Abstract
Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). It is associated with increased vascular permeability, activation of coagulation cascade, and renin secretion, which may lead to the acute renal failure typically associated with accelerated hypertension. The histologic picture of SRC is that of a thrombotic microangiopathy process with prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sclerosis. Renal biopsies play an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in SSc patients, helping to predict the clinical outcome and optimizing patient management. Kidney transplantation may be the only treatment option available for a subset of SRC patients who develop end-stage renal failure despite aggressive angiotensin-converting enzyme inhibitor therapy. However, the posttransplant outcome for SSc patients is currently suboptimal compared to the general renal transplant population.
Highlights
Systemic sclerosis (SSc) is a multisystem autoimmune disorder that can manifest as either the diffuse cutaneous or the limited cutaneous variant, distinguished by the degree and the extent of cutaneous sclerosis [1]
Most frequently seen in dcSSc, Scleroderma renal crisis (SRC) can occur in patients with lcSSc [2, 3] and rarely in patients with no significant dermal sclerosis termed systemic sclerosis sine scleroderma [4]
Overexpression of endothelin-1, a protein that plays a role in blood vessel constriction, and its receptor endothelin-B has been demonstrated in the small vessels of two SRC patients [12]
Summary
Systemic sclerosis (SSc) is a multisystem autoimmune disorder that can manifest as either the diffuse cutaneous (dc) or the limited cutaneous (lc) variant, distinguished by the degree and the extent of cutaneous sclerosis [1]. The etiology of SCR remains incompletely understood, with most models of pathogenesis suggesting an initial trigger of vascular endothelial injury. Antiendothelial cell antibodies, which are capable of inducing endothelial cell apoptosis [10] have been detected in up to 85% of SSc patients [11]. Subsequent to the potential endothelial triggering injury, the proposed cascade of histologic alterations is initiated by rapid increase in endothelial permeability and intimal edema. This places the subendothelial connective tissue in direct contact with circulating blood elements activating the coagulation cascade and vascular thrombosis. A milder form of vascular pathology, manifested usually as fibrointimal thickening can often be observed in SSc patients without SRC [14]. A detailed histologic assessment can confirm the clinical diagnosis and help exclude potential overlapping or superimposed etiologies
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