Source: International Congress 2014 – Clinical management of interstitial lung diseases and vasculitis
Disease area: Interstitial lung diseases, Respiratory infections

Abstract

Background: Granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis(MPA) are antineutrophil cytoplasmic antibodies (ANCA) – associated vasculitides with significant morbidity and mortality.Objective: We evaluated the evolution of 68 patients diagnosed with vasculitis between 1997 and 2013, treated with conventional treatment (prednisolone and pulse cyclophosphamide initially, and after remission with prednisolone and azathioprine).Results: We evaluated 68 patients (36 females), median age of 54 years (range 17-84), with a median duration of follow up of 39.5 months (range 1-144 months), 26 with GPA, 40 with MPA and 2 with EGPA. Seven patients out of 13 performed had alveolar haemorrhage at broncho-alveolar lavage. Seventeen patients had relapses (25%), 1 developed subglottic stenosis, 1 retro-orbital pseudo tumor and 2 patients developed opportunistic infectious of the lung cavities and needed lung resection. Due to immunosuppressive therapy 2 patients developed lung tuberculosis (one multi-drug-resistant) and 1 pulmonary nocardiosis. Patients with GPA had renal failure at diagnosis in a procent of 46,2% and MPA 90%. Twenty seven patients needed definitive hemodialysis for renal failure. Fifteen patients died: 10 with alveolar haemorrhage, 3 with sepsis, 1 with stroke and one cancer. MPA diagnosis, older age and renal failure were predictors of death.Conclusions: Significant differences exist between GPA and MPA at the initial presentation that can predict the evolution of the disease. The management of patients with ANCA-associated vasculitides is difficult and marked by the adverse effects of the therapy.

Rating:

Share or cite this content

Citations should be made in the following way:
I. Belaconi, C. Toma, S. Stancu, S. Dumitrache-Rujinski, C. Tudose, A. Croitoru, L. Grigoriu, D. Leonte, E. Magheran, M. Bogdan (Bucharest, Romania). ANCA associated vasculitis – Evaluation of diagnosis, treatment and outcome in a group of 68 patients. Eur Respir J 2014; 44: Suppl. 58, 4515

Member's Comments

No comment yet.

Related content which might interest you: 30 years experience of plasmapheresis using in complex treatment of pulmonary sarcoidosis (PS) Source: International Congress 2014 – Clinical management of interstitial lung diseases and vasculitis Year: 2014 B-lymphocyte targeted therapy for patients with progressive fibrosing mediastinitis Source: International Congress 2014 – Modern approach and old dilemmas in lung diseases Year: 2014 The presentation and outcome of chronic pulmonary lobar collapse in children Source: International Congress 2014 – Neonatal and paediatric intensive care Year: 2014 Clinical characteristics and follow up in adult patients with primary ciliary dyskinesia Source: International Congress 2015 – New frontiers in the management of interstitial and orphan lung diseases Year: 2015 Patients’ adherence to chronic treatment of pulmonary diseases Source: International Congress 2015 – Clinical assessment and monitoring of asthma and COPD Year: 2015 Pharmacogenetic and clinicopathological markers of BA-COPD (overlap syndrome) Source: International Congress 2014 – COPD - from biomarkers to novel therapeutic opportunities Year: 2014 Risk factors for active tuberculosis in patients with inflammatory bowel disease: A case-control study Source: International Congress 2015 – Latent TB infection and screening Year: 2015 Prevalence of extra-pulmonary comorbidities in COPD patients and its correlation with severity of disease Source: International Congress 2014 – Latest insights in chronic care Year: 2014 Clinical outcomes of patients with idiopathic pulmonary fibrosis in clinical practice: Follow-up results of the INSIGHTS-IPF registry Source: International Congress 2015 – IPF: clinical aspects II Year: 2015 Clinical, functional and laboratory assessment of treatment efficacy in COPD patients Source: International Congress 2014 – Predictors Year: 2014 Pulmonary manifestations in patients with IgG4-related disease at a tertiary care institute Source: International Congress 2015 – Acute and chronic lung diseases: good clinical practice Year: 2015 Prevalence of chronic ischaemic heart disease in idiopathic pulmonary fibrosis: A single center study Source: International Congress 2014 – Clinical management of interstitial lung diseases and vasculitis Year: 2014 Efficacy and safety of modified anti-tubercular treatment(ATT) regimens in treatment of tuberculosis(TB) in patients with underlying chronic liver disease(CLD) Source: International Congress 2016 – Drug-resistant tuberculosis and experiences with delamanid and bedaquiline Year: 2016 Characteristics of essential hypertension in COPD patients Source: International Congress 2014 – COPD: points to ponder Year: 2014 Is clinical presentation different between men and women with COPD? Source: International Congress 2014 – Clinical presentations Year: 2014 Degree of knowledge among physicians about the diagnosis and treatment of alpha-1 antitrypsin deficiency Source: International Congress 2015 – COPD: chronic disease and comorbidities Year: 2015 Features of systemic inflammation in patients with severe community-acquired (CAP) pneumonia of different etiology in HIV-infected patients Source: International Congress 2015 – Immunodepression and severe pneumonia Year: 2015 Chronic granulomatous disease diagnosed in adulthood- Report of 2 Cases Source: International Congress 2015 – Immune mechanisms in the human lung Year: 2015 Subcutaneous sarcoidosis: A clinical analysis of nine patients Source: International Congress 2015 – Sarcoidosis: clinical Year: 2015 Clinical features and outcomes of patients with myositis associated-interstitial lung disease Source: Virtual Congress 2021 – Prognostic markers of disease progression in non-idiopathic pulmonary fibrosis interstitial lung diseases Year: 2021