title: A case of Sjögren syndrome with pulmonary amyloidosis complicating microscopic polyangiitis.
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.
authors: Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan. shin-ya@hotmail.co.jp
authors: Shin-Ya
authors: SY
authors: Kawashiri
authors: Mami
authors: M
authors: Tamai
authors: Satoshi
authors: S
authors: Yamasaki
authors: Hideki
authors: H
authors: Nakamura
authors: Tomoyuki
authors: T
authors: Kakugawa
authors: Noriho
authors: N
authors: Sakamoto
authors: Yuji
authors: Y
authors: Ishimatsu
authors: Atsushi
authors: A
authors: Kawakami
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000276:immunology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000150:complications* / Q000276:immunology; D055953:Microscopic Polyangiitis / Q000150:complications* / Q000276:immunology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic