The evaluation of approaches to the treatment of myasthenia gravis.

Анотація

Myasthenia gravis is a relatively rare autoimmune disease with an undetermined aetiology which affects neuromuscular junctions. Currently, the following approaches to the treatment of myasthenia gravis are mainly distinguished: symptomatic treatment with anticholinesterase inhibitors (AChEIs), immunomodulatory therapy (“basic” therapy) with glucocorticoids, cytostatics, monoclonal antibodies; surgical treatment — thymectomy; short-term treatment with plasmapheresis and intravenous administration of immuno­globulin. The efficiency of treatment approaches to myasthenia gravis in Ukraine remains insufficiently studied. The purpose of this work is to analyse the therapeutic approaches in patients with myasthenia gravis depending on the clinical form and severity of the disease. Between 2014 and 2017, 182 patients with myasthenia gravis have been examined, out of which 147 (80.8%) were the patients with the generalized form of the disease and 35 (19.2%) — with its ocular form. The clinical neurological examination included the collection of complaints, an anamnesis of disease and life as well as a neurological examination. In all the patients, the level of antibodies to acetylcholine receptors (AchR) and to muscle-specific tyrosine kinase (MuSK) has been measured, in terms of quantity as well, using the enzyme-linked immunosorbent assay (ELISA), and the presence of antibodies to titin and SOX1 has also been detected by means of indirect immunofluorescence. Of the total sample, less than a third (28.0%) of the patients examined received basic therapy; among them, there were no patients with the ocular form and only 34.7% — with the generalized form (p<0.001). Basic therapy is found more often among the patients with class II myasthenia gravis (51.9%), with a statistically significant (p<0.001) higher share of the patients receiving such a therapy than in classes III and IV (26.6% and 22.6% respectively). The structure of therapy in patients with classes III and IV has not shown any statistically significant difference (p=0.658), with symptomatic treatment being the predominant type of therapy. Undergoing basic therapy reduces the chances of a severe clinical course of myasthenia gravis (the QMG score of 17 and higher) — OR=0.52 (95.0% CI 0.14-0.90), p=0.032; fatal cases of the disease — OR=0.36 (95.0% CI 0.02-0.70), p=0.049. When basic therapy is used, the survival rate of the patients (Figure 2) is 42.0 years on average (95% CI 42.0-42.7) which is considerably higher (p=0.021) compared to that of the patients receiving symptomatic treatment only — 33.0 years (95% CI 30.9-36.7). Immunomodulatory therapy was prescribed for only 28% of the patients in the total sample, for none of the patients with the ocular form of myasthenia gravis, and for 34.7% of the patients with the generalized myasthenia gravis. The prescription of immunomodulatory therapy reduces relative risks of a severe clinical course of myasthenia gravis — OR=0.52 (95.0% CI 0.14-0.90), p=0.032. The prescription of immunomo­dulatory therapy decreases the probability of a fatal outcome of the disease — OR=0.36 (95.0% CI 0.02-0.70), p=0.049). With the use of immunomodulatory therapy, the patient survival rate rises considerably reaching an average of 42.0 years (95% CI 42.0-42.7), which is much higher compared to that in the group of the patients receiving symptomatic treatment only — 33.0 years (95% CI 30.9-36.7), p=0.021.