Systemic rheum/muscular dz

Question 1

Polymyalgia rheumatica

Answer 1

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

Question 2

Polymyositis

Answer 2

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

10% risk of malignancy

Question 3

Dermatomyositis

Answer 3

Muscle inflammation

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

Anti-Jo1+

15% risk malignancy

Question 4

Diagnostic criteria of SLE

Answer 4

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

Question 5

SLE presentation

Answer 5

young, middle age women, black

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

Question 6

Tx of SLE

Answer 6

steroids - glucocorticoids
NSAIDs
Hydroxychloroquine

Advanced w/ nephritis - cyclophosphamide

Question 7

Lupus nephritis

Answer 7

Leading cause of death in lupus patients

wire loop lesions, deposition of immunocompleses
“Wire lupus”

Question 8

Why does SLE increase risk of severe infections?

Answer 8

decreased C3 and C4 –> immunodeficiency

Question 9

Drug induced lupus

Answer 9

anti-histone Ab

Drugs: "SHIPP"
Sulfonamides
Hydrazine
Isoniazid
Phenytoin
Procainamide

Question 10

Scleroderma - diffuse

Answer 10

Anti-DNA topoisomerase I Ab - Anti-Scl70

widespread
rapidly progress
viscera involved early

Question 11

Scleroderma

Answer 11

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

Question 12

CREST syndrome

Answer 12

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

Question 13

Sjogren syndrome triad

Answer 13

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

Question 14

Sjogrens incidence, assoc sx, risk factors

Answer 14

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

Question 15

Fibromyalgia

Answer 15

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

Question 16

Conditions with ANA+

Answer 16

sjogrens
scleroderma 
polymyositis/dermatomyositis
RA
JIA
Mixed connective tissue dz

Question 17

Becker’s muscular dystrophy

Answer 17

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

Question 18

Duchenne muscular dystrophy

Answer 18

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