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Dual anti-neutrophil cytoplasmic antibody-related pauci-immune crescentic glomerulonephritis in a patient with Sjögren’s syndrome

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Abstract

Sjögren’s syndrome is an autoimmune disease that primarily affects exocrine glands. Renal involvement of Sjögren’s syndrome may lead to tubulointerstitial disease, whereas secondary glomerulopathies such as anti-neutrophil cytoplasmic antibody (ANCA)-related pauci-immune crescentic glomerulonephritis are rarely observed. In addition, crescent glomerulonephritis that is simultaneously positive for both myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA has never been reported in Sjögren’s syndrome. Here, we report a case of pauci-immune crescentic glomerulonephritis exhibiting positivity for both MPO- and PR3-ANCAs in a patient with primary Sjögren’s syndrome. A 71-year-old female was hospitalized for cough, blood-tinged sputum, and dyspnea two weeks after diagnosis with Sjögren’s syndrome. On admission, serum anti-nuclear antibody, anti-Ro/SS-A antibody, MPO-ANCA, and PR3-ANCA were all positive, and serum blood urea nitrogen and creatinine (Cr) levels were 42.7 and 2.9 mg/dL, respectively. On the seventh day of hospitalization, the patient’s serum Cr level was 5.7 mg/dL, indicating rapidly progressive glomerulonephritis. Renal biopsy resulted in the diagnosis of ANCA-related pauci-immune crescentic glomerulonephritis, for which intravenous methylprednisolone (7 mg/kg/day) was administered for three consecutive days, followed by combination therapy with oral prednisolone (1 mg/kg/day) and intravenous cyclophosphamide (500 mg/m2). The patient was positive in the Schirmer’s I test, and a salivary gland biopsy showed sialadenitis with lympho-plasmacytic infiltrations. On day 28 of hospitalization, the patient was discharged after amelioration of respiratory symptoms and azotemia. At 6 months after discharge, the patient continued to receive appropriate daily medications and was negative for both MPO- and PR3-ANCAs, with a slight elevation in serum Cr levels.

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Authors and Affiliations

  1. Division of Nephrology, Department of Internal Medicine, Catholic University of Daegu School of Medicine, 17-Gil 33, Duryugongwon-ro, Nam-gu, Daegu, 42472, Korea

    In Hee Lee

  2. Division of Rheumatology, Department of Internal Medicine, Catholic University of Daegu School of Medicine, Daegu, Korea

    Seong-Kyu Kim

  3. Department of Pathology, Keimyung University School of Medicine, Daegu, Korea

    Min-Kyung Kim

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  1. In Hee Lee
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Correspondence to In Hee Lee.

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In Hee Lee, Seong-Kyu Kim, and Min-Kyung Kim declares that they have no conflict of interests.

Informed consent

This study was approved by the IRB (CR-16-018-L). Written informed consent was not required from the patient for publication of this case report. A copy of the IRB exemption is available for review by the editor of this journal.

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Lee, I.H., Kim, SK. & Kim, MK. Dual anti-neutrophil cytoplasmic antibody-related pauci-immune crescentic glomerulonephritis in a patient with Sjögren’s syndrome. Rheumatol Int 36, 1327–1334 (2016). https://doi.org/10.1007/s00296-016-3520-5

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  • DOI: https://doi.org/10.1007/s00296-016-3520-5

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