Main features of granulomatous-lymphocytic interstitial lung disease (GLILD) and sarcoidosis

Main featuresGLILDSarcoidosis
Gamma globulinGenerally decreased (may be normal in IgG subclass deficiency), low serum IgA level and higher IgM levels have been reportedNormal or increased, no specific Ig class or subclass level alteration
ACEGenerally normalOften increased
Decreased circulating switched-memory B-cellsFrequentNot reported
Increased circulating CD21 low B-cellsFrequentNot found
BALF lymphocytosisFrequent (>20%)Frequent
Elevated BALF CD4:CD8 ratioReported in a small case seriesTypical in acute Lofgren Syndrome
Recurrent infectionsGenerally reportedInfrequent
Autoimmune cytopeniaFrequentNot associated, cytopenia may be due to bone marrow granulomatous infiltration or splenomegaly
SplenomegalyFrequentSpleen may be involved, splenomegaly is infrequent and generally secondary to severe liver disease
Nodular regenerative hyperplasia of the liverIncreased likelihoodLiver involvement is often asymptomatic, biopsies may show granulomatous hepatitis
Gastrointestinal involvementReported in 15%Rare
Eye involvementNot reportedFrequent
PLH histological and radiological evidence (e.g. LIP and FB)TypicalNot present
Hilar adenopathyMay be presentTypical feature
Lung nodules size and distribution on HRCTOften >1 cm, with random or predominantly basal distributionTypically <1 cm, with mainly apical and peri-lymphatic distribution
BronchiectasisFrequentTraction bronchiectasis may be found in advanced fibrotic disease
PrognosisSlowly progressing restrictive lung disease with poor prognosisGenerally good prognosis, spontaneous remission may frequently occur, particularly in acute (Lofgren Syndrome) presentation

ACE: angiotensin converting enzyme; BALF: bronchoalveolar lavage fluid; PLH: pulmonary lymphoid hyperplasia; LIP: lymphocytic interstitial pneumonia; FB: follicular bronchiolitis; HRCT: high-resolution computed tomography; Ig: immunoglobulin. Data from [16, 105, 106].