Rare Diseases, Ciccia (UniNa): “For Egpa multidisciplinary centers of diagnosis and therapy”

“It is an extremely complex disease, potentially even lethal, if not intercepted early and treated adequately. This is why multidisciplinary centers are set up both in the diagnosis and treatment phases”. This is how Francesco Ciccia, full professor and director of the UOC Rheumatology at the Luigi Vanvitelli University of Campania, School of Medicine and Surgery, Department of Precision Medicine, defines Egpa, eosinophilic granulomatosis with polyangiitis, to Adnkronos, on the occasion of the ‘Egpa Study group’ meeting taking place these days in Palermo.

“This rare disease manifests itself with symptoms that can be nonspecific and that can also confuse both the patient and the general practitioner – explains Ciccia – because very often the disease, a systemic vasculitis, begins with allergic symptoms – sinusitis, rhinitis, sometimes even bronchial asthma – which however begins at a slightly older age, that is, around 20-30 years of age. This is followed by other systemic symptoms and the presence, at the blood level, of high levels of anti-neutrophil cytoplasmic autoantibodies (ANCA) and hypereosinophilia, that is, very high levels of a particular type of immune cells. The patient often arrives late at the rheumatologist, that is, after having seen the pulmonologist and the immunologist. The diagnosis is therefore late, when there is already involvement of other organs such as the lung, the nervous system, central or peripheral, and also the heart”. For a timely diagnosis of the patient “a multidisciplinary approach is necessary – the professor emphasizes – which certainly includes interaction with the immunologist, with the allergist, but also with a general practitioner. As for the treatment – which today can count on specific drugs for Egpa – although the therapeutic approach is primarily in the hands of the rheumatologist, it is important that the allergist, the ENT specialist, the internist or the immunologist are involved to take into account the specific manifestations of the pathology in each patient”.

In the South, there is a good spread of rheumatology centers with the capacity and expertise to take care of patients with Egpa at a multidisciplinary level. “In Campania, in addition to the center in Naples, there are facilities throughout the region. Of course – Ciccia observes – it is a disease that does not require, like a cardiac disease, a wide spread of intensive cardiac therapies for a certain group of inhabitants in the area. So, in reality, the real problem is the correct identification of the patient and his referral to the dedicated center. There are also reference centers in Puglia, Basilicata and Calabria, as well as in Sicily. However, there needs to be greater awareness on the part of all the actors involved in the diagnostic process of the existence of the disease and of dedicated therapies, which – he concludes – turn off the symptoms, modify the history of the disease and improve the survival of the patient”.