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The burden of disease in seronegative myasthenia gravis: a patient-centered perspective
Sophie Lehnerer1,2,3,4, Regina Stegherr5, Ulrike Grittner4,5
1Department of Neurology with Experimental Neurology, Freie Universität Berlin and Humboldt-Universität zu Berlin, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Objective:
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 the snMG status, there may be a diagnostic delay. Moreover, there are limited data on treatment response in comparison to AChR-Ab+ patients. This study examines the burden of disease, treatment response, and quality of life of snMG patients in comparison to AChR-ab+ MG patients and healthy controls.
Methods:
A questionnaire-based survey was conducted collecting sociodemographic and clinical data including antibody status, therapy, treatment response, and self-rated disease severity along with standardized assessments such as MG-ADL (activities of daily living) and the Short Form Health (SF-36, generic Health-Related Quality of Life, HRQoL). HRQoL was evaluated through matched-pairs analyses. Participants from a general health survey served as the control group. Negative binomial regression was applied to evaluate the impact of antibody status on MG-ADL.
Results:
Compared to AChR-ab+ patients, snMG patients (n = 237) were younger at symptom onset [median age 42 (IQR 30.5/53) vs. 51 (31/64) years, p < 0.001] and had longer diagnostic delays. Complete stable remission was less frequent in snMG patients (15.9% vs. 27.8%, p < 0.001), and they reported higher disease severity (52.8% medium, 9.5% severe vs. 41.9% medium, 8.5% severe, p = 0.005). snMG patients had higher MG-ADL scores [median 5 (IQR 2/9) vs. 3 (1/6), p < 0.001] and more employment restrictions (64.4% vs. 49.3%, p < 0.001). Furthermore, compared to healthy controls, snMG patients showed worse outcomes in all domains of the SF-36.
Conclusion:
The burden of disease in snMG patients is higher compared to AChR-ab+ MG due to delay in diagnosis, worse treatment response, and sociodemographic factors. These findings highlight the challenges patients and treating physicians face in snMG. There is a high need for earlier diagnosis, improved diagnostic tools, and inclusion of snMG patients in clinical trials to address their unique therapeutic challenges.
Clinical Trial Registration:
clinicaltrials.gov, identifier NCT03979521. Registered 7 June 2019 (retrospectively registered).
Insights
Seronegative myasthenia gravis (snMG) patients face greater disease burden, including diagnostic delays and poorer treatment response, compared to AChR-Ab+ patients. Earlier diagnosis and improved tools are crucial for snMG management.
Area of Science:
- Neurology
- Immunology
- Autoimmune Diseases
Background:
- Myasthenia gravis (MG) is an autoimmune disorder often linked to acetylcholine receptor (AChR) autoantibodies.
- A significant subset of MG patients, termed seronegative MG (snMG), lack detectable antibodies, potentially leading to diagnostic delays and limited treatment data.
- This study investigates the disease burden, treatment outcomes, and quality of life in snMG patients relative to AChR-antibody-positive (AChR-Ab+) MG patients and healthy controls.
Purpose of the Study:
- To compare the disease burden, treatment response, and health-related quality of life (HRQoL) between snMG patients and AChR-Ab+ MG patients.
- To assess the impact of snMG on daily living activities and employment.
- To highlight the challenges in diagnosing and treating snMG.
Main Methods:
- A questionnaire-based survey collected sociodemographic, clinical, and treatment data.
- Standardized assessments included the Myasthenia Gravis Activities of Daily Living (MG-ADL) scale and the Short Form 36 (SF-36) for HRQoL.
- Negative binomial regression analyzed the impact of antibody status on MG-ADL scores, with matched-pair analyses for HRQoL.
Main Results:
- snMG patients (n=237) were diagnosed at a younger age and experienced longer diagnostic delays compared to AChR-Ab+ patients.
- Complete stable remission was less frequent in snMG patients (15.9% vs. 27.8%).
- snMG patients reported higher disease severity, elevated MG-ADL scores (median 5 vs. 3), and more employment restrictions (64.4% vs. 49.3%), with worse SF-36 outcomes across all domains compared to healthy controls.
Conclusions:
- The disease burden in snMG is significantly higher than in AChR-Ab+ MG, attributed to diagnostic delays, suboptimal treatment response, and sociodemographic factors.
- These findings underscore the challenges faced by snMG patients and their physicians.
- There is a critical need for earlier diagnosis, enhanced diagnostic tools, and the inclusion of snMG patients in clinical trials to address their specific therapeutic needs.
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