Cardiac tamponade with scleroderma renal crisis in systemic sclerosis: a case report.

Abstract

Introduction and importance: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis of internal organs and vasculopathy. It commonly involves the skin and multiple organs, including the kidneys and cardiovascular system. Cardiac tamponade in SSc is an uncommon manifestation, often occurring alongside scleroderma renal crisis (SRC).

Case presentation: A 50-year-old woman presented with sudden shortness of breath, chest pain, and reduced urine output. Diagnostic tests revealed a large pericardial effusion leading to cardiac tamponade, acute kidney injury, and microangiopathic hemolytic anemia. Clinical assessment and immunological testing confirmed the diagnosis of systemic sclerosis. The patient received pericardiocentesis, hemodialysis, and was treated with diuretics and angiotensin-converting enzyme inhibitors (ACEIs).

Discussion: Pericardial effusion is frequently seen in SSc, but it is difficult to determine whether it directly contributes to or results from SRC. Early diagnosis and the use of ACEIs have shown promising benefits in managing SRC.

Conclusion: This case underscores the rare yet critical presentation of SSc with concurrent cardiac tamponade and SRC, emphasizing the importance of early diagnosis and multidisciplinary management.