- Design
- GRADE-based clinical practice guideline
- Population
- Adults with common variable immunodeficiency disorders
- Primary outcome
- Screening and diagnosis of GLILD
- Effect
- Screening recommended for all adults, preferably by HRCT; most recommendations conditional
Granulomatous-lymphocytic interstitial lung disease (GLILD) is an inflammatory lung complication of common variable immunodeficiency that can progress to fibrosis and is associated with worse outcomes. Until now there has been no accepted guideline, and practice has varied widely. This European Respiratory Society Clinical Research Collaboration guideline used GRADE methodology and evidence-to-decision frameworks.
It recommends screening all adults with common variable immunodeficiency for GLILD, preferably with high-resolution CT. Evaluation should be multidisciplinary. Lung biopsy is not mandatory but is needed for atypical presentations. The work-up should exclude pulmonary infection, use bronchoalveolar lavage to look for lymphocytic alveolitis, and assess lung function including gas transfer. Most recommendations are conditional because the underlying evidence is of low certainty.
For pulmonologists, the change is that GLILD is no longer something to look for only when symptoms appear. Patients with common variable immunodeficiency are often managed by immunologists, so a shared screening pathway is needed. Imaging access and cost will shape how this is applied in India.
- Screen all adults with common variable immunodeficiency for GLILD, preferably with HRCT.
- Exclude infection before attributing lung changes to GLILD.
- Use bronchoalveolar lavage to look for lymphocytic alveolitis as part of the work-up.
- Include gas transfer in lung function testing.
- Reserve biopsy for atypical presentations, decided at a multidisciplinary meeting.
Why it matters
GLILD has been found late because nobody owned the screening; this guideline assigns it.
Don't overread it
Most recommendations are conditional and rest on low-certainty evidence.
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