Nintedanib and intensive immunosuppressive therapy to treat rapidly progressive interstitial lung disease presenting anti-ARS antibodies

Toyoshi Yanagihara; Kunihiro Suzuki; Ayaka Egashira; Naruhiko Ogo; Tatsuma Asoh; Tsukasa Nara; Kensaku Takatsuki; Seiji Yoshizawa; Sy Giin Chong; Naoki Hamada; Takashige Maeyama

doi:10.1016/j.rmcr.2020.101272

Nintedanib and intensive immunosuppressive therapy to treat rapidly progressive interstitial lung disease presenting anti-ARS antibodies

Respir Med Case Rep. 2020 Oct 28:31:101272. doi: 10.1016/j.rmcr.2020.101272. eCollection 2020.

Authors

Affiliations

¹ Department of Respiratory Medicine, And Department of Rheumatology, Hamanomachi Hospital, Fukuoka, 810-8539, Japan.
² Research Institute for Diseases of the Chest, Kyushu University, Fukuoka, 812-8582, Japan.
³ Department of Rheumatology, Hamanomachi Hospital, Fukuoka, 810-8539, Japan.
⁴ Firestone Institute for Respiratory Health, Research Institute at St Joseph's Healthcare, Department of Medicine, McMaster University, Hamilton, ON, Canada.

Abstract

We describe a case of fulminant onset, rapidly progressive-interstitial lung disease (RP-ILD) with anti-ARS antibodies (anti-PL-7). The patient was successfully treated with nintedanib in addition to intensive immunosuppressive therapies, including intravenous cyclophosphamide. Nintedanib has just been approved for treatment of progressive fibrosing ILD, but to date, no reports of RP-ILD treated with nintedanib have been published. This case report may advance discussions regarding the use and timing of nintedanib in treating RP-ILD.

Keywords: Anti-ARS antibody; Clinically amyopathic dermatomyositis; Nintedanib; Progressive fibrosing interstitial lung disease; Rapidly progressive interstitial lung disease.

Publication types

Case Reports