B6-023 Dermatomyositis/Scleroderma Flashcards
proximal muscle weakness with skin involvement
dermatomyositis
proximal muscle weakness without skin involvement
polymyositis
anti-Jo 1 antibody
polymyositis
occurs in older adults with distal extremity involvement
responds poorly to treatment
inclusion body myositis
have 1 or all of the following findings:
myositis, ILD, raynaud’s, inflammatory arthropathy, mechanic’s hand
antisynthetase syndrome (Anti-Jo-1)
early complement deposition with vascular involvement
dermatomyositis
B cells and CD 4+ T cells in perifasicular and perivascular infiltration
dermatomyositis
endomysial CD8+ T cells around non-necrotic fibers
polymyositis/IBM
vacuoles with rim of eosinophilic granules
IBM
used to treat dysphagia caused by dermatomyositis
IVIG
1st line steroid sparing agents used with high dose steroid for dermatomyositis [2]
methotrexate
azathioprine
used for ILD due to dermatomyositis or resistant dermatomyositis [2]
rituximab
mycophenolate
treatment of dermatomyositis
- prednisone- high dose, taper
- immunosuppressant
- long term management and reassessment
what antibodies have high risk of malignancy associated with dermatomyositis?
anti-p155/140
anti-MJ
only skin and soft tissue involvement
no Raynaud’s
kids > adults
localized scleroderma
puffiness of hands
Raynauds
sclerodactly
salt and pepper vitiligo
reduced oral aperture
scleroderma
triad of pathophysiology of scleroderma
- autoantibodies
- obliterative vasculopathy
- fibrosis
main effector cytokine that drives myofibroblast differentiation
TGF-b
**causes fibrosis
late vascular manifestation of limited cutaneous scleroderma
pulmonary hypertension
all manifestations of diffuse cutaneous scleroderma occur […] in the timeline
early
antibody strongly associated with limited cutaneous systemic sclerosis
Anti-centromere Ab
CREST syndrome patients have
limited cutaneous systemic sclerosis
increased risk of isolated PAH
limited cutaneous systemic sclerosis
what antibody is associated with diffuse cutaneous scleroderma?
anti-topoisomerase-1 Ab (Scl-70)
what type of scleroderma is more likely to have severe interstitial lung disease?
diffuse cutaneous scleroderma
what antibody is associated with skin thickening that might predate Raynaud’s?
anti-RNA polymerase III