Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


Predictors of Outcome in Juvenile Polyarteritis Nodosa: A Multicenter Study.

Falcini1,  Fernanda, Torre7,  Francesco La, Martini7,  Giorgia, Vittadello7,  Fabio, Boncompagni10,  Antonella, Corona6,  Fabrizia, Alessio3,  Maria

Dpt of BioMedicine, Division of Rheumatology, Transition Unit, University of Florence
Scientific Institute G. Gaslini, Genoa
Dpt of Medicine, Division of Rheumatology, Ospedale Pediatrico
Dpt of Pediatrics, Rheumatology Unit, Ospedale Federico II
Dpt of Pediatrics, University of Chieti, Chieti
Dpt of Pediatrics, University of Messina
Dpt of Pediatrics, University of Milan
Dpt of Pediatrics, University of Padua
San Matteo Hospital, Pavia
Scientific Institute Bambino Gesu, Rome

Background:

Polyarteritis nodosa (PAN) is a necrotizing vasculitis, seldom reported in childhood and adolescence. The disease is more frequent in Asian populations but it has been reported in all ethnical groups.

Aims:

1.To describe the clinical features, at onset and during the disease course; 2.To look for possible predictive factors related with outcome or persistent damage in a cohort of Italian pts with paediatric onset PAN.

Method:

A retrospective data collection of demographic, clinical and therapeutic characteristics from 50 Caucasian pts (21M, 29F) fulfilling EULAR/PRES criteria for the diagnosis of PAN, from 8 Paediatric Rheumatology Units and 1 Transition Unit, were collected. Mean age at onset was 7.9 yrs (range 2–16 yrs) and the mean follow up 6.2 yrs (range 0.3–16.4 yrs).Correlation among symptoms and internal organs involvement at onset, during the disease course and final outcome or persistent damage was made.

Results:

At onset, skin involvement and systemic symptoms were the most common findings in 36/50 pts (72%); nodules the main cutaneous manifestation (40%), fever the most frequent systemic symptom (66%). Other early clinical manifestations were musculo-skeletal symptoms (54%), renal (12%), CNS involvement (10%), peripheral nervous system (6%), cardiac involvement (6%), and gastrointestinal manifestations (4%). All pts received corticosteroids either oral or IV, 16 pts (32%) azathioprine, 20 pts cyclophosphamide (20%, 15 oral and 5 iv), 9 (18%) thalidomide, 7 (14%) IVIG, 5 (10%) methotrexate, 4 (8%) mycophenolate mofetil, and 2 (4%) biological agents (etanercept and infliximab). At the last follow up visit, 25 pts (50%) were in remission off therapy, 17 (34%) were under control on immunosuppressive drugs and 6 (12%) had persistent relapsing course. Two pts deceased because of ischemic cerebral infarction. At onset, the presence of renal involvement and fatigue significantly correlated with a bad outcome. CNS involvement, as seizures and paralysis, and nephrogenic hypertension, during the disease course, significantly correlated with the development of persistent damage.

Conclusion:

The present study shows that in Caucasian children, PAN is quite severe despite the use of immunosuppressive drugs. Renal and CNS involvement seem to be the main factors affecting the final outcome.

To cite this abstract, please use the following information:
Falcini, Fernanda, Torre, Francesco La, Martini, Giorgia, Vittadello, Fabio, Boncompagni, Antonella, Corona, Fabrizia, et al; Predictors of Outcome in Juvenile Polyarteritis Nodosa: A Multicenter Study. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :2033
DOI: 10.1002/art.29798

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