Granulomatous lymphocytic interstitial lung disease
Granulomatous-lymphocytic interstitial lung disease (GL-ILD) is a relatively recent term given to describe non-infectious diffuse lung disease complications that have been reported to traditionally develop in common variable immunodeficiency (CVID) patients.
Epidemiology
Associations
Although it is almost always associated with CVID, some papers also report the occurrence of these conditions with primary immunodeficiency states such as :
- Kabuki syndrome
- LRBA mutations
- X-linked inhibitor of apoptosis
- CTLA4 mutations
- Good syndrome
- RAG mutations
- hypogammaglobulinemia of unclear etiology in infancy
- 22q11.2 deletion syndrome
Pathology
They exhibit both granulomatous and lymphoproliferative histologic patterns consisting of:
- lymphocytic interstitial pneumonia (LIP)
- follicular bronchiolitis and
- lymphoid hyperplasia
Radiographic features
CT
Pulmonary findings tend to have a mid- to lower zones distribution and are usually better depicted on CT , including:
- diffuse micronodules
- interlobular septal thickening
- bronchiectasis: tend to be mild
- lymphadenopathy
Splenomegaly is almost always seen in those patients.
Treatment and prognosis
Immunosuppressive therapy (e.g. rituximab) has been successfully used in the treatment of granulomatous lymphocytic interstitial lung disease. The presence of granulomatous lymphocytic interstitial lung disease in association with common variable immunodeficiency confers a poorer prognosis and also carries an increased risk of lymphoproliferative disorders.
