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Updated: Jun 23, 2026

Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
[Refractory dermatomyositis associated with chronic organizing pneumonia treated with rituximab: report of one case]
Jorge Yáñez V1, Marcela Cisternas M, Velia Saldías H
1Departamento de Enfermedades Respiratorias, Pontificia Universidad Católica de Chile, Santiago, Chile.
Abstract:
Chronic organizing pneumonia (COP) has often been reported as a pulmonary manifestation of collagen vascular diseases, mainly rheumatoid arthritis, but the association of COP and dermatomyositis (DM) has rarely been documented. We report a 55 year-old woman with well-documented DM and a COP. She was refractory to steroids and two other immunosuppressive agents therapy (cyclophosphamide and azathioprine). Therefore, rituximab (2 x 1 g infusions) was used for treatment. During the following weeks her strength gradually increased while creatine kinase (CK), C reactive protein and erythrocyte sedimentation rate normalized. After 6 months, she had a relapse with increased muscle enzymes, fever and moderate muscle weakness. After a second course of rituximab (2 x 1 g infusions), the patient demonstrated a remarkable clinical response as indicated by an increase in muscle strength and moderate decline in creatine kinase levels. Lung abnormalities resolved significantly on high resolution chest CT scan. Thus, B-cell depletion therapy with rituximab used alone or in combination with other immunosuppressants may be a viable option in patients with polymyositis-dermatomyositis and pneumonia refractory to current therapies.
Insights
Rituximab effectively treated chronic organizing pneumonia (COP) in a patient with dermatomyositis (DM) refractory to other therapies. This B-cell depletion therapy offers a potential treatment option for similar complex cases.
Area of Science:
- Rheumatology and Pulmonology
- Immunotherapy and Autoimmune Diseases
Background:
- Chronic organizing pneumonia (COP) is a known pulmonary manifestation of collagen vascular diseases, predominantly rheumatoid arthritis.
- The association between COP and dermatomyositis (DM) is infrequently documented in medical literature.
- Standard treatments for refractory autoimmune-related lung disease often include corticosteroids and other immunosuppressants.
Observation:
- A 55-year-old woman with established dermatomyositis presented with refractory COP.
- The patient showed poor response to steroids, cyclophosphamide, and azathioprine therapies.
- Initial treatment with rituximab resulted in improved muscle strength and normalized inflammatory markers.
Findings:
- A relapse occurred after 6 months, characterized by elevated muscle enzymes, fever, and muscle weakness.
- A second course of rituximab led to significant clinical improvement, including increased muscle strength and reduced creatine kinase levels.
- High-resolution chest CT scans demonstrated substantial resolution of pulmonary abnormalities post-rituximab treatment.
Implications:
- B-cell depletion therapy using rituximab may represent a viable therapeutic strategy for patients with dermatomyositis and refractory COP.
- Rituximab, alone or in combination with other immunosuppressants, shows promise for managing complex autoimmune-related pulmonary conditions.
- This case highlights the potential of targeted immunotherapies in challenging autoimmune-associated lung diseases.
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