Treatment of newly diagnosed patients with myasthenia gravis at a regional hospital in years 2003–2013

Abstract

Izhodišča: Miastenija gravis (MG) je najpogostejša avtoimunska bolezen živčno-mišičnega stika. S sodobnimi zdravili in s podporo novejših metod invazivnega zdravljenja se je MG iz potencialno smrtne bolezni prelevila v eno najbolj terapevtsko vodljivih nevroloških bolezni. Novejše epidemiološke študije kažejo, da incidenca in prevalenca MG naraščata. To si razlagamo z večjim zavedanjem o obstoju bolezni, izboljšanimi možnostmi diagnosticiranja in zdravljenja ter s staranjem prebivalstva. Tako se je povečalo tudi število obravnav bolnikov na sekundarni ravni. O obravnavi MG na ravni Slovenije zaenkrat ni sistematično zbranih podatkov. Namen prispevka je predstaviti klinične značilnosti in retrospektivno analizo obravnave novodiagnosticiranih bolnikov z MG v Splošni bolnišnici Izola med 1. 1. 2003 in 31. 12. 2013.

Metode: Pregledali smo medicinsko dokumentacijo vseh novoodkritih bolnikov z MG, diagnosticiranih v izbranem obdobju v Splošni bolnišnici Izola, in zbrali podatke o demografskih značilnostih, pridruženih boleznih, obliki, kliničnemu poteku, zdravljenju in izidu zdravljenja MG.

Rezultati: Prepoznali smo 29 bolnikov (14 žensk in 15 moških). Povprečna starost bolnic je bila 59,4 ± 21,2 let, bolnikov pa 56,3 ± 19,8 let. V opazovani populaciji je 58,6 % bolnikov v krvi imelo izražena protitelesa za acetilholinske receptorje. Od pridruženih bolezni sta bili najpogosteje prisotne avtoimunske bolezni ščitnice in druge kronične bolezni. Večina bolnikov (68,9 %) je zbolela s prizadetostjo zunanjih očesnih mišic. Ta se je v 65 % primerov s časom razširila na skeletne mišice. Hudo poslabšanje MG v obliki miastenske krize je doživelo 10,3 % bolnikov. Bolniki so bili v 68,9 % zdravljeni izključno simptomatsko. Timektomiranih je bilo 13,7 % bolnikov. Ob zdravljenju je 13,7 % bolnikov doseglo popolnoma stabilno remisijo bolezni. Pri 55,1 % bolnikov se je stanje pomembno izboljšalo, pri 20,68 % bolnikov se je stanje le delno izboljšalo in pri 6,8 % bolnikov je stanje kljub zdravljenju ostalo nespremenjeno. Petletna smrtnost bolnikov, izračunana po metodi Kaplan Meier, je znašala 13,5 %.

Zaključki: Klinične značilnosti in izid zdravljenja novodiagnosticiranih bolnikov z MG v Splošni bolnišnici Izola so bile, razen glede smrtnosti, primerljive s podatki iz literature. Višja smrtnost bolnikov (domnevno zaradi neprepoznane miastenske krize) si razlagamo verjetno kot posledico premajhnega zavedanja, da je miastenska kriza s pravilnimi pristopi zdravljenja ne glede na starost bolnika terapevtsko dobro odzivno in potencialno ozdravljivo stanje.

Alternate abstract:

Background: Myasthenia gravis (MG) is the most common autoimmune disorders of the neuromuscular junction. Advances in intensive care in conjunction with the advent of novel treatments have made MG one of the most treatable neurological disorders. The incidence and prevalence rates of MG have increased over time due to greater disease awareness, better diagnostic methods and ageing of the population. Due to these facts, the number of MG patients evaluated at the secondarylevel regional hospitals has increased. So far, no data on the evaluation of MG in Slovenia have been systematically collected. The aim and scope of this article is to present the analysis of the evaluation of newly diagnosed MG patients at the General hospital of Isola for the time period between 1 January 2003 and 31 December 2013.

Methods: We reviewed medical files of MG patients diagnosed at the General hospital of Isola in the above mentioned time period and collected data on patients’ demographic and clinical characteristics in addition to examining their clinical course and treatment outcome.

Results: Twenty-nine patients were identified (14 women, 15 men). Te mean age at disease onset was 59.4 ± 21.2 years for women and 56.3 ± 19.8 years for men. In the study population, 58.6 % of the patients were acetylcholine receptor antibody positive. Besides MG, patients most often had thyroid and other chronic diseases. Most patients (68.9 %) presented with extraocular disease symptoms, which progressed to generalized disease in 65 % of cases. Myasthenic crisis occurred in 10.3 % of patients. In 68.9 % of cases, the patients were treated exclusively with symptomatic therapy. Tymectomy was performed in 13.7 % of cases. On treatment, 13.7 % achieved a stable complete remission of MG symptoms, in 55 % the condition substantially improved, in 20.6 % improvement was only partial, and in 6.8 % of patients the condition remained unchanged. The fiveyear mortality of the study population, estimated according to the Kaplan-Meier method was 13.5 %.

Conclusions: With the exception of a higher mortality rate, the patients’ characteristics and clinical course of the disease were similar to data reported in other epidemiological studies. The patients’ higher mortality rate was considered to be a consequence of the lack of intensive treatment in patients with myasthenic crisis due to the unawareness of the reversibility of the condition with an adequate treatment.