Meet the Expert Immune dysregulation & autoimmunity

DIAGNOSIS OF GRANULOMATOUS-LYMPHOCYTIC INTERSTITIAL LUNG DISEASE IN COMMON VARIABLE IMMUNODEFICIENCY USING A SET OF COMBINED DIAGNOSTIC CRITERIA

Lecture Time
15:20 - 15:30
Presenter
  • Børre Fevang, Norway
Room
Copper
Date
18.09.2019, Wednesday
Session Time
14:35 - 15:30
Presentation Topic
Immune dysregulation & autoimmunity

Abstract

Background and Aims

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.

Methods

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.

Table1.Diagnostic criteria for GLILD
Major

Typical radiology

Typical histopathology on lung biopsy/thoracic lymph node

Reduced/decrease of DLCO/FVC

Exertional dyspnea

Negative microbiological analyses of BAL-F

Minor

Splenomegaly

Immune cytopenias

Elevated IgM

Low IgA

Low switched-memory B-cells

Increased CD21Low B-cells

Granulomas in other tissue

Generalized lymphadenopathy

Genetics associated with interstitial lung disease

Results

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.

glild_in_cvid_esidfigure1.jpg

Conclusions

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.

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