Objective: Polyarteritis nodosa (PAN) is a rare vasculitis in childhood and poor information is known about its long-term outcome. Our aim was to describe the clinical features, at onset and during the disease course, of childhood- onset PAN and identify a potential correlation with persistent organ damage and worse outcome in a cohort of paediatric patients with a confirmed diagnosis of PAN. Methods: A retrospective collection of demographic and clinical data of 52 Caucasian children diagnosed with PAN, fulfilling the EULAR/PRES diagnostic criteria, recruited from eight paediatric rheumatologic centres and one transition unit, was performed. A statistical correlation was made between clinical involvement at onset or during the overall disease course and patients' final outcome. Results: Data from 52 patients (31 males, 21 females) were collected: their mean age at onset was 7.9 years (median 6.3) and mean follow-up period was 6.2 years (median 5.4). At the last follow-up visit, 27 patients (51.9%) were off therapy in clinical remission, 17 (32.7%) were in clinical remission while on medication, and 6 (11.6%) had a persistent or relapsing disease course. Two patients (3.8%) deceased because of severe cerebral involvement. Cranial nerve palsy during the disease course was significantly correlated with a worse prognosis (p=0.011). The presence of nephrogenic hypertension at onset and seizures during the disease course were significantly associated with the development of irreversible organ damage (p= 0.040 and 0.011, respectively). Conclusion: Childhood PAN is a severe disease with substantial risk of long-term morbidities. In our cohort of patients the worst outcome was significantly correlated with renal and neurological involvement. © Clinical and Experimental Rrheumatology 2014.

Clinical overview and outcome in a cohort of children with polyarteritis nodosa / Falcini, F.; La Torre, F.; Vittadello, F.; Rigante, D.; Martini, G.; Corona, F.; Buoncompagni, A.; Alessio, M.; Cortis, E.; Insalaco, A.; Magni-Manzoni, S.; Breda, L.; Matucci-Cerinic, M.; Zulian, F.. - In: CLINICAL AND EXPERIMENTAL RHEUMATOLOGY. - ISSN 0392-856X. - 32:82(2014), pp. S134-S137.

Clinical overview and outcome in a cohort of children with polyarteritis nodosa

La Torre F.;Corona F.;Alessio M.;
2014

Abstract

Objective: Polyarteritis nodosa (PAN) is a rare vasculitis in childhood and poor information is known about its long-term outcome. Our aim was to describe the clinical features, at onset and during the disease course, of childhood- onset PAN and identify a potential correlation with persistent organ damage and worse outcome in a cohort of paediatric patients with a confirmed diagnosis of PAN. Methods: A retrospective collection of demographic and clinical data of 52 Caucasian children diagnosed with PAN, fulfilling the EULAR/PRES diagnostic criteria, recruited from eight paediatric rheumatologic centres and one transition unit, was performed. A statistical correlation was made between clinical involvement at onset or during the overall disease course and patients' final outcome. Results: Data from 52 patients (31 males, 21 females) were collected: their mean age at onset was 7.9 years (median 6.3) and mean follow-up period was 6.2 years (median 5.4). At the last follow-up visit, 27 patients (51.9%) were off therapy in clinical remission, 17 (32.7%) were in clinical remission while on medication, and 6 (11.6%) had a persistent or relapsing disease course. Two patients (3.8%) deceased because of severe cerebral involvement. Cranial nerve palsy during the disease course was significantly correlated with a worse prognosis (p=0.011). The presence of nephrogenic hypertension at onset and seizures during the disease course were significantly associated with the development of irreversible organ damage (p= 0.040 and 0.011, respectively). Conclusion: Childhood PAN is a severe disease with substantial risk of long-term morbidities. In our cohort of patients the worst outcome was significantly correlated with renal and neurological involvement. © Clinical and Experimental Rrheumatology 2014.
2014
Clinical overview and outcome in a cohort of children with polyarteritis nodosa / Falcini, F.; La Torre, F.; Vittadello, F.; Rigante, D.; Martini, G.; Corona, F.; Buoncompagni, A.; Alessio, M.; Cortis, E.; Insalaco, A.; Magni-Manzoni, S.; Breda, L.; Matucci-Cerinic, M.; Zulian, F.. - In: CLINICAL AND EXPERIMENTAL RHEUMATOLOGY. - ISSN 0392-856X. - 32:82(2014), pp. S134-S137.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11588/821352
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