Systemic sclerosis Flashcards
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
