Vol.21 No.6

Case Report

A case of Sjögren syndrome with pulmonary amyloidosis complicating microscopic polyangiitis

Authors

Shin-ya Kawashiri1 , Mami Tamai1 , Satoshi Yamasaki1 , Hideki Nakamura1 , Tomoyuki Kakugawa2 , Noriho Sakamoto2 , Yuji Ishimatsu2 , Atsushi Kawakami1

  • Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
  • Second Department of Internal Medicine, Nagasaki University, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
Received:

8 February 2011

Accepted:

25 February 2011

Published online:

25 March 2011

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Abstract

We present the case of a 71-year-old Japanese woman with Sjögren syndrome complicated with pulmonary amyloidosis who developed microscopic polyangiitis. She was admitted because of fever, weight loss, and paresthesia of the plantae and fingers. Chest computed tomography revealed bilateral multiple nodules with calcification in the lung. Transbronchial lung biopsy specimens showed AL-type (primary) amyloid deposits. The patient also tested positive for myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA), with changes in the MPO-ANCA titer that paralleled changes in the symptoms. Nerve conduction velocity findings showed mononeuropathy multiplex. Livedo vasculitis was observed on bilateral lower legs, and skin biopsy showed leukoclastic vasculitis. We diagnosed the patient as having microscopic polyangiitis.

Key words

Sjögren syndrome - Pulmonary amyloidosis - Microscopic polyangiitis - MPO-ANCA