Systemic rheum/muscular dz Flashcards

1
Q

Polymyalgia rheumatica

A

70 yo, woman

proximal joint pain/stiffness - hips, shoulder girdle, neck

malaise, wt loss, fever

no weakness of muscles

Assoc w/ giant cell arteritis

Labs: elevated ESR, normal CK

Tx: prednisone

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2
Q

Polymyositis

A

CD8+ injury to muscle of shoulder and pelvic girdle
-muscle bx
Lab: Elevated CK and aldolase
+ANA, +anti-Jo1

10% risk of malignancy

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3
Q

Dermatomyositis

A

Muscle inflammation

Skin: malar rash, heliotrope rash, gottron’s papules, shawl sign, V sign
mechanics hands

Anti-Jo1+

15% risk malignancy

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4
Q

Diagnostic criteria of SLE

A

Need 4/11

Malar rash
discoid rash - atrophic scarring
photosensitivity
painless oral ulcers

arthritis - nonerosive - two joints
Serositis - pleuritis, pericarditis
+ANA - nonspecific

Renal: proteinuria, cellular casts
Neuro: seizures, psychosis
Heme: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
Immune: 
-Antiphopholipid Ab (aka anticardiolipin Ab) = hypercoag
-anti-dsDNA Ab - specific
-anti-Smith Ab - specific
-false positive VDRL
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5
Q

SLE presentation

A

young, middle age women, black

fatigue, wt loss, fever
LAD
Splenomegaly
Libman Sacks endocarditis - sterile vegetations, both sides of valve
Raynauds
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6
Q

Tx of SLE

A

steroids - glucocorticoids
NSAIDs
Hydroxychloroquine

Advanced w/ nephritis - cyclophosphamide

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7
Q

Lupus nephritis

A

Leading cause of death in lupus patients

wire loop lesions, deposition of immunocompleses
“Wire lupus”

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8
Q

Why does SLE increase risk of severe infections?

A

decreased C3 and C4 –> immunodeficiency

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9
Q

Drug induced lupus

A

anti-histone Ab

Drugs: "SHIPP"
Sulfonamides
Hydrazine
Isoniazid
Phenytoin
Procainamide
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10
Q

Scleroderma - diffuse

A

Anti-DNA topoisomerase I Ab - Anti-Scl70

widespread
rapidly progress
viscera involved early

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11
Q

Scleroderma

A

progressive systemic sclerosis
Fibrosis and excess collagen deposit –> skin tight, shine, no wrinkles
-can’t extend fingers/grip

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12
Q

CREST syndrome

A

Anti-centromere Ab

Limited scerloderma

Calcinoshs - subepithelial Ca2+ deposits
Raynaud's
Esophageal dysmotility
Sclerodactylyl - scarring of fingers
Telangectasias

Limited skin involvement, less visceral involvement
slower progression

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13
Q

Sjogren syndrome triad

A

Dry eyes - xerophthalmia: dryness, conjunctivitis, sensation of sand in eyes - lacrimal involvement

Dry mouth - xerostomia: dysphagia, difficulty swallowing - parotid enlargement b/l, increased dental caries

Arthritis

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14
Q

Sjogrens incidence, assoc sx, risk factors

A

women 50-60s
Anti-Ro (anti SSA)
Anti-La (anti SSB)

Dx: lip bx
increased risk of B cell lymphoma

Assoc sx: reflux esophagitis, xerotrachea - dry throat, nasal dryness, chronic bronchitis, vaginal dryness, dyspareunia

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15
Q

Fibromyalgia

A

Excess muscle tenderness in 11 of 18 trigger points

Chronic generalized pain (increased sensitivity to pain), fatigue, sleep disturbances, HA, cognitive difficulty, mood disturbances

30% depression and/or anxiety

tx:
Pharm: pregabalin (neuropathic pain), milnacipran (SNRI)
-amitriptyiline, low dose analgesic (APAP, NSAIDs), fluoxetine

Non pharm: reassurance, exercise, stretching, sleep, relaxation techniques, stress reduction

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16
Q

Conditions with ANA+

A
sjogrens
scleroderma 
polymyositis/dermatomyositis
RA
JIA
Mixed connective tissue dz
17
Q

Becker’s muscular dystrophy

A

in frame mutation (del) of dystrophin
less severe - walk until 15
later onset - adolescent/early adulthood

18
Q

Duchenne muscular dystrophy

A

sx start less than 5 yo, wheelchair bound by 12
X linked frame shift deletion of dystrophin gene

accelerated muscle breakdown - increased CK

weak pelvic girdle m, pseudo hypertrophy of calf - fibrofatty replacement
Gower’s maneuver