Interferon-α induced remission in three patients with eosinophilic granulomatosis and polyangiitis. A case study
نویسندگان
چکیده
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small vessel vasculitis associated with asthma and eosinophilia. Optimal therapy for maintenance of remission is yet to be defined. We present a case-series of three patients with EGPA in whom IFN-α, an immunomodulatory cytokine induced remission, which was maintained even after discontinuation of the drug. In all patients (ages 60, 51, and 50 years), remission was associated with normalisation of eosinophil counts and IgE-levels. Moreover, the patients remained in remission for one to four years. Two patients did not need further immunosuppression, one patient required low dose maintenance therapy. Although reversible side effects occur, IFN-α-therapy induces long-term remission of EGPA even after discontinuation of treatment.
منابع مشابه
Eosinophilic Granulomatosis with Polyangiitis Manifested by Cholecystitis and Mononeuritis Multiplex: A Case Report
Eosinophilic granulomatosis with polyangiitis formerly named “Churg-Strauss syndrome (CSS)” is a systemic disease with bronchial asthma, hypereosinophilia, and systemic vasculitis. We report a case of CSS with cholecystitis and mononeuritis multiplex. A 50-year-old woman with a history of sinusitis and bronchial asthma of 8 years’ duration was referred with a complaint of left-hand deformity an...
متن کاملEpidemiology of Vasculitides in Khorasan Province, Iran
Vasculitides are a heterogeneous group of more than 20 diseases defined by inflammation and destruction of blood vessels. We aimed to study the demographic characteristics of the primary vasculitides in the North East of Iran. We retrospectively studied the medical records of patients diagnosed with any kind of vasculitis at the Clinic and Department of Rheumatology of the Imam Reza Hospital, M...
متن کاملAn Unusual Presentation of Granulomatosis with Polyangiitis
The granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a pauci-immune vasculitis involving small- and medium-sized blood vessels. Classically, the patients inflicted with this disease present with a triad of necrotizing granulomatous inflammation of the respiratory tract, cutaneous necrotizing vasculitis, and glomerulonephritis (GN). The antinuclear (ANA) and...
متن کاملAmantadine Plus Interferon-α Versus Interferon-α Monotherapy for the Treatment of Chronic Hepatitis-C Infection in β-Thalassemia Major Patients: A Randomized Double Blinded Pilot Study in Shiraz, Iran
Background: Hepatitis-C infection is a major problem in chronically transfused patients. We compared Interferon-α (INF-α) monotherapy with combination of INF-α and amantadine in the treatment of β-thalassemia major patients who were chronically infected with HCV.Materials and Methods: Forty six thalassemia major patients who were chronically infected with HCV were randomly divided into two grou...
متن کاملTreatment of ANCA-Associated Vasculitis in Adults
Vasculitis is the general term used to describe diseases associated with inflammation of the blood vessels. This inflammation results in end-organ ischemia and damage with lifethreatening consequences. Treatment is tailored to the type of vasculitis the patient has, prognostic features and disease severity. Two main treatment phases are recognized: induction of remission, and maintenance of rem...
متن کامل