It’s been awhile since our last ILD round-up, but we are glad to be back in action! This week we talked about a young man with CVID, ITP and persistent groundglass opacities. We discussed a concern for granulomatous and lymphocytic interstitial lung disease (GL-ILD)
I. What is GL-ILD?
Seen in patients with CVID. With the advent of effective therapies (namely IVIG), increased prevalence of non-infectious complications of CVID (non-infectious complications now seen in 70% of patients with CVID).
GL-ILD is seen in 8-20% of cases of CVID, making it the most common ILD in this condition. It may also be seen in CTLA-4 deficiency. Associated with splenomegaly, immune-mediated cytopenias, and ITP. BAL demonstrates increased percentage of CD21lo B cells. Pathology demonstrates a peribronchiolar lymphocytic infiltrate, usually associated with granulomas (94% in one case series) and often with organization.
Image: peribronchial and interstitial lymphocytic infiltration
Image: Epithelioid granuloma
II. What are the characteristic HRCT findings of GL-ILD?
Solid & subsolid nodules, groundglass opacities, reticulations, mediastinal and hilar adenopathy. Less likely to contain reticulation, TBE, honeycombing, masses or consolidation. A radiologic DDX includes infection, organizing pneumonia, LIP, sarcoidosis and lymphoma. As alreadt noted, splenomegaly (bottom image) is common.
III. How is GL-ILD treated?
Consensus guidelines with strong agreement that mainstay of therapy is optimization of IVIG therapy. Whether to proactively treat or enter active surveillance after IVIG optimization is less well established, nor is whether antibiotic prophylaxis (as is often the case in CVID) is warranted. Corticosteroids are frequently used, but without clear evidence basis or consensus. Potential second line agents include azathioprine, rituximab, MMF.
Sources:
- J Allergy Clin Immunol Pract. (2017); 5:938-945. (link)
- Front Immunol. (2021); 12:627423. (link)
- Hum Pathol (2016); 46(9): 1306-1314. (link)
- https://radiopaedia.org/cases/granulomatous-lymphocytic-interstitial-lung-disease?lang=us