Rare Diseases, Egpa, Guggino (UniPa): “New therapies for complex autoimmune pathology”

The origin of Egpa, eosinophilic granulomatosis with polyangiitis, “a vasculitis characterized by a series of systemic manifestations, is unknown. However, we know that it is certainly a rare autoimmune disease that can also be associated with the presence of some autoantibodies, in particular anti-neutrophil cytoplasmic antibodies (Anca), so much so that we speak of an Anca-associated vasculitis precisely because of the presence of anti-myeloperoxidase antibodies (anti-Mpo)”. This was stated by Giuliana Guggino, full professor of Rheumatology at the University of Palermo and head of the Rheumatology Unit of the Paolo Giaccone Polyclinic, to Adnkronos on the occasion of the ‘Egpa Study group’ meeting taking place these days in Palermo.

Egpa “is a systemic vasculitis that can involve multiple organs. In most cases – explains Guggino – it can manifest itself with late-onset asthma, with rhinitis, which is then associated with the presence of transient evanescent pulmonary infiltrates and hypereosinophilia”, that is, the increase in the blood of eosinophilic granulocytes, a particular type of immune system cells. “In addition to peripheral blood hypereosinophilia, there is eosinophilic infiltration of tissues with involvement of many other organs: heart, kidneys, muscles, nervous system and so on. Often this involvement of other organs can be present from the beginning and be the onset of the pathology”.

The patient with Egpa “usually turns to allergists and immunologists – continues the professor – precisely for the airway manifestations, and only later, when other systemic signs and symptoms appear, does he consult the rheumatologist”. Given the complex nature of the disease and the need to quickly undertake specific therapies, “early management in dedicated reference centers is important. In Sicily they are present and distributed both in the eastern and western areas to ensure the correct management of the patient, also ensuring a multidisciplinary approach. At the Policlinico Giaccone in Palermo, for example, Pneumology and Rheumatology share a dedicated pneumoeumatology clinic for rapid diagnostic assessment and correct management of the patient. Today – Guggino specifies – we are able to treat and welcome all patients for whom dedicated treatments are available today that make use of glucocorticoids, and other immunosuppressive therapies in particular. For some years we have had the possibility of using biotechnological drugs such as monoclonal antibodies that block B cells, for example rituximab, or more recent generation, such as anti-interleukin-5 (mepolizumab), which are indicated in the treatment of Egpa. These more modern drugs have improved the prognosis and survival of these patients who – he concludes – can have a better quality of life, thanks to good control of the disease”.