The burden of disease in seronegative myasthenia gravis: a patient-centered perspective

Frontiers in Immunology, Journal Year: 2025, Volume and Issue: 16

Published: April 8, 2025

Myasthenia gravis (MG) is an autoimmune disorder primarily caused by autoantibodies against the acetylcholine receptor (AChR). Approximately 15% of MG patients, categorized as seronegative (snMG), lack detectable antibodies. Due to snMG status, there may be a diagnostic delay. Moreover, are limited data on treatment response in comparison AChR-Ab+ patients. This study examines burden disease, response, and quality life patients AChR-ab+ healthy controls. A questionnaire-based survey was conducted collecting sociodemographic clinical including antibody therapy, self-rated disease severity along with standardized assessments such MG-ADL (activities daily living) Short Form Health (SF-36, generic Health-Related Quality Life, HRQoL). HRQoL evaluated through matched-pairs analyses. Participants from general health served control group. Negative binomial regression applied evaluate impact status MG-ADL. Compared (n = 237) were younger at symptom onset [median age 42 (IQR 30.5/53) vs. 51 (31/64) years, p < 0.001] had longer delays. Complete stable remission less frequent (15.9% 27.8%, 0.001), they reported higher (52.8% medium, 9.5% severe 41.9% 8.5% severe, 0.005). scores 5 2/9) 3 (1/6), more employment restrictions (64.4% 49.3%, 0.001). Furthermore, compared controls, showed worse outcomes all domains SF-36. The due delay diagnosis, factors. These findings highlight challenges treating physicians face snMG. There high need for earlier improved tools, inclusion trials address their unique therapeutic challenges. clinicaltrials.gov, identifier NCT03979521. Registered 7 June 2019 (retrospectively registered).

Language: Английский

---

Serum fibrinogen is not elevated in patients with myasthenia gravis

Scientific Reports, Journal Year: 2025, Volume and Issue: 15(1)

Published: April 15, 2025

Myasthenia gravis (MG) is an autoimmune, autoantibody-mediated disease characterized by fatigable weakness of skeletal muscles. MG a heterogeneous that currently lacks robust biomarker for diagnosing all subtypes. Residual serum fibrinogen was found to be elevated 1000-fold in patients with one study and posited represent universal diagnostic MG. We set out confirm subtypes employed multiple methodologies compare levels between controls, using samples from independent cohorts. With enzyme-linked immunosorbent assay (ELISA), sera were not significantly different controls. And plasma samples, had lower amount compared Using liquid chromatography-mass spectrometry (LC-MS), the abundance fibrinogen-α fibrinogen-ß fibrinogen-γ Our results do support underscore need replication novel findings ensure our common goal identifying effective biomarkers

Language: Английский

---

Differences Between Females and Males in the Diagnostic Delay and Clinical Course of Thymectomised Myasthenia Gravis

European Journal of Neurology, Journal Year: 2025, Volume and Issue: 32(5)

Published: May 1, 2025

ABSTRACT Introduction Myasthenia gravis (MG) is an autoimmune disease presenting typically at earlier age in females compared to males. However, whether sex affects the diagnostic delay and clinical course of MG has not been extensively explored systematic cohorts. Methods We conducted a retrospective single‐center cohort study on 251 thymectomized patients, including 124 males 127 females. Mean follow‐up was 10.7 (±9.1) years post‐thymectomy. analyzed factors associated with longer type first‐onset symptoms (ocular vs. generalized). For 195 patients nonthymomatous generalized (gMG) pre‐thymectomy, we estimated effect delay, onset, reaching complete stable remission (CSR) or minimal need for medication (MNM) during postoperative follow‐up. also assessed their overall in‐hospital treatments, immunosuppressant use, pyridostigmine dose last visit. Results Generalized onset were more frequent than ( n = 90, 70.9% 65, 52.4%, respectively; p < 0.001). Diagnostic significantly (6.0 months [0–117.5] 3.2 [0.1–84.0]; 0.012). First‐onset did affect post‐thymectomy prognosis gMG. achieved CSR (17.1% 4.5%; 0.006) MNM (30.8% 16.9%; 0.029) frequently required fewer treatments (40.6% 55.2%; 0.010) immunosuppressants (29.2% 61.7%; 0.001) Conclusions report sex‐related differences length prognosis, origins which should be further studied.

Language: Английский

---

Clinical and Demographic Features of Distal Extremity Weakness in Myasthenia Gravis - A Scoping Review

medRxiv (Cold Spring Harbor Laboratory), Journal Year: 2024, Volume and Issue: unknown

Published: Aug. 17, 2024

Abstract Introduction Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease primarily affecting extraocular, bulbar, axial, and proximal extremity muscle groups. However, subgroup of patients experience distal weakness, known as myasthenia (DMG). Despite its longtime observation, DMG’s clinical demographic features remain poorly understood. This scoping review aims to summarise current evidence regarding the demographic, clinical, diagnostic MG who exhibit atypical, weakness. Methods We conducted by searching following electronic databases: Embase, Medline (Ovid), Scopus, Web Science, ResearchGate, covering period from their inception up August 2024. included all types publications due limited number articles available on DMG. Since we wanted work individual-level patient data, selected papers based our inclusion exclusion criteria, interpreted data descriptively. Results After screening initial 2162 publications, assessed 55 for eligibility. 23 (11 case reports, 5 letters editor, 2 poster abstracts, series) fulfilled criteria. Demographic details 43 were extracted further analysis. The male-to-female ratio was 1.4:1, with mean (SD) age onset at 40.2 ± 17.2 DMG 44.9 16.3. As 38% developed weakness onset, in total 55% showed symptoms within first year diagnosis. In women DMG, generalised often present (83%), whereas men had similar percentages ocular (52%) (48%) onset. most frequently reported weaknesses finger extension, followed wrist elbow abduction. Ankle dorsiflexion form lower Conclusion Distal (DMG) rare yet debilitating subtype gravis. When evaluating symptoms, it crucial consider various differential diagnoses resulting this context, would like emphasise two MG-associated conditions, namely myasthenia-inflammatory myopathy (MG-IM) myasthenic neuromyopathy. Our highlights need future prospective studies address unanswered questions improve outcomes quality life Highlights Significant muscles (3-7%) Finger extensors are affected Approximately experienced MG, disease. At 83% presented (48%). clinically

Language: Английский

---

Epidemiology, management and patient needs in myasthenia gravis: an Italian multistakeholder consensus based on Delphi methodology

BMJ Open, Journal Year: 2024, Volume and Issue: 14(12), P. e086225 - e086225

Published: Dec. 1, 2024

Objectives To provide comprehensive information on the burden of myasthenia gravis (MG) in Italy, including unmet needs patients and several other aspects related to disease, based skilled viewpoints MG experts. Design Iterative analysis conducted accordance with best practices Delphi method, anonymity, controlled feedback, statistical stability consensus. Setting participants 24 clinicians, 18 public health experts 4 patient associations completed all iterations between April 3 July 2023, for a total 46 from Italian Regions. Outcome measures Five areas investigation were examined: epidemiology Italy characteristics disease; diagnostic issues journey; during acute chronic phases MG; quality life; management MG. Consensus was defined by both percentage level agreement panellists or median value responses. Results We reported high (ie, >66.7% panellists) prevalence incidence disease issues. A strong impact life also emerged. Cross-agreement achieved among different subgroups representatives) most items proposed. Conclusions This study provided guidance educational practical highlighted severity its role patients’ life. few gaps handling

Language: Английский

---