Abstract
Common variable immunodeficiency (CVID) is a primary immunodeficiency that is characterized
by hypogammaglobulinemia and poor/absent specific antibody production. Granulomatous
and lymphocytic interstitial lung disease (GLILD) is an increasingly recognized complication
of CVID, occurring in 10 to 20% of patients. GLILD is characterized by non-necrotizing
granuloma, lymphocytic interstitial pneumonitis and follicular bronchiolitis-histological
patterns that are typically present in the same biopsy. GLILD is a multisystem disease
and is frequently accompanied by diffuse adenopathy, splenomegaly, and extrapulmonary
granulomatous disease most commonly in the lymph nodes, spleen, liver, and gastrointestinal
tract. The presence of noncaseating granuloma in the lung along with some of the extrapulmonary
features of GLILD may lead to an incorrect diagnosis of sarcoidosis. However, GLILD
differs from sarcoidosis in several important ways including mode of presentation,
extrapulmonary manifestations, radiographic abnormalities on high-resolution computed
tomography scan of the chest, and laboratory features (serum immunoglobulins, bronchoalveolar
lavage, and histopathology). The misdiagnosis of sarcoidosis in a patient with CVID
and GLILD can lead to inappropriate treatment and increase the morbidity and mortality
of the disorder.
Keywords
common variable immunodeficiency - sarcoidosis - granulomatous and interstitial lung
disease - immunodeficiency
