What does this research mean for the field?

Granulomatous-lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID) can present as a rapidly progressive condition that is refractory to standard immunoglobulin replacement and immunosuppressive therapies, leading to fatal infectious complications. Novelty: ClaimNovelty.CONTRADICTORY. Consensus alignment: ConsensusAlignment.CHALLENGES_CONSENSUS.

What question did this study set out to answer?

This case examines the rapid progression and complications of granulomatous-lymphocytic interstitial lung disease in a patient with common variable immunodeficiency.

June 1, 2026Internal Medicine0 citationsOpen Access

Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency with a Rapid Progression and Refractory Infections

RTRyuki TokunagaKumamoto University Hospital AMAiko MasunagaKumamoto University Hospital MKMasashi KogaKumamoto University Hospital

Key Points

This case examines the rapid progression and complications of granulomatous-lymphocytic interstitial lung disease in a patient with common variable immunodeficiency.
Case study of a 34-year-old woman with common variable immunodeficiency and pulmonary abnormalities.
Surgical biopsy confirmed granulomatous-lymphocytic interstitial lung disease.
Interventions included prednisolone and cyclosporine, alongside immunoglobulin replacement therapy and erythromycin.
Lung lesions and hypoxemia progressed despite treatments, leading to severe complications.
The patient died from infectious complications after treatment failure.

Abstract

A 34-year-old woman with common variable immunodeficiency (CVID) developed pulmonary abnormalities despite adequate subcutaneous immunoglobulin replacement and long-term low-dose erythromycin therapy. Her lung lesions and hypoxemia progressed, and a surgical biopsy confirmed granulomatous-lymphocytic interstitial lung disease (GLILD). Prednisolone and cyclosporine were administered. However, her condition worsened, and she died of infectious complications. Although patients with CVID-GLILD often respond to immunoglobulin replacement therapy or immunosuppression, this case was rapidly progressive and refractory to treatment.

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