Granulomatosis eosinofílica con poliangeitis: presentación inusual de una enfermedad rara en la edad pediátrica / Eosinophilic granulomatosis with polyangiitis: unusual presentation of a rare disease in pediatric age

La granulomatosis eosinofílica con poliangitis (GEPA), es un trastorno poco frecuente en la edad pediátrica, representando menos de un 2% de las vasculitis en pediatría. Su etiología es desconocida y de difícil diagnóstico debido a la variabilidad en su cuadro clínico. Los pacientes presentan como rasgo característico antecedentes de asma, rinitis alérgica y eosinofilia en sangre periférica. A nivel microscópico es una vasculitis necrosante que afecta a vasos de pequeño a mediano calibre, presencia de granulomas y eosinofilia extravascular. La GEPA es una vasculitis asociada a la presencia de anticuerpos anticitoplasma de neutrófilo (ANCA), su positividad en niños sólo se encuentran en menos del 40% de los casos. Presentamos el caso de una escolar de 8 años con cuadros respiratorios recurrentes asociados a eosinofilia superior al 10%, manifestaciones cutáneas importantes, esofagitis eosinofílica y polineuropatía, finalmente diagnosticado con GEPA-ANCA-PR3 (ANCA específico para proteinasa 3).

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disorder in pediatric age, accounting for less than 2% of vasculitis in pediatrics. Its etiology is unknown and difficult to diagnose due to the variability in its clinical picture. Patients have a history of asthma, allergic rhinitis and eosinophilia in peripheral blood as a characteristic feature. Microscopically, necrotizing vasculitis that affects small to medium-caliber vessels, the presence of granulomas and extravascular eosinophilia. GEPA is a vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA), its positivity in children is only found in less than 40% of cases. We present the case of an 8-year-old schoolgirl with recurrent respiratory symptoms associated with eosinophilia greater than 10%, important skin manifestations, eosinophilic esophagitis and polyneuropathy, finally diagnosed with GEPA-ANCA-PR3 (specific ANCA for proteinase 3).

A Case of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss, EGPA) Presenting With Relapsed Mononeuritis Multiplex

We report a case of eosinophilic granulomatosis with polyangiitis (EGPA), a rare multisystem disorder characterized by difficult-to-control asthma, hypereosinophilia and polyneuropathy. We also discuss the Five Factor Score (FFS) risk stratification strategy, which is used to quantitate the extent of the disease and guide treatment strategy.

Churg Strauss Syndrome in a 40 year old woman

Cutaneous vasculitis is a common manifestation of many systemic diseases. In the setting of asthma, eosinophilia and multiple disparate signs and symptoms, more serious cause of vasculitis like Churg-Strauss syndrome (CSS) should always be considered.