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Updated: Aug 8, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary mucosa-associated lymphoid tissue lymphoma in a patient with common variable immunodeficiency syndrome
F Reichenberger1, C Wyser, M Gonon
1Division of Pneumology, Department of Internal Medicine, University Hospital, Basel, Switzerland.
Insights
Common variable immunodeficiency (CVID) patients with progressive lung disease may develop mucosa-associated lymphoid tissue (MALT) lymphoma. This rare B-cell lymphoma should be considered in CVID patients with worsening pulmonary symptoms.
Area of Science:
- Immunology
- Oncology
- Pulmonology
Background:
- Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by recurrent sinopulmonary infections.
- Malignancies, particularly non-Hodgkin's lymphoma, are recognized late complications of CVID.
Observation:
- A CVID patient presented with recurrent sinopulmonary infections, interstitial lung changes, and hepatic granulomas.
- Despite immunoglobulin therapy, the patient's pulmonary reticular and nodular changes progressed.
- Video-assisted thoracoscopic lung biopsy revealed a low-grade B-cell mucosa-associated lymphoid tissue (MALT) lymphoma of the bronchus.
Findings:
- The lung biopsy confirmed MALT lymphoma without evidence of infection.
- This finding suggests a potential link between CVID and the development of pulmonary MALT lymphoma.
Implications:
- Pulmonary MALT lymphoma should be included in the differential diagnosis for progressive lung disease in CVID patients.
- Early recognition and diagnosis of MALT lymphoma in CVID can guide appropriate treatment and management strategies.
Abstract:
Common variable immunodeficiency syndrome (CVID) is a primary immunodeficiency typically presenting with recurrent sinopulmonary infections. Non-Hodgkin's lymphoma and other secondary cancers are typical late complications of CVID. We report on a patient suffering from CVID with a history of recurrent sinopulmonary infections, interstitial pulmonary changes and hepatic granulomas. Despite treatment with intravenous immunoglobulin followed by a reduction in the number of pulmonary infections, reticular and nodular lung changes progressed. Video-assisted thoracoscopic lung biopsy showed a low-grade B cell lymphoma of the mucosa-associated lymphoid tissue (MALT) of the bronchus without evidence of pulmonary infection. In conclusion, MALT lymphoma of the lung should be considered in the differential diagnosis of progressive lung disease in CVID.
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