Renal scleroderma crisis. Role of sclerodermic vasculopathy in the induction of cutaneous and visceral fibrosis. Report of two cases

Although fibrosis and vasculopathy coexist in most patients with progressiv e systemic sclerosis, it is not clear if these events are the result of an unique etiologic factor or if one is consequence of the other. We report tw o cases of progressive systemic sclerosis that evolved to a renal scleroder ma crisis. A 36 years old female presented with a Sjogren syndrome and pain ful subcutaneous nodules whose biopsy showed perivascular lymphocytic infil tration perivascular thickening and normal skin. The ESR was 100 mm/h. She developed an hypertensive crisis and progressive renal failure, followed by a rapidly evolving progressive systematic sclerosis. The patient died in t he course of this crisis. A 32 yeats old female with a progressive systemic sclerosis refractory to D-penicillamine treatment, receiving cyclosporin, presented a renal scleroderma crisis, that was successfully treated, with c omplete recovery of renal function. We highlight the different evolution of these cases, probably due to an early diagnosis and a better experience in the management of this condition.