uncommon rheum diseases Flashcards
describe Raynaud’s phenomenon (vasospasm)
blanching and pain in fingers in cold, due to bad circulation/vessel shutdown. fingers turn white then purple
what are periungual changes?
dilated capillary loops in cuticles
what feature can be used to score the extent of scleroderma?
firm induration on skin of hand
how does firm induration differ from dactylitis?
involves all fingers, doesn’t fluctuate
what are the clinical features of scleroderma?
- Raynaud’s
- periungual changes
- firm induration
- GI involvement
- lung involvement
- anti-scl-70+/anti-centromere
what are the 3 phases of skin fibrosis in scleroderma?
- early inflamed phase
- tethered fibrotic phase
- late softening phase
CREST syndrome is typical of (limited/diffuse) scleroderma
limited
what is the major morbidity in limited scleroderma?
vasculopathy (painful digital ulcers, pulmonary HTN)
which Ab is associated with diffuse scleroderma?
anti-scl-70
what is a critical feature of diffuse scleroderma?
early onset pulmonary fibrosis (50%)
the most common COD in scleroderma used to be ___ but is now___
renal failure
pulmonary HTN
what lead to the decreased COD due to renal failure in scleroderma?
ACE inhibitors
limited scleroderma has a (better/worse) prognosis than diffused
better
what are the principle therapies for scleroderma?
- vasculopathy (Ca2+ channel inhibitor, phosphodiesterase, endothelin)
- fibrogenesis (target fibroblasts and growth factors)
- abnormal immune response (immune suppressives)
when is cyclophosphamide used in scleroderma?
severe Sx, pulmonary involvement
how can SCT help in scleroderma?
improve skin (regress tightening), help gut, improve survival
doesn’t help w organ involvement
what is nintedanib and when is it used?
anti-fibrotic therapy, lung involvement in scleroderma (doesn’t reverse disease)
what are gottron’s papules?
red sclay patches over knuckles and extensor surfaces (knee and hips)
what are the clinical features of inflammatory myositis?
-chronic inflammation of striated muscles prox muscle weakness -involvement of lungs, heart, GI, joints -high CK, +anti-jo-1 on labs \+/- rash in V/shawl sign on sun exposure
the most common type of adult myositis is:
PM
what is the difference between the pathogenesis of DM and PM?
DM-immune complex, specific Ab, microangiopathy, complement activation
PM-direct T cell mediated muscle attack
what are the features of inclusion body myositis?
-M>F, onset 50s
-nonreponsive to prednisone
-asymmetrical muscle weakness
myopathic and neuropathic changes on EMG
-mononuclear cell infiltrates and amyloid vacuoles on biopsy (red-rimmed vacuoles)
which type of myositis has the best prognosis? which type has the worst?
best: DM
worst: inclusion body/PM with anti-SRP
what are the features of ANCA vasculitis?
- wrist drop (radial nerve damage)
- livedo reticularis (vessel blockage-white spots)
- palpable purpura
- bloody sputum (med emergency)
- hema/proteinuria
- +c-ANCA/PR3-ANCA
- pulmonary nodules
- mononeuritis multiplex
- granulomatosis/polyangiitis
every system is adrift
how does vasculitis happen?
vessel inflmaed due to cytokines, lumen is reduced, flow is reduced, lead to organ damage and necrosis
- vessel weakening
- vessel thinning and aneurysm
giant cell arthritis is a (small/med/large) vessel vasculitis
large
GCA happens in the ____ and can lead to____
carotid artery/aorta
blindness
targeted therapy for GCA include Abs for this interleukin:
IL6
