Topa, Antonietta (2020) Investigation of clinical and pathogenic heterogeneity in Chronic Inflammatory Demyelinating Polyneuropathy. [Tesi di dottorato]

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Abstract

My aim during the last three years was to create a detailed database of CIDP patients, in order to analyse the data collected in a retrospective manner and to take part to national projects on the disease. In fact, since aspects of CIDP are still unsolved, such as clinical variability, pathogenesis, prognostic factors, outcome measures and response to treatment, and considering that CIDP is a rare disease, a nation-wide collaboration is indispensable. In Section 1 I will describe the clinical and epidemiological features of CIDP patients who referred, in the last 15 years, to our Centre of Neuromuscular Disease at University Federico II of Naples and that I selected among all the patients with disimmune diseases of peripheral nervous system. In Section 2 I will summarise the preliminary conclusions derived from the creation of a web-based national database promoted by the Humanitas Institute that involved more than 500 patients, among which 51 come from our Neuromuscular Centre in Naples. Section 3 is dedicated to the pathogenesis, in particular to the emerging role of antibodies against paranodal proteins in the determination of specific morphologic alterations and clinical phenotypes. In particular, I will speculate on the pathogenesis of CIDP with antibodies against Neurofascin 155. Then I will report the results derived from our collaboration with the IRCCS Mondino Foundation, Pavia, Italy, which analyzed the sera from different national centres, including our centre, in order to identify and characterize the anti-nodal and paranodal antibodies incidence and their correlation with phenotypic aspects in a large cohort of patients.

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