Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy)

Abstract

Purpose of review: Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, was first documented in 1997 as a scleroderma-like fibrosing entity of the skin in association with renal insufficiency. Rheumatologists, along with other specialists, may be the first to encounter these patients; and both a familiarity with the disorder and vigilance for its cardinal features is in order. This review provides an update and highlights recent theories, achievements and ongoing research in understanding this emerging and enigmatic disorder.

Recent findings: Clinical reports support the evidence of nephrogenic systemic fibrosis as a systemic disease and emphasize an increase in its recognition in the United States, Europe and Asia. The most recent work supports a model whereby circulating fibrocytes together with fibrogenic factors lead to the evolution of this disabling and sometimes fatal disorder.

Summary: While a specific cause of nephrogenic systemic fibrosis remains to be established, the pathogenesis seems to be multifactorial, with the postulated involvement of the circulating fibrocytes. Recent published data including information from the Yale University NSF Registry has shed light on the clinical spectrum, cause, pathogenesis and treatment options. Clinical awareness of nephrogenic systemic fibrosis is still emerging and future studies are warranted to clarify its etiopathogenesis and effective therapies.