Staged multi-target therapy for anti-MDA5-positive dermatomyositis-associated rapidly progressive interstitial lung disease: a case report.
5 affiliations
- 1Department of Respiratory and Critical Care Medicine.The 940th Hospital of Joint Logistic Support Force of Chinese People's Liberation Army, Lanzhou, 730050, China.
- 2The First Clinical Medical College, Gansu University of Chinese Medicine, Lanzhou, 730000, China.
- 3Department of Reproductive Center, The 940th Hospital of Joint Logistic Support Force of Chinese People's Liberation Army, Lanzhou, 730050, China.
- 4The First Affiliated Hospital, And College of Clinical Medicine of Henan University of Science and Technology, Luoyang, China.
- 5Department of Joint Surgery, The 940th Hospital of Joint Logistic Support Force of Chinese People's Liberation Army, Lanzhou, 730050, China.
Abstract
Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis-associated rapidly progressive interstitial lung disease is associated with acute respiratory deterioration and high mortality. A man in his 40s presented with fever, dry cough, progressive dyspnea, mechanic's hand-like lesions and severe hypoxemic respiratory failure. Chest high-resolution computed tomography demonstrated rapidly progressive bilateral ground-glass and reticular opacities. Myositis-specific antibody testing showed strong anti-MDA5 positivity, with concomitant anti-EJ, anti-TIF1γ and anti-Ro52 positivity. Initial intravenous methylprednisolone at 80 mg/day produced limited improvement. Early multi-target escalation included divided-dose methylprednisolone, tofacitinib, short-course intravenous immunoglobulin, Pneumocystis jirovecii prophylaxis and an individualized reduced-dose rituximab regimen consisting of 200 mg followed by three weekly 300-mg infusions. Mycophenolate mofetil was briefly withheld during acute escalation and subsequently resumed for maintenance. Oxygenation improved within 48 h, and serial imaging demonstrated progressive regression of the pulmonary abnormalities. During approximately 6.5 months of follow-up, the patient remained clinically stable without a detected serious opportunistic infection. Because several interventions overlapped and rituximab has a delayed immunological effect, the early response cannot be attributed to rituximab alone. This case illustrates the potential clinical value of staged multi-target treatment adjustment and infection-risk management in severe anti-MDA5-associated rapidly progressive interstitial lung disease.
Related
- Candidate molecular endotypes and dynamic risk prediction in anti-MDA5-positive dermatomyositis-associated interstitial lung disease: a systematic scoping review
- Rituximab Combined With Azathioprine for Relapsed Anti-MDA5 Antibody-Positive Dermatomyositis With Interstitial Lung Disease: A Case Report.
- New-onset Anti-melanoma Differentiation-associated Gene 5 Antibody Positive Clinically Amyopathic Dermatomyositis Following COVID-19
- Blood-based biomarkers associated with progressive pulmonary fibrosis in idiopathic inflammatory myositis-associated interstitial lung disease: A retrospective cohort study from a respiratory center
- Granulomatous Interstitial Nephritis Associated With Adalimumab Therapy in a Patient With Rheumatoid Arthritis: A Case Report
BibTeX
@article{zhang2026staged,
title = {{Staged multi-target therapy for anti-MDA5-positive dermatomyositis-associated rapidly progressive interstitial lung disease: a case report.}},
author = {Lvdan Zhang and Mengwen Jiao and Yan Hou and Youchen Jie and Shaokang Mo and Wei Chen and Qingliang Xue and Yongjie Qiao},
journal = {Respiratory medicine case reports},
year = {2026},
month = sep,
doi = {10.1016/j.rmcr.2026.102521},
url = {https://europepmc.org/article/MED/42898059},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Staged multi-target therapy for anti-MDA5-positive dermatomyositis-associated rapidly progressive interstitial lung disease: a case report.
AU - Lvdan Zhang
AU - Mengwen Jiao
AU - Yan Hou
AU - Youchen Jie
AU - Shaokang Mo
AU - Wei Chen
AU - Qingliang Xue
AU - Yongjie Qiao
PY - 2026
DA - 2026/09/29
JO - Respiratory medicine case reports
DO - 10.1016/j.rmcr.2026.102521
UR - https://europepmc.org/article/MED/42898059
AB - Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis-associated rapidly progressive interstitial lung disease is associated with acute respiratory deterioration and high mortality. A man in his 40s presented with fever, dry cough, progressive dyspnea, mechanic's hand-like lesions and severe hypoxemic respiratory failure. Chest high-resolution computed tomography demonstrated rapidly progressive bilateral ground-glass and reticular opacities. Myositis-specific antibody testing showed strong anti-MDA5 positivity, with concomitant anti-EJ, anti-TIF1γ and anti-Ro52 positivity. Initial intravenous methylprednisolone at 80 mg/day produced limited improvement. Early multi-target escalation included divided-dose methylprednisolone, tofacitinib, short-course intravenous immunoglobulin, Pneumocystis jirovecii prophylaxis and an individualized reduced-dose rituximab regimen consisting of 200 mg followed by three weekly 300-mg infusions. Mycophenolate mofetil was briefly withheld during acute escalation and subsequently resumed for maintenance. Oxygenation improved within 48 h, and serial imaging demonstrated progressive regression of the pulmonary abnormalities. During approximately 6.5 months of follow-up, the patient remained clinically stable without a detected serious opportunistic infection. Because several interventions overlapped and rituximab has a delayed immunological effect, the early response cannot be attributed to rituximab alone. This case illustrates the potential clinical value of staged multi-target treatment adjustment and infection-risk management in severe anti-MDA5-associated rapidly progressive interstitial lung disease.
SN - 2213-0071
ER -