systemic MSK diseases Flashcards
what causes:
african american female of reproductive to middle age with:
rash on face (worse in sun) + joint pain + nonspecific findings: fatigue, fever, weight loss, LAD, splenomegaly, Raynaud phenomenon (vasospasm of finger due to exposure to cold), edema
systemic lupus erythematosus
diagnostic criteria for systemic lupus erythematosus
4/11 (have at least four at any time during course of disease, don’t have to be at same time)
–skin disorders:
malar rash
discoid rash: raised erythematous base with scaling (face, scalp, ears), atrophic scar (SEAL)
photosensitivity
PAINLESS oral ulcers
–inflammatory disorders:
arthritis: non-destructive in at least 2 joints (vs OA - destructive)
serositis: pleuritis or pericarditis
+ ANA (very high, 1:160)
–organ system disorders:
renal: proteinuria, cellular casts, lupus nephritis
neuro: seizures, psychosis
heme: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
immune: +antiphospholipid Ab, anti-dsDNA, anti-Smith (specific), false-positive VDRL
PAINLESS oral ulcers
SLE (a diagnostic criteria)
wart-like sterile vegetations on both sides of valve can be found in
SLE
called Libman-Sacks endocarditis (LSE in SLE)
“wire loops” thickening of capillary walls with deposits of immune complexes is found in
SLE
called lupus nephritis: type III hypersensitivity
“wire lupus” in lupus
can progress to renal failure → death
helpful for diagnosis of renal disease in lupus
anti-dsDNA antibodies - specific for renal disease in lupus
helpful for screening of lupus
ANA - antinuclear antibodies
sensitive but not specific: good screening test
Negative = no lupus
Positive = may have lupus
conditions that can have a + ANA
Sjogren syndrome Scleroderma Polymyositis + dermatomyositis RA Juvenile Idiopathic Arthritis Mixed CT disease
helpful for screening of drug-induced lupus
Antihistone antibody - sensitive for drug-induced lupus
complication of + antiphospholipid = anticardiolipin antibody in lupus
antibodies cause hypercoguable disorders (prolong PTT, ↑ risk arteriovenous thromboembolism)
complication of ↓C3, C4 in lupus
↑risk of serious infections
treatment for lupus
NSAIDs
steroids
hydroxychlorquine
advanced (lupus nephritis): cyclophosphamide
female with excessive fibrosis + collagen deposition throughout body → interfere with tissue function
skin manifestations: shiny (no wrinkles) + tight skin, ulcers on fingers, can’t extend fingers/grip
scleroderma (systemic sclerosis)
type of scleroderma with:
widespread skin involvement
rapid progression
early visceral involvement
diffuse scleroderma
associated with anti-Scl-70 antibody = anti-DNA topoisomerase I antibody
diffuse scleroderma
type of scleroderma with: skin manifestations: fingers + face only benign clinical course, progresses less rapidly less visceral involvement and CREST syndrome Calcinosis (subepithelial calcium deposits) Raynaud phenomenon Esophageal dysmotility Sclerodactyly (scarring of fingers) Telangiectasia
limited scleroderma
associated with anti-centromere antibody
limited scleroderma (Crest syndrome = antiCentromere antibody)
female, 40-60 yo with: xerophthalmia: dry, conjunctivitis, sensation of sand in eyes xerostomia arthritis bilateral parotid enlargement labs: antinuclear autoantibodies: SS-A (anti-Ro) and/or SS-B (anti-La) \+ RF possible
Sjogren syndrome: autoimmune destruction of exocrine glands
complication of sjogren syndrome
dental caries
↑ risk mucosa-associated lymphoid tissue (MALT lymphoma) - a B cell lymphoma - unilateral parotid enlargement
what are the following symptoms called dry eyes dry mouth nasal dryness vaginal dryness chronic bronchitis reflux esophagitis
sicca symptoms
X-linked FRAMESHIFT mutation that cause deletion of dystrophin gene → no anchoring of muscle fibers → accelerated muscle breakdown →↑ CK + aldolase
Duchenne MD = Deleted Dystrophin
X-linked POINT mutation that causes mutation in dystrophin gene
becker MD
duchenne MD
positive gower maneuver
duchenne MD
teenager or young adult with proximal muscle weakness
becker MD (less severe than duchenne)
50-70 yo female with JOINT pain + stiffness in shoulders + hips (proximal joints) + fever, weight loss, malaise
no muscular weakness or muscle pain
↑ESR
normal CK + aldolase
polymyalgia rheumatica (not a muscle problem)
associated with temporal arteritis
polymyalgia rheumatica
treatment of polymyalgia rheumatica
low dose corticosteroids
proximal muscle weakness is due to a
muscle disease
distal muscle weakness is due to a
neuro disease
progressive symmetric proximal muscle weakness (shoulder, pelvic girdle) due to muscle inflammation
muscle bx = muscle inflammation
caused by CD8+ T cell injury to muscle
↑CK + aldolase
+ anti-Jo-1, aldolase (released from inflamed muscle)
↑risk of malignancy
polymyositis
progressive symmetric proximal MUSCLE weakness (shoulder, pelvic girdle) due to muscle inflammation AND
malar rash
Gottron papules: red, scaly, knuckles, knee, elbows
heliotrope rash (periorbital)
“shawl and face” rash
“mechanics hands”: rough, cracked fingers + hands
“v sign” anterior chest
↑CK, anti-Jo-1, aldolase (released from inflamed muscle)
↑risk of malignancy
dermatomyositis
chronic generalized pain (excess muscular tenderness at trigger points with pressure in 11 of 18 spots), fatigue, sleep disturbances, headache, cognitive difficulty, mood disturbances (some have depression, anxiety)
fibromyalgia
treatment of fibromyalgia
fda: pregabalin (anticonvulsant - tx for neuropathic pain), milnacipran (SNRI)
traditional: amitryptiline (TCA), low dose NSAID/acetaminophen, fluoxetine (SNRI)
most common cause of death in SLE
lupus nephritis
