CASE REPORT

A case of proteinase 3-antineutrophil cytoplasmic antibody-positive Sjögren's syndrome complicated with interstitial nephritis

Authors

Kumiko Shimoyama¹, Noriyoshi Ogawa¹, Yoshiaki Dei¹, Daisuke Suzuki¹, Miwako Saito¹ and Hideharu Hayashi¹

1. Third Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu 431-3192, Japan

Received:

22 December 2006

Accepted:

18 June 2007

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Abstract

A 65-year-old woman who had fever was admitted. Laboratory data showed renal tubular dysfunction, elevated C-reactive protein, soluble interleukin-2 receptor (sIL-2R), and IgG. Her blood showed proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) as well as antinuclear antibody and anti-Ro/SS-A antibody. Salivary gland and renal biopsy showed inflammatory infiltration of lymphocytes. A diagnosis of Sjogren's syndrome (SS) and interstitial nephritis was made. β2-microglobulin, sIL-2R, IgG, and PR3-ANCA were decreased in response to medium-dose oral prednisolone. Antineutrophil cytoplasmic antibody could be a new marker for extraglandular features of SS. It would be beneficial for SS patients who have positive ANCA to investigate extraglandular lesions such as interstitial nephritis.

Key words

Extraglandular lesions - Interstitial nephritis - Proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) - Sjögren's syndrome