Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Aug 6, 2023; 11(22): 5351-5357
Published online Aug 6, 2023. doi: 10.12998/wjcc.v11.i22.5351
Treatment of refractory anti-melanoma differentiation-associated gene 5 anbibody-positive dermatomyositis complicated by rapidly progressing interstitial pulmonary disease: Two case reports
Qiao-Hong Wang, Li-Heng Chen
Qiao-Hong Wang, Department of Rheumatology, Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310009, Zhejiang Province, China
Li-Heng Chen, Department of Endocrinology, Fourth Affiliated Hospital, College of Medicine, Zhejiang University, Yiwu 322000, Zhejiang Province, China
Author contributions: Wang QH was involved in conceptualization, supervision, and manuscript writing; Chen LH was involved in manuscript writing.
Informed consent statement: Informed consent was obtained from the patients for the publication of this case report.
Conflict-of-interest statement: The authors declare that they have no conflicting interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Qiao-Hong Wang, MS, Associate Chief Physician, Department of Rheumatology, Second Affiliated Hospital, College of Medicine, Zhejiang University, No. 88 Jiefang Road, Hangzhou 310009, Zhejiang Province, China. 2202015@zju.edu.cn
Received: April 5, 2023
Peer-review started: April 5, 2023
First decision: May 19, 2023
Revised: May 30, 2023
Accepted: July 4, 2023
Article in press: July 4, 2023
Published online: August 6, 2023
Abstract
BACKGROUND

Anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5 Ab+) dermatomyositis complicated with rapidly progressive interstitial lung disease (anti-MDA5 Ab+ DM-RP-ILD) has an unclear underlying mechanism with no recommended unified treatment plan. Herein, one of the cases that we report (Case 2) was successfully treated with tocilizumab despite having lung infection.

CASE SUMMARY

Case 1 was a 30-year-old woman who was admitted due to recurrent rash for 5 mo, fever and cough for 1 mo, and chest tightness for 3 d. She was diagnosed with non-myopathic dermatomyositis (anti-MDA5 Ab+) and interstitial pneumonia, and was treated with the combination of hormone therapy and cyclophosphamide followed by oral tacrolimus. Case 2 was a 31-year-old man admitted due to systemic rash accompanied by muscle weakness of limbs for more than 1 mo, and chest tightness and dry cough for 4 d. He was diagnosed with dermatomyositis (anti-MDA5 Ab+) and acute interstitial pneumonia with Pneumocystis jirovecii and Aspergillus fumigatus infections and was treated with hormone therapy (without cyclophosphamide) and the combination of tocilizumab and tacrolimus. The condition of both patients eventually improved and they were discharged and showed clinically stable condition at the latest follow-up.

CONCLUSION

Tocilizumab could be a salvage treatment for patients with anti-MDA5 Ab+ DM-RP-ILD who are refractory to intensive immunosuppression.

Keywords: Anti-melanoma differentiation-associated gene 5 antibody-positive, Dermatomyositis, Progressive interstitial lung disease, Interstitial lung disease, Tocilizumab, Case report

Core Tip: The early detection of myositis-related antibody profile and its concentration together with serum ferritin and cytokine levels is key to the clinical diagnosis and prognosis of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis complicated with rapidly progressive interstitial lung disease (anti-MDA5 Ab+ DM-RP-ILD). For patients with anti-MDA5 Ab+ DM-RP-ILD refractory to intensive immunosuppression, tocilizumab could be a salvage treatment.