Outcome measures and treatment effectiveness in late onset myasthenia gravis
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(2020) 2:45
Neurological Research and Practice
RESEARCH ARTICLE
Open Access
Outcome measures and treatment effectiveness in late onset myasthenia gravis Francesca Pasqualin1 , Silvia V. Guidoni1, Mario Ermani2, Elena Pegoraro2 and Domenico M. Bonifati1*
Abstract Background: Recently different subtypes of myasthenia gravis (MG) have been described. They differ for clinical features and pathogenesis but the prognosis and response to treatment is less clear. The aim of the study was to evaluate outcome and treatment effectiveness including side effects in late onset MG (LOMG) compared with early onset MG (EOMG). Methods: We analysed retrospectively 208 MG patients. Clinical features were recorded as well as treatment and side effects. Outcome at the last follow-up was evaluated with MGSTI and MGPIS scales. Results: The 208 patients included were classified as follow: 36 ocular MG, 40 EOMG, 72 LOMG, 25 thymomaassociated, 14 anti-MuSK and 21 double seronegative. Similar positive outcome was achieved in either early and late onset subgroup. We found pharmacological remission and minimal manifestations at the MGFA-PIS in the 95% and 94,4% of EOMG and LOMG respectively but in LOMG a lower dose of immunosuppressors (MGSTI< 2) was required compared to EOMG (p = 0,048). Severe side effects were present in a small percentage of patients in both group but diabetes was more frequent in LOMG vs EOMG (2,2% vs 5%, p = 0.017). Conclusions: Despite LOMG has more comorbidities that might interfere with treatment and outcome, therapeutic management does not seem to differ between EOMG and LOMG. A similar positive outcome was seen in both subgroups but LOMG group seems to require lower doses of medication to control symptoms. Keywords: MGFA-PIS, MGSTI, Myasthenia gravis, Characterization, Outcome, LOMG
Introduction Myasthenia gravis (MG) is an autoimmune disorder due to antibodies against post-synaptic membrane proteins. Different subgroups have been described. The most accepted classification divides myasthenia gravis into the following subgroups: ocular; thymoma-associated; early onset and late-onset generalized with antibodies against acetylcholine receptor (AChR), anti-muscle specific tyrosine kinase (MuSK) positive and double seronegative [1]. They differ for clinical features and probably pathogenesis
* Correspondence: [email protected] 1 Ospedale regionale Ca’ Foncello, Unit of Neurology, 31100 Treviso, Italy Full list of author information is available at the end of the article
but prognosis and response to treatment in different subgroups is less clear. Treatment strategies have changed in time with improved knowledge of pathogenic mechanisms and introduction of new medications. Nowadays treatment includes cholinesterase inhibitors (symptomatic treatment), steroids, immunosuppression and thymectomy. Therapy is often empirical because very few drugs have undergone randomised double blind clinical trial. Guidelines are mainly based on observational retrospective and prospective studies or expert opin
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