Egpa: White paper calls to action, early diagnosis can cure

Asthma in adulthood, nasal polyposis, and chronic rhinosinusitis are the symptoms that could hide a more serious condition. We are talking about EGPA (acronym for Eosinophilic Granulomatosis with Polyangiitis), once known as Churg-Strauss syndrome, named after the pathologists who first described it; a rare, systemic and chronic disease. EGPA is a vasculitis, i.e. an inflammation of small and medium-sized blood vessels, accompanied by an increase in eosinophils (a class of white blood cells) in both blood and tissue. If not treated properly and promptly, it can lead to damage to various organs such as the lungs, kidneys, heart, skin and nervous system. There are no precise data on its epidemiology, but it is estimated that there are 10-15 cases per million inhabitants in Italy.

EGPA is a complex disease and, due to its wide variety of manifestations, it is not easily diagnosed, especially in the early stages when it starts with asthma, chronic rhinosinusitis with nasal polyps. These symptoms may be reminiscent of more common diseases, although they present in an atypical manner, such as the late onset of asthma, with worsening requiring increasingly intensive treatment. This phase can last from months to years, making it difficult to suspect EGPA. Symptoms taken alone are not enough to raise the doubt, a broader clinical picture is needed.

Suspicion usually comes in a second phase in which there is a significant increase in the number of eosinophils in the blood (detectable by blood count) with possible cardiovascular manifestations, gastrointestinal or respiratory symptoms. A further possible onset is the so-called 'vasculitic' phase, in which the inflammation may variously present as red spots detected on the skin, tingling, pain, reduced sensitivity in the limbs or cranial nerves and sometimes kidney involvement, or other visceral localisations.

Initial symptoms, due to their similarity to certain diseases including allergic diseases, often lead to an important diagnostic delay. Early diagnosis is possible, but there is a need for greater knowledge of the disease, more targeted training, and greater synergy between specialists, as well as the creation of shared PDTAs.

Until recently, EGPA could mainly be managed with high doses of cortisone, with a potential negative impact on the patient's quality of life due to the development of diabetes, hypertension, cataracts, osteoporosis and psychological disorders that made treatment difficult to sustain in the long term. Today, thanks to progress and a greater understanding of the role of the cells responsible for the disease (eosinophils), specific therapies have been studied. This has drastically changed the history and management of patients with EGPA: solutions can be used to control, first of all, the respiratory manifestations; while in long-term management, new treatments can be used that can act on the eosinophilic inflammation to the point of reducing or even completely discontinuing the use of cortisone.