PROGRESSIVE SYSTEMIC-SCLEROSIS WITH RENAL CRISIS

Progressive systemic sclerosis is a connective tissue disease. Its maj or hallmarks are uncontrolled and irreversible proliferation of connec tive tissue. vascular lesions as well as autoimmunologic factors. PSS is clinically devided into 2 different courses. PSS type ZI is charact erized by a rapid progressive course where visceral participation occu rs relatively early. Autopsy studies suggest that 60 - 80% of patients with scleroderma have renal involvement. Most patients present in the cooler months, suggesting a contributory, role of cold-induced vasosp asm which might exacerbate renal ischemia and hypersecretion of renin. Scleroderma renal crisis develops in 10 - 15% of cases. The renal cri sis is associated with abrupt onset of marked hypertension and renal f ailure. The primary histologic changes in the kidney affect the interl obular arteries, the glomeruli, and the interstitium. Therapy is most likely to be effective if initiated early before marked and often irre versible morphologic alterations have occurred. Because of that, follo wing and monitoring particularly those patients: with rapid skin invol vement during the first 5 years of scleroderma is recommended. Treatme nt of hypertension is the mainstay of therapy in scleroderma renal cri sis. An angiotensin converting enzyme (ACE) inhibitor is the agent of choice. When compared to other antihypertensive agents, ACE inhibitors have increased antihypertensive efficacy, appear to be associated wit h improved survival, and lead to better preservation of renal function . Hermodialysis may become necessary. In some patients considerable re covery of renal function, which may permit the discontinuation of dial ysis, is observed, Therefore, any decisions regarding renal transplant ation should not be made during or shortly after the acute episode, Es sential sentence. Renal crisis develops in 10 - 20% of cases with rapi d progressive course of PSS. Aggressive treatment of hypertension with an ACE inhibitor as the agent of choice can stabilize anal function a nd improve survival.