Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918

This article has now been updated. Please use the final version.

Immunosuppressive Treatment for an anti-U1 Ribonucleoprotein Antibody-positive Patient with Pulmonary Arterial Hypertension: A Case Report
Kazuya MatsumotoYoshia MiyawakiTakayuki KatsuyamaTakato NakadoiKenta ShidaharaKei HiroseShoichi NawachiYosuke AsanoYu KatayamaEri KatsuyamaMariko Takano-NarazakiYoshinori MatsumotoAtsushi MoriSatoshi AkagiKen-Ei SadaJun Wada
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JOURNAL OPEN ACCESS Advance online publication

Article ID: 1407-22

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Abstract

A 34-year-old woman with pulmonary arterial hypertension (PAH) was admitted to the hospital. She had been diagnosed with PAH three years earlier and treated with triple vasodilator therapy. She was positive for anti-U1 ribonucleoprotein antibodies but did not show any other symptoms associated with autoimmune diseases. Corticosteroid and cyclophosphamide therapy was administered, suspecting the involvement of immunological pathophysiology. After 3 weeks, the mean pulmonary artery pressure decreased from 50 to 38 mmHg without any change in the vasodilators. Immunosuppressive therapy was effective in this patient with PAH with an anti-U1 ribonucleoprotein-antibody-positive response and might be an option for patients with these specific features.

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© 2023 by The Japanese Society of Internal Medicine
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