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Abstract

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Article in Japanese

Case Report

A case of Sjögren syndrome complicated by ANCA-related diffuse alveolar hemorrhage and antiphospholipid syndrome

Kyoko Yagyua  Kazuya Kojimaa  Takako Okaa  Noritaka Seikea  Haruhiko Matsushitaa  Kazuto Hiratab 

aDepartment of Respiratory Medicine, Izumi Municipal Hospital
bDepartment of Respiratory Medicine, Osaka City University Graduate School and Faculty of Medicine

ABSTRACT

A 75-year-old woman with Sjögren's syndrome was referred to our hospital because of an abnormal chest radiograph. A bronchofiberscopic examination revealed diffuse alveolar hemorrhage. We treated it as antineutrophil cytoplasmic antibody (ANCA)-related diffuse alveolar hemorrhage because of positive myeloperoxidase (MPO)-ANCA. Blood sputum disappeared when combination therapy of corticosteroid and cyclophosphamide was performed. However, the D-dimer value was high, and the estimated pulmonary artery pressure was increased. On a contrast-enhanced CT examination, a left pulmonary artery thrombus was observed. Anticardiolipin antibody was confirmed to be positive, and the patient was diagnosed with antiphospholipid antibody syndrome. No reports of a simultaneous occurrence of ANCA-related alveolar hemorrhage or antiphospholipid antibody syndrome follow-up with Sjögren's syndrome have been made.

KEYWORDS

Sjögren's syndrome  ANCA-associated pulmonary hemorrhage  Antiphospholipid syndrome 

Received 17 Feb 2017 / Accepted 23 Jun 2017

AJRS, 6(5): 383-388, 2017

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