Treatment Of Granulomatous Disease Research Articles

A case of persistent hypercalcaemia in the treatment of granulomatous disease

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

InterMune Divests Declining Actimmune® to Focus on Esbriet®

InterMune has agreed to sell the rights to Actimmune® (interferon gamma-1b), which is approved for the treatment of chronic granulomatous disease and severe, malignant osteopetrosis, to Vidara Therapeutics International for US$55 M in cash plus royalties over 2 years. The proceeds from the transaction will help InterMune to invest in the registration and commercialisation of its idiopathic pulmonary fibrosis drug Esbriet® (pirfenidone) in Europe and elsewhere.

Maladie granulomateuse au cours du déficit immunitaire commun variable

Purpose Common variable immunodeficiency (CVID), defined by defective production of immunoglobulins, is the most common primary immunodeficiency in adulthood requiring a medical follow-up. Repeated bacterial infections and/or autoimmune manifestations and/or benign lymphoproliferation (including follicular hyperplasia and/or granulomatous disease) are the hallmark of the disease. This review aims at describing recent advances in the understanding and treatment of granulomatous disease in CVID. Current knowledge and key points Clinical features of granulomatous disease in CVID can mimic sarcoidosis, remarkable by the low levels of circulating immunoglobulins. Granulomas may be found in several organs in a single patient, and the main features are pulmonary, lymphoid, cutaneous, hepatic or splenic. The features of CVID is remarkable by the high frequency of autoimmune diseases complicating the immunodeficiency. Some immunological abnormalities have been described in such patients, including lymphopenia, decreased T-cells proliferations to mitogens and antigens. Rare polymorphisms in the gene encoding TNFα (Tumor Necrosis Factor) have been identified in CVID patients with granulomatous disease. Future prospects and projects The evolution of the disease is severe, particularly when the lung is involved. Treatment consists in immunoglobulins substitution, immunosuppressive agents (corticosteroids, cyclophosphamide) and anti-TNFα antibodies. These treatments are difficult to manage in such immunocompromised patients.

Etanercept treatment of cutaneous granulomas in common variable immunodeficiency

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia, poor antibody responses, and recurrent bacterial infections, usually of the sinorespiratory tract. A not uncommon complication is granuloma of the lungs, spleen, liver, and/or skin. We report the case of an 18-year-old boy with CVID and chronic granulomas of the left arm (since 13 years of age) refractory to treatment with antibiotics, intravenous immunoglobulin, antifungal agents, systemic and intralesional steroids, IFN-gamma, cyclosporine, methotrexate, hydroxychloroquine, localized radiation therapy, and surgical excision. The lesions improved after treatment with the systemic administration of the TNF-alpha inhibitor etanercept for 1 year. Etanercept prevents soluble TNF from binding to its cell membrane receptor, leading to inhibition of its inflammatory cascade. We recommend further trials of etanercept in patients with CVID with noninfectious recalcitrant granulomas.