Anti-centromere antibody-positive primary Sjögren's disease with exudative pleural effusion: a case report and literature review
Masashi Uchikawa, Masako Tsukamoto, Kiichi Sugito, Shinya Asatani, Masahiro Nishihara, Kumiko Akiya, Miho Oshima, Hirotake Inomata, Noboru Kitamura, Junki Kurita, Hiroyuki Hao, Hideki NakamuraAbstract
Sjögren’s disease (SjD) is a systemic autoimmune disorder with glandular and extra-glandular manifestations. Pleural effusion is a rare complication of SjD among different types of pulmonary involvement. Herein, we report a case of primary anti-centromere (ACA)-positive primary SjD with pleural effusion. A 79-year-old woman was admitted to our hospital with a 3-month history of leg edema. She was diagnosed with primary SjD based on positive Schirmer and Saxon tests, and her anti-SS-A/Ro antibody test was negative but ACA and anti-SS-B/La tests were positive. The patient’s symptoms promptly improved following prednisolone treatment. This is the first reported case of ACA-positive primary SjD complicated with pleural effusion, and the possible presence of pleural effusion should be considered in SjD, even in patients with ACA-positive primary SjD.