PReS-FINAL-2321: Clinical manifestations of granulomatosis with polyangiitis in 8 children from south-east region of Poland
نویسندگان
چکیده
Introduction Granulomatosis with polyangiitis (GPA) is a necrotising granulomatous vasculitis affecting the small and medium blood vessels in particular of airways and kidneys. The incidence of GPA in Europe is 25-150 per 1 million per year. This disease typically occurs in the 4th or 5th decades of life and in children it usually cause diagnostic and therapeutic difficulties. Subglottic stenosis and nasal deformity are frequently registered in this group of young patients.
منابع مشابه
PReS-FINAL-2360: Severe disease course of pediatric-onset granulomatosis with polyangiitis as compared to adult patients: a long-term, single center, follow up study
Methods The study included children and adult patients selected on the basis of complete data set and follow up of at least one year, followed at the pediatric and adult Rheumatology Centers, University Hospital of Padua, over a period of 20 years (1993-2013). GPA was diagnosed according to the ACR and EULAR/PRES criteria. Clinical features, instrumental findings, laboratory parameters and ther...
متن کاملOtologic Manifestations and Progression in Patients with Wegener’s granulomatosis: A Survey in 55 Patients
Introduction: Granulomatosis with polyangiitis (GPA; also known as Wegener’s granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otolo...
متن کاملEosinophilic 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 کامل