Hemolytic uremic syndrome and pericarditis as early manifestations of primary Sjögren's syndrome

Hemolytic uremic syndrome (HUS) consists of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Autoimmune diseases have seldom been reported to be the etiology of HUS. Primary Sjogren’s syndrome (pSS) is a systemic autoimmune disease affecting primarily the exocrine glands. Symptomatic pericarditis and pulmonary hemorrhage are rare manifestations in pSS patients. We describe the unusual case of a pSS patient with the initial presentation of HUS and pericarditis and a fatal progression of pulmonary hemorrhage. A renal biopsy established the diagnosis of HUS with histologically proven arterial thrombotic microangiopathy and glomerular and tubular ischemic necrosis. The relationships between HUS and pericarditis and pSS will be discussed in this report.