospasms Protect hands from injury, wounds heal slowly and increase infection Lubricating lotion Stop smoking Avoid sympathomimetic drugs (decongestants, diet pills, amphetamines) Calcium channel blockers (Slow-release nifedipine, amlodipine) ARBs, sympatholytics, topical nitrates, PDE-V inhibits, SSRIs IV prostacyclin if threat of digital loss Sympathectomy if frequent and severe

Question 40

Initial sign = Raynaud phenomenon and may precede others by YEARS Polyarthralgia, weight loss, myalgias, malaise (diffuse) Non-pitting subcutaneous edema associated w/ pruritus becoming shiny, skin becoming hardened involving trunk and proximal extremities Telangiectasias, pigmentation, depigmentation Ulceration of fingertips and calcification Dysphagia and reflux symptoms GI = fibrosis and decreased motility, diarrhea/constipation, diverticuli Pulmonary fibrosis and vascular disease Pericarditis, heart block, myocardial fibrosis, RHF (pulm HTN) Renal crisis 30-50y women>men

Answer 40

scleroderma

Question 41

Diffuse fibrosis of skin and internal organs – chronic with unknown cause Limited (80%) confined to face, neck (more risk for ischemia), distal extremities or diffuse forms Limited = CREST syndrome Calcinosis cutis-calcium salts deposited in skin and tissue Raynaud phenomenon Esophageal motility disorder Sclerodactyly Telangiectasia Either can have small/large bowel hypomotility with constipation/diarrhea, malabsorption, obstruction, distention Diffuse = trunk involved with proximal extremities, tendon friction rubs over forearm/shins (leathery, crepitus feel) w/ lungs, heart, GI tract, kidneys

Answer 41

scleroderma

Question 42

CREST indicates

Answer 42

scleroderma

Question 43

CREST means

Answer 43

Calcinosis cutis Raynaud phenomenon Esophageal motility disorder Sclerodactyly Telangiectasia

Question 44

LABS: mild anemia, renal crisis (microangiopathic hemolytic anemia, proteinuria) ANA Anti-SCL-70 = diffuse Anticentromere antibodies anti-RNA polymerase III antibodies Consider clinically: skin thickening involving fingers, skin manifestations, Raynaud’s, SS-related antibodies

Answer 44

scleroderma

Question 45

How do you treat scleroderma?

Answer 45

Calcinosis can be observed, but topical sodium thiosulfate, colchicine, minocycline can help Symptomatic + supportive Raynaud's = CCB Esophageal symptoms = liquid/crushed meds only, avoiding late night meals, + PPI GI bacteria: abx HTN renal crisis = captopril or other ACE-I Interstitial lung disease = mycophenolate mofetil or cyclophosphamide Methotrexate for skin disease Digital ulceration = bosentan Pulmonary HTN = sildenafil or prostaglandins Immunoablative therapy