Slowly progressive interstitial lung disease preceding typical dermatomyositis symptoms in anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis

Taisuke Isono; Hiromi Nakajima; Kenji Takano; Yoichi Kobayashi; Yoshinori Kawabata; Yoshihiko Shimizu; Noboru Takayanagi

doi:10.1016/j.rmcr.2021.101491

Slowly progressive interstitial lung disease preceding typical dermatomyositis symptoms in anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis

Respir Med Case Rep. 2021 Jul 27:34:101491. doi: 10.1016/j.rmcr.2021.101491. eCollection 2021.

Authors

Taisuke Isono¹, Hiromi Nakajima¹, Kenji Takano¹, Yoichi Kobayashi¹, Yoshinori Kawabata², Yoshihiko Shimizu², Noboru Takayanagi¹

Affiliations

¹ Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Saitama, Japan.
² Departments of Pathology, Saitama Cardiovascular and Respiratory Center, Saitama, Japan.

Abstract

A 73-year-old woman who visited our hospital complaining of dry cough for three months was refractory to antimicrobial therapy. Chest computed tomography revealed subpleural consolidation. Specimens obtained from surgical lung biopsy revealed subpleural perilobular airspace organization and fibrosis. After the biopsy, mechanic's hand and Gottron's papules appeared, and anti-melanoma differentiation-associated gene 5 (MDA5) antibody was found to be positive. Subsequently, anti-MDA5 antibody measured in cryopreserved serum from her first admission proved to be positive. It is difficult to suspect the presence of anti-MDA-5 antibody in patients with interstitial lung disease without typical dermatomyositis symptoms or slow disease progression.

Keywords: ANCA, cytoplasmic autoantibody; Anti-melanoma differentiation-associated gene 5; BAL, bronchoalveolar lavage; CADM, clinically amyopathic dermatomyositis; CMV, cytomegalovirus; CT, computed tomography; Clinically amyopathic dermatomyositis; DM, dermatomyositis; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IVCY, intravenous cyclophosphamide; Interstitial lung disease; KL-6, Krebs von den Lungen-6; MDA5, anti-melanoma differentiation-associated gene 5; PSL, prednisolone; PaO2, partial pressure of oxygen; RP-ILD, rapidly progressive interstitial lung disease; SLB, surgical lung biopsy; Slowly progressive; Surgical lung biopsy; TBLB, transbronchial lung biopsy.

Publication types

Case Reports