ICM - Rheum 5 Flashcards
this inflammatory myopathy is due to problems with the humoral immune system
dermatomyositis
these inflammatory myopathies are due to cell-mediated response
polymyositis and inclusion-body
rash seen over knuckles/elbows in dermatomyositis
Gottron’s papules
this inflammatory myopathy is at increased risk for malignancies
dermatomyositis
inflammatory infiltrate seen in dermatomyositis biopsy
perivascular and perimysial
inflammatory infiltrate seen in polymyositis and inclusion-body myositis biopsy
endomysial
treatment for inflammatory myopathies
corticosteroids, immunosuppresion (methotrexate, cyclophosphamide)
muscle most affected in inflammatory myopathies (symmetrical proximal muscle weakness)
neck flexors, shoulder girdle, pelvic girdle
enzymes that are elevated in inflammatory myopathies
CPK, LDH, aldolase, AST, ALT
inflammation seen in inflammatory myopathies
lymphocytes
pathology: perivascular and perifascicular lymphocytic infiltrate, immune complex deposition in vessels, perifascicular atrophy and fibrosis
dermatomyositis
pathology: cellular infiltrate within fascicle, inflammatory cells invade individual muscle fibers
polymyositis
pathology: perivascular exudates rare, characteristic intracellular lined vacuoles; intracytoplasmic or intranuclear tubular or filamentous inclusions
inclusion body myositis
MCTD is overlap of these 3 conditions –> high ANA and anti-RNP
polymyositis, scleroderma, SLE
myositis specific Ab –> association with interstitial lung disease, Raynaud’s, arthritis, mechanics hands
anti-histidyl tRNA synthetase (anti-Jo1)
myositis specific Ab –> rapid onset of classical dermatomyositis
anti Mi2
pathognomic for dermatomyositis or scleroderma
nail fold capillary changes
5 diagnostic criteria for inflammatory myopathies
proximal muscle weakness, elevation serum muscle enzymes, EMG, biopsy, rash
possible pulmonary problems associated with inflammation myopathies
interstitial lung disease, infection
peak incidence (time) for inflammatory myopathy related malignancy
within 2 years
top 3 malignancies related to inflammatory myopathies
breast, lung, ovary
mainstay of therapy for inflammatory myopathies
corticosteroids
patient population affected mostly by scleroderma
female, 30-40 yo
DDX for Raynaud’s phenomenon
scleroderma, SLE, MCTD, vasculitis, medications
most common cause of death related to scleroderma
interstitial fibrosis/pulmonary HTN