Novembre 2020 - Volume XXXIX - numero 9

Medico e Bambino


Problemi speciali

La granulomatosi eosinofilica con poliangioite: dalla pelle al cuore

Matteo Pavan1, Anna Agrusti2, Andrea Trombetta2, Serena Pastore1, Alberto Tommasini1,2, Valentina Moressa1, Federico Marchetti3, Andrea Taddio1,2, Alessandro Ventura2

1IRCCS Materno-Infantile “Burlo Garofolo”, Trieste
2Università di Trieste
3UOC di Pediatria e Neonatologia, Ospedale di Ravenna, AUSL della Romagna

Indirizzo per corrispondenza: serena.pastore@burlo.trieste.it

Eosinophilic granulomatosis with polyangiitis: from skin to heart (Churg-Strauss syndrome)

Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia

Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs.
Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission.
Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

Vuoi citare questo contributo?

M. Pavan, A. Agrusti, A. Trombetta, S. Pastore, A. Tommasini, V. Moressa, F. Marchetti, A. Taddio, A. Ventura La granulomatosi eosinofilica con poliangioite: dalla pelle al cuore. Medico e Bambino 2020;39(9):569-574 https://www.medicoebambino.com/?id=2009_569.pdf


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