title: A case of anti-aminoacyl tRNA synthetase (ARS) antibody-positive polymyositis (PM)/dermatomyositis (DM)-associated interstitial pneumonia (IP) successfully controlled with bosentan therapy.
abstract: A 72-year-old woman was admitted to our hospital and was diagnosed with interstitial pneumonia (IP) associated with amyopathic dermatomyositis (ADM). The patient experienced three acute IP exacerbations in the 7 years that followed, which were each treated and resolved with steroid pulse therapy. The patient was closely examined for respiratory failure with right heart catheterization (RHC), which demonstrated that she had a mean pulmonary artery pressure (mPAP) of 34 mmHg. The patient was thus diagnosed as having pulmonary hypertension (PH) associated with anti-synthetase syndrome (ASS) and was started on bosentan therapy, which led to improvements in mPAP as well as in subjective symptoms over time. Indeed, she had had no acute exacerbations with serum markers of IP remaining low over 6 years following initiation of bosentan therapy, suggesting that bosentan may have a role in controlling IP. In addition, she was confirmed to be anti-ARS antibody-positive after 5 years of bosentan therapy, when anti-aminoacyl tRNA synthetase (anti-ARS) antibody testing became available.
authors.affiliation: Respiratory Disease Center, Chiba Hokusoh Hospital, Nippon Medical School, Chiba, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Naito
authors.affiliation: Respiratory Disease Center, Chiba Hokusoh Hospital, Nippon Medical School, Chiba, Japan.
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Tanaka
authors.affiliation: Respiratory Disease Center, Chiba Hokusoh Hospital, Nippon Medical School, Chiba, Japan.
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Hino
authors.affiliation: Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Gemma
keywords: ADM, amyopathic dermatomyositis; ASS, anti-synthetase syndrome; Bosentan; CVD, collagen-vascular disease; Collagen-vascular disease; Endothelin antagonist; IP, interstitial pneumonia; Interstitial pneumonia; PAP, pulmonary artery pressure; PH, pulmonary hypertension; PM, polymyositis; Pulmonary hypertension; TR, tricuspid regurgitation