Susaс’s syndrome is an autoimmune inflammatory endotheliopathy at the confluence of neurology, ophthalmology, and otology

Background. Susaс’s syndrome is a rare autoimmune vascular-occlusive disease, which is accompanied by damage to the endothelium of blood vessels by circulating antibodies and leads to multiple microinfarcts. This pathological process is characterized by damage to the small arterial vessels of the central nervous system, retina, and inner ear, which is manifested by the classic triad of the following symptoms: encephalopathy, retinal artery occlusion, and sensorineural hearing loss. In addition, Susaс’s syndrome is characterized not only by the classic triad of symptoms, but also by the classic triad of neuroimaging signs, namely: damage to the white matter of the brain, the presence of foci in the deep structures of the gray matter, and leptomeningeal damage. To date, this disease remains insufficiently studied, and therefore the exact frequency and prevalence of this disease have not been definitively established. This process is complicated by frequent diagnostic errors and cases of incorrect diagnosis. This is due to the fact that in most patients a complete combination of clinical and neuroimaging signs at the onset of the disease is rare, and the symptoms of target organ damage are manifested by variable and non-specific signs. Purpose – еarly detection and timely adequate treatment of this pathology are key to preventing irreversible damage and improving the prognosis and quality of life of patients. This work is aimed at increasing the vigilance of ophthalmologists, neurologists and otolaryngologists, as specialists who are most often encountered in clinical practice with this syndrome, since Susak’s syndrome requires rapid and aggressive treatment with the use of corticosteroids, intravenous immunoglobulin, and in the case of its fulminant course, the use of monoclonal antibodies and cytostatics. Materials and Methods. Based on a review of current data on the topic of our study, we analysed the original articles, literature, meta-analyses and book chapters. We used clinical and neurological, biochemical, neuroimaging and audiometric methods of research. Results. Diagnosis of Susak’s syndrome requires a comprehensive approach, which includes a carefully collected medical history, a qualitative physical examination, as well as additional examination methods such as MRI of the brain, audiometry, and ophthalmological examination. Conclusions. Despite numerous studies, growing awareness of doctors, progress in diagnosis and treatment, Susak’s syndrome still remains a unique challenge for the attending physician due to nonspecific symptoms, lack of specific diagnostic markers, and universal treatment protocols. That is why vigilance when detecting typical neurological, ophthalmological or otological symptoms and interdisciplinary involvement of specialists of these profiles are so important to improve the results of treatment of such a complex disease.