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

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Abstract

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 Sjögren’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.

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Abbreviations

HUS:

Hemolytic uremic syndrome

pSS:

Primary Sjögren’s syndrome

SLE:

Systemic lupus erythematosus

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Authors and Affiliations

  1. Division of Allergy, Immunology and Rheumatology, Chi Mei Medical Center, Tainan, Taiwan

    Hung-An Chen

  2. Division of Allergy, Immunology and Rheumatology, Buddhist Tzu Chi General Hospital, Taipei Branch, Taipei, Taiwan

    Chun-Hsiung Chen

  3. Division of Allergy, Immunology and Rheumatology, Chang Bing Show Chwan Memorial Hospital, No. 6, Lugong Rd, Lukang Zhen, Changhua County, 505, Taiwan

    He-Hsiung Cheng

Authors
  1. Hung-An Chen
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  2. Chun-Hsiung Chen
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  3. He-Hsiung Cheng
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Correspondence to He-Hsiung Cheng.

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Chen, HA., Chen, CH. & Cheng, HH. Hemolytic uremic syndrome and pericarditis as early manifestations of primary Sjögren’s syndrome. Clin Rheumatol 28 (Suppl 1), 43–46 (2009). https://doi.org/10.1007/s10067-009-1121-3

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  • DOI: https://doi.org/10.1007/s10067-009-1121-3

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