Renal involvement in systemic sclerosis.

Abstract

Systemic sclerosis is a complex connective tissue disease characterized by immune dysregulation, widespread vasculopathy, and progressive fibrosis affecting the skin and internal organs. Among its visceral complications, renal involvement, manifesting as scleroderma renal crisis, remains organ and life-threatening if not detected. Although its mortality has markedly declined with the life-saving introduction of angiotensin-converting enzyme inhibitors, renal complications remain a major determinant of morbidity, mortality, and long-term health outcomes. Beyond scleroderma renal crisis, a spectrum of renal abnormalities, and secondary renal injury from hypertension or therapeutic agents, contribute to the overall disease burden. This chapter review synthesizes evidence to provide an in-depth analysis of renal involvement in systemic sclerosis. The epidemiology, pathophysiology, clinical presentation, diagnostic approaches, management strategies, and emerging research directions are reviewed, along with developments in precision medicine, single-cell sequencing, molecular profiling, and complement-directed therapies are also discussed, highlighting gaps and future directions to optimize positive outcomes.