All are true about scleroderma crisis except?

Scleroderma crisis occurs in 12% of cases with systemic sclerosis. It is characterised by accelerated hypeension and a rapid decline in kidney function. Histopathological examination shows arcuate aery intimal narrowing and medial proliferation. This is characteristically called onion skinning. Anti-U3-RNP may identify the young patients at risk for scleroderma crisis. Anti-centromere is a negative predictor for scleroderma crisis. Maximum moality in scleroderma occurs due to Pulmonary aery hypeension and pulmonary fibrosis. NOTE : =In Scleroderma renal crisis - Prompt treatment with an ACE inhibitorcan reverse acute renal failure. =ACE inhibitors should be used even if the patient requires dialysis suppo. =MC cause of death in Systemic Sclerosis : Pulmonary Aerial Hypeension