In our coverage of myasthenia gravis this year, the top developments show the effects of COVID-19 continue to be uncovered, a new FDA approval for generalized disease, and assessing patient outcomes following uncontrolled disease and respiratory distress.
Our top news for myasthenia gravis (MG) this year included 2 case reports of potentially COVID-19–induced disease, which show the effects of the SARS-CoV-2 virus are still being uncovered; the FDA approval of rozanolixizumab-noli; why uncontrolled disease is a persistent issue in the MG space; and a novel test to measure respiratory function and assess respiratory muscle weakness, which can be a life-threatening complication of MG.
We hope you enjoy these 2023 highlights, and please visit our MG page for even more news.
5. Uncontrolled Disease Common in Patients Grappling With Myasthenia Gravis
This real-world study published in Orphanet Journal of Rare Disease explored why patients who have MG often have chronically uncontrolled disease, comparing outcomes between 5 European countries and confirming previous findings from the United States. Principal findings included that approximately a quarter of patients received a misdiagnosis, two-thirds of patients were severely symptomatic even after treatment, and diagnosing physicians were not always neurologists. The findings highlighted a need for more therapies to treat the chronic autoimmune disorder and that health care providers beyond neurologists need more education on MG.
4. COVID-19 May Accelerate Onset of Myasthenia Gravis, Case Study Suggests
In this first of 2 articles detailing a potential connection between COVID-19 and MG, a case report of a female patients discussed how she developed MG within 2 weeks of COVID-19 infection. Among her MG symptoms, she had fluctuating double vision, moderately slurred speech, and drooping of the right upper eyelid; her Myasthenia Gravis Composite score was 26/50. Two subsequent hospitalizations led to removal of her thymus gland and a biopsy, which showed diffuse lympho-follicular hyperplasia. The report’s authors noted that among previous reports of patients with COVID-19–induced MG, the principal presenting symptom in generalized muscle weakness.
3. Single-Count Breath Test Can Help Assess Respiratory Function in MG
Respiratory muscle weakness is a potentially life-threatening complication of MG. At present, the standard of care is to use forced vital capacity (FVC) to evaluate respiratory function in this patient population; however, equipment access is difficult for some and facial muscle weakness can make it difficult to adequately administer the test. Investigators evaluated the potential of the single-count breath test (SCBT), which may be easier for patients to take vs FVC, since no equipment is required; instead they only need to sit upright, take a deep breath, and count upward using their normal speaking voice. Overall, the SCBT had a moderate correlation with FVC, meaning the test has potential, but the authors said additional evaluation is needed to validate their findings.
2. FDA Approves Rozanolixizumab-noli for Generalized Myasthenia Gravis
In June, the FDA handed down this approval for adult patients who have generalized MG and are anti–acetylcholine receptor or anti–muscle-specific tyrosine kinase antibody positive, making rozanolixizumab-noli the only FDA-approved treatment for this type of MG. Data from the phase 3 MycarinG study supported this decision, which was granted under the Priority Review designation. Two hundred patients were enrolled in the phase 3 study at sites in Asia, Europe, and North America, with patients randomized to rozanolixizumab 7 or 10 mg/kg or placebo. Myasthenia Gravis Activities of Daily Living score reductions were greater in both rozanolixizumab groups, and although these patients had more treatment-emergent adverse events vs the placebo group, most were mild to moderate.
1. Case Investigated of Patient With Possible COVID-19–Induced Myasthenia Gravis
In August, this case report was published of a patient with COVID-19–induced seronegative MG. He initially presented to the emergency department with shortness of breath and dry cough and a medical history that included hypertension, morbid obesity, obstructive sleep apnea, and interstitial lung disease. Proximal muscle weakness and ptosis were found after the patient could not be weaned off mechanical ventilation, and the neurology team that was brought in found him positive for antibodies against lipoprotein-related protein 4 but negative for anti-AChR autoantibodies and anti–MuSK-MG, “suggesting double-negative MG.” The patient was also on azithromycin for suspected pneumonia, but this medication is not recommended in patients who have underlying MG. More investigation is needed, the authors, said, into COVID-19–induced MG.
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