[Acquired hypogammaglobulinemia and multifocal granulomatosis]

In adult acquired hypogammaglobulinaemia multi focal granulomas have often been described and have regularly led to the hypothesis of an association with sarcoidosis. We present a case of this type in a man aged 29 who was a smoker with a hypoglobulinaemia involving IgG, IgA and IgM and which was discovered following pneumococcal pneumonias. He presented with a significant hepatosplenomegaly and absent cutaneous reactions to T dependent antigens with an elevated ACE activity. Histological examination of the splenectomy specimen and of the liver biopsy showed an infiltration by epithelioid follicles and confluent giant cells without necrosis. The pulmonary studies showed a normal chest radiograph but the bronchial biopsy again found a granulomatous infiltration. The broncho-alveolar lavage was cytologically normal and a very slight and paradoxical reduction of the alveolar immunoglobulins was noted implying either an active intra-alveolar concentration of immunoglobulins or a local synthesis. In the light of the few reported cases it seems that the diagnosis of sarcoidosis should be dismissed here in favour of multi focal granulomatosis with hypogammaglobulinaemia. In hypogammaglobulinaemia there is no clinical or biological method (IDR tuberculin, ACE, Kveim, histology) to confirm a superadded diagnosis of sarcoidosis.