Systemic sclerosis Flashcards
(16 cards)
Chemotherapeutic agents implicated in SSc
Taxanes, gemcitabine
Autoantibody in systemic sclerosis(diffuse)
Topoisomerase 1 (Scl 70) - associated with ILD Anti RNA polymerase III
Autoantibody in limited scleroderma
Anti- centromere - associated with pulmonary HTN
Autoantibody associated with renal crisis and skin manifestations,malignancy within 2-5yrs of diag
Anti-RNA Polymerase III Ab
Most common cause of death in Diffuse SSc
Pulmonary involvement
NSIP>UIP
Scleroderma renal crisis manifestations
New onset BP>150/85 or increase in >20mmHg from usual SBP
AKI stage I or >50% increase in serum Cr from baseline/absolute increase of 26.5
Usually in early diffuse SSc
Scleroderma renal crisis management
ACEI/ARBs
MCTD - diagnostic features
Features of SLE,SSc,RA,myositis
Raynauds very common
Pulm HTN -MC cause of death
LAb- ANA, ENA-U1RNP, SSA often +
Puffy hands,RNP without dsDNA = MCTD
Role of nail fold capillaroscopy
Used to diagnose systemic sclerosis
Antibody associated with highest risk of renal crisis in systemic sclerosis
Anti-RNA polymerase3 ab
Early internal organ involvement in which systemic sclerosis
Diffuse type
Antibody associated with pulmonary HTN
Anti- U3 RNP
Most common feature of limited scleroderma
Telengectasia
Hallmark feature of diffuse scleroderma
Calcinosis
Most common histology of ILD in SS
NSIP
Most useful agent in scleroderma associated lung disease
Cyclophosphamide
