Musculoskeletal - Skin - Connective Tissue_2 Flashcards
presentation of dermatomyositis?
similar to polymyositis, but also invovles malar rash, Gottron’s papules. heliotrope rash, shawl and face rash, mechanic’s hands
dermatomyositis carries ↑ risk of what?
occult malignancy
pathology in dermatomyositis?
perimysial inflammation and atrophy with CD4+ T cells
findings in polymyositis/dermatomyositis?
↑ CK • ANA (+) • anti-Jo-1 Ab (+)
treatment for polymyositis/dermatomyositis?
steroids
what is the frequency of myasthenia gravis?
most common NMJ disorder
frequency of lambert eaton myasthenic syndrome?
uncommon
pathophysiology of MG?
autoantibodies to postsynaptic AChR
pathophysiology of LEMS?
autoantibodies to presynaptic Ca++ channel →↓ ACh release
clinical presentation of MG?
ptosis • diplopia • weakness • worsens with muscle use
clinical presentation of LEMS?
proximal muscle weakness • improves with muscle use
MG associated with what?
thymoma • thymic hyperplasia
LEMS associated with what?
SCLC
what happens with AChE inhibitor administration in MG?
reversal of symptoms
what happens with AChE inhibitor administration in LEMS?
no effect
what is myositis ossificans?
metaplasia of skeletal muscle to bone following muscular trauma
myositis ossificans is most often seen where?
in upper or lower extremity
myositis ossificans may present how?
as suspicious mass at site of known trauma or as incidental finding on radiography
what happens in scleroderma (SS)?
excessive fibrosis and collagen deposition throughout the body
common manifestation of SS?
sclerosis of skin, manifesting as puffy and taut skin with absence of wrinkles • also sclerosis of renal, pulm, CV, GI systems
most likely cause of death in SS?
sclerosis of pulmonary system
epidemiology of SS?
75% female
2 major types of SS?
diffuse scleroderma • CREST syndrome
clinical features of diffuse scleroderma?
widespread skin involvement, rapid progression, early visceral involvement