Correct!
4 1 and 2 or 5. 1 and 3
GLILD occurs in 20 to 30% of patients with SCID and may occur in other patients with a primary immunodeficiency. The disease results from lymphocytic infiltration and/or granulomas in the lungs in which other causes are excluded. It may be a pulmonary manifestation of a more diffuse lymphoproliferative disorder as lymphadenopathy and splenomegaly are common. Biopsy in our patient was thought to be overly risky and currently he is being monitored for progression with a plan to treat with rituximab and azathioprine or mycophenolate should he progress.
References
- Lonni S, Chalmers JD, Goeminne PC, et al. Etiology of Non-Cystic Fibrosis Bronchiectasis in Adults and Its Correlation to Disease Severity. Ann Am Thorac Soc. 2015 Dec;12(12):1764-70. [CrossRef] [PubMed]
- Schmalstieg FC, Goldman AS. Immune consequences of mutations in the human common gamma-chain gene. Mol Genet Metab. 2002 Jul;76(3):163-71. [CrossRef] [PubMed]
- Verbsky JW, Hintermeyer MK, Simpson PM, et al. Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency. J Allergy Clin Immunol. 2021 Feb;147(2):704-712.e17. [CrossRef] [PubMed]
Home/Pulmonary