(Adnkronos) - La miastenia grave “E’ una malattia autoimmune cronica che fornisce la giunzione neuromuscolare, alterando la trasmissione dell'impulso tra il nervo e il muscolo ed è una patologia rara, non rarissima: si stima, infatti, che siano affette da miastenia grave circa 17mila persone in Italia ed è caratterizzata da una affaticabilità muscolare” ovvero quando “i sintomi peggiorano con lo sforzo e migliorano con il riposo”. Così Raffaele Iorio, professore di Neurologia presso l’università Cattolica del Sacro Cuore, Fondazione policlinico universitario Agostino Gemelli Irccs di Roma, intervenendo all’incontro con la stampa organizzato da Argenx, a Milano, per presentare i risultati del primo studio in real world su scala globale sull’impatto sociale e produttivo della miastenia grave su pazienti, familiari e caregiver.
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00:00What is Myasthenia Grave?
00:04Myasthenia Grave is a chronic autoimmune disease
00:08that affects the neuromuscular junction,
00:10altering the transmission of the impulse between the nerve and the muscle,
00:14and it is a rare disease, not very rare.
00:17It is estimated that about 17,000 people in Italy are affected by Myasthenia Grave,
00:23and it is a disease characterized by muscle fatigue,
00:28that is, the symptoms worsen with effort and improve with rest.
00:32It is a disease that can involve several muscle districts,
00:38it is a heterogeneous disease, it can involve the extraocular muscles,
00:42giving rise to diplopia, double vision, palpebral ptosis,
00:46the muscles that are important for phonation and chewing,
00:49therefore giving difficulty in speaking, chewing, but also in swallowing,
00:53and it can also give fatigue to the arteries.
00:56The most severe event is when the muscles are involved in the respiratory tract,
01:03in this case the patient can go into respiratory failure
01:06and may require mechanical ventilation support and intensive care.
01:10From a therapeutic point of view, Myasthenia Grave is a disease
01:14that responds quite well to traditional therapy.
01:18However, there are about 10-15% of patients who do not respond to traditional therapies,
01:25which are inhibitors of acetylcholinesterase, corticosteroids,
01:31immunosuppressors such as azathioprine or mycophenolate.
01:35Moreover, there are several patients who suffer from side effects
01:40of the therapy with chronic corticosteroids.
01:43These patients can benefit from innovative therapies,
01:47among these, for example, Lefkartijimod,
01:49which is a fragment of an antibody that promotes the degradation of IgG pathogens,
01:55reducing the level of antibodies that cause the disease,
01:58such as the antibody specific to the receptor of acetylcholinesterase,
02:01thus improving the symptoms and reducing the disability associated with this pathology.