Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency

J Allergy Clin Immunol. 2004 Aug;114(2):415-21. doi: 10.1016/j.jaci.2004.05.057.


Background: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low levels of serum immunoglobulins and an inability to make specific antibodies.

Objective: We sought to determine the prevalence, clinical characteristics, and effect on survival of noninfectious pulmonary disease in patients with CVID.

Methods: A retrospective analysis of 69 patients with CVID was performed. Patients were divided into 3 groups on the basis of the type of pulmonary disease present: group 1 (n=29), no pulmonary disease; group 2 (n=23), chronic respiratory symptoms without diffuse radiographic abnormalities; and group 3 (n=18), chronic respiratory symptoms and diffuse radiographic abnormalities. Group 3 patients were divided into 2 subgroups on the basis of the histopathologic pattern seen on biopsy. Group 3A (n=13) included patients with granulomatous lung disease, lymphocytic interstitial pneumonia, follicular bronchiolitis, and lymphoid hyperplasia, a group of syndromes referred to as granulomatous-lymphocytic interstitial lung disease (GLILD). Group 3B (n=5) consisted of patients with all other types of interstitial lung disease (ILD).

Results: Fifty-eight percent of patients with CVID had noninfectious pulmonary complications. Group 3A (GLILD) patients had worse prognosis than the other groups, with a median survival of 13.7 versus 28.8 years (P<.001). Lymphoproliferative disease occurred in 31% of patients with GLILD. GLILD was associated with the presence of dyspnea (P<.05); splenomegaly (P<.05); restrictive pulmonary physiology; consolidation, ground-glass, and reticular radiographic abnormalities; and low CD3+ (P<.05) and CD8+ cell populations (P<.01).

Conclusion: ILD is common in patients with CVID. The presence of GLILD was associated with a worse prognosis and increased prevalence of lymphoproliferative disorders.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Common Variable Immunodeficiency / complications
  • Common Variable Immunodeficiency / mortality*
  • Female
  • Granuloma / diagnostic imaging
  • Granuloma / epidemiology*
  • Granuloma / pathology
  • Humans
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / epidemiology*
  • Lung Diseases, Interstitial / pathology
  • Male
  • Middle Aged
  • Prevalence
  • Prognosis
  • Radiography
  • Retrospective Studies