[Lymphomatoid granulomatosis]

Lymphomatoid granulomatosis, reported in 450 cases in the literature, is a pathological and clinical entity frequently associated with systemic manifestations. The nodular lymphoproliferative formations lead to vessel destruction which immunohistochemistry and molecular biology has linked to T-lymphocyte proliferation. The major pulmonary and extra-pulmonary manifestations are described here together with diagnostic criteria. The prognosis has been greatly improved with the use of corticosteroids and cyclophosphamide-corticoid combinations.