Ten to twenty percent of CVID patients develop symptomatic interstitial lung disease. Granulomatous-lymphocytic interstitial lung disease (GLILD) is a distinct clinic-radio-pathological interstitial lung disease associated with lymphocytic infiltrate and/or granuloma in the lung. GLILD is associated with other granulomas, hepatosplenomegaly, lymphadenopathy, immune cytopenias, low IgA, high IgM, increased CD21Low B-cells and decreased switched memory B-cells. Suspicion of GLILD will often be raised on radiologic findings and as lung biopsy carries a high risk of complications we explored whether a set of combined diagnostic criteria could be helpful in identifying patients with a probable or possible diagnosis.
We defined a set of major and minor criteria that should be present for the diagnosis of definitive (all five major), probable (three/four major, four minor) and possible (two major, four minor) GLILD. We then identified 31 CVID-patients with radiological findings suspicious of GLILD and characterized them according to these criteria.
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We identified three patients with definitive and 17 patients with probable GLILD. Eight patients had possible GLILD while three patients with radiologic features lacked substantial support for the diagnosis.
The use of combined diagnostic criteria can be helpful in identifying patients with a likely diagnosis of GLILD also in the absence of histopathological findings.