Diffuse Cutaneous Systemic Sclerosis With Normotensive Scleroderma Renal Crisis and Myopericarditis: A Case Report

Résumé fourni par la source

Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis and multi-organ dysfunction, primarily affecting the heart, lungs, and kidneys. Scleroderma renal crisis (SRC) can present as hypertensive or normotensive, with the latter being more challenging to diagnose due to the absence of hypertension at onset. Normotensive SRC carries a worse prognosis, with an increased risk of renal failure and a poor response to treatment. The presence of cardiac complications, such as myopericarditis, further exacerbates the clinical course, creating significant management challenges. Moreover, hypotension in normotensive SRC complicates therapeutic interventions, particularly the use of angiotensin-converting enzyme (ACE) inhibitors. This case report highlights a patient with diffuse cutaneous systemic sclerosis (dcSSc) who presented with normotensive SRC complicated by myopericarditis, resulting in acute renal and heart failure. It underscores the need for early recognition of this rare form of renal crisis, especially when accompanied by cardiac complications, given its atypical presentation without significant hypertension at onset. The report emphasizes the critical importance of identifying risk factors and addressing the challenges of managing normotensive SRC to improve patient outcomes.

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