Severe Pericardial Effusion Heralding Scleroderma Renal Crisis: A Case Report and the Literature Review

Introduction: Both scleroderma renal crisis and severe pericardial effusion are rare and life-threatening complications of systemic sclerosis. This article describes a case of scleroderma renal crisis heralded by severe pericardial effusion. Case Presentation: The patient was a 39-year-old woman with a history of 8 years diffuse cutaneous scleroderma and extensive skin involvement who referred to Imam Khomeini Hospital Complex, Tehran, Iran, with worsening dyspnea and pleuritic chest pain. Echocardiography revealed severe pericardial effusion, which was drained by the pericardial window. Thereafter, the patient became gradually anuric and her blood pressure was uncontrolled despite maximum doses of antihypertensive agents, including captopril. The serum creatinine level was increased progressively; thus, hemodialysis started. Concomitantly, microangiopathic hemolytic anemia complicated the course of the disease, which responded to plasma exchange. Kidney biopsy revealed pieces of evidence indicating thrombotic microangiopathy. Conclusions: This case report denotes to a probable association between scleroderma the renal crisis and severe pericardial effusion. Therefore, physicians should be vigilant about the renal crisis in patients with systemic sclerosis and pericardial effusion.