PURPOSE: Granulomatous diseases comprise a heterogeneous group of disorders. Sarcoidosis and Common Variable Immunodeficiency (CVID) affect the same organs with granuloma and their histological appearance is indistinguishable.
METHODS: Retrospective analysis of patients’ clinical records followed in our clinic with sarcoidosis or granulomatous disease and CVID.
RESULTS: Five patients were followed, 3 men and 2 women, mean age of 39 years (ranging 20-71). Mean age of first diagnosis was 32 (ranging 8-70), and time elapsed until second diagnosis was 68 months (ranging 4-156). Two patients were first diagnosed with sarcoidosis. One was diagnosed after chemotherapy for chronic lymphocytic leukemia and supported by high ACE levels, mediastinal and hilar adenopathies, micronodules (stage II) and granuloma in transcarinal biopsy. Clinical features coincided with the appearance a significant decrease of IgM, IgG and IgA levels. Second patient was diagnosed with sarcoidosis at the age of 7 (pulmonary, splenic and cardiac). Diagnosis was supported by bronchial biopsy with granuloma and cardiac involvement. One year later, diagnosis of CVID was made. Both patients are still being treated with low dose steroids, have stable chronic disease and reduced TLCO. The second patient receives immunoglobulin replacement therapy (IRT) due to repeated respiratory infections. In the group of patients first diagnosed with CVID, no one fulfilled criteria of sarcoidosis: HRCT features, although abnormal weren’t typical for sarcoidosis, all had high ACE levels, granuloma at bronchial or transbronchial pulmonary biopsy (infection was ruled out in two patients but a Mycobacterium gordonae later grew up on bronchial secretions of a third one), one had hepatic granuloma, mediastinal and axilar adenopathies. One had a reduced TLCO. All patients are under IRT and were treated at one point with low dose steroids. At present only one has stable disease, the other two have rising ACE levels and worsening of pulmonary infiltrates. All patients had splenomegaly.
CONCLUSIONS: In this group of patients all had positive ACE, mediastinal adenopathies and splenomegaly, regardless of a confident diagnosis of sarcoidosis. This diagnosis appears to be based on an HRCT with micronodulation around the bronchovascular branches. Granuloma per se is regarded as nonspecific and not strong enough to support a diagnosis of sarcoidosis. The line that separates both entities is tenuous.
CLINICAL IMPLICATIONS: More studies are needed to better understand these manifestations.
DISCLOSURE: The following authors have nothing to disclose: Nelson Marçal, Margarida Aguiar, Inês Claro, Sara Silva, Susana Silva, Ana Mendes, Bugalho Almeida
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