MuSK myasthenia gravis as a manifestation of immune restoration disease in an HIV-positive patient

Ragunathan, Karthik; Pathak, Bandana; Dahal, Kumud

doi:10.1007/s00415-015-7639-1

Cited by 9 publications

(5 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…MG, including anti-MuSK related MG, occurring during immune reconstitution has been reported (6,13,39,40). Ten patients in our cohort who were commenced on ARTs after the diagnosis of MG showed transient worsening of their MG in the initial stages of commencing ARVs.…”

Section: Discussionmentioning

confidence: 65%

A Comparison of Clinical, Electro-Diagnostic, Laboratory, and Treatment Outcome Differences in a Cohort of HIV-Infected and HIV-Uninfected Patients With Myasthenia Gravis

2021

View full text Add to dashboard Cite

There is limited literature comparing the clinical parameters and treatment outcomes in HIV-infected and HIV-uninfected myasthenia gravis (MG) patients. The aim of the study was to investigate the clinical differences and treatment outcomes in the two categories of patients, particularly the safe use of immunosuppressive therapy in immunocompromised patients. The study was a retrospective analysis of medical records of MG patients from the neuromuscular unit at Inkosi Albert Luthuli Central Hospital in Kwa-Zulu Natal between 2003 and 2019. One hundred and seventy-eight (178) patients fulfilled the clinical criteria for MG. Twenty-four (13.4%) were HIV-infected and 154 (86.5%) were HIV-uninfected. There were 116 (65%) females, median 45 years, (IQR 40–62), 90 (50.5%) black African, 66 (37%) Indian, 20 (11.2%) white, and 2 (1.1%) of mixed ancestry. In the HIV-infected cohort, 20 (87%) had generalized MG, 12 (50%) bulbar, and 14 (60.9%) respiratory onset MG, 12 (50%) presented with MG Foundation of America (MGFA) class five diseases at diagnosis, six (25%) presented with MG crisis during the 5-year follow-up. Thirteen (54%) of the HIV-infected group required rescue therapy using (plasma exchange or IV immunoglobulin) combined with pulse cyclophosphamide compared with 17 (11%) in the HIV-uninfected cohort, respectively. At 5 years, 8 (33%) of the HIV-infected group remained refractory to treatment compared with 10 (6.5%) HIV-uninfected cohort, respectively. No adverse events were documented in HIV-infected patients receiving combination rescue therapy (PLEX or IVIG combined with IV cyclophosphamide). In conclusion HIV-infected MG patients are more likely to require combination rescue therapy with PE/IVIG and IV cyclophosphamide compared with those who were HIV-uninfected. No side effects were documented in the HIV-infected group receiving the above therapy.

show abstract

Section: Discussionmentioning

confidence: 65%

A Comparison of Clinical, Electro-Diagnostic, Laboratory, and Treatment Outcome Differences in a Cohort of HIV-Infected and HIV-Uninfected Patients With Myasthenia Gravis

2021

View full text Add to dashboard Cite

show abstract

“…Our patient experienced typical symptoms of eyelid ptosis and progressive weakness, numbness, and stiffness from the lower extremities to the cervical muscles, but was negative for common MG antibodies, which partially caused the difficulty with the initial diagnosis. In the adult Chinese MG population, AChR, MuSK, Titin and RyR antibody levels are reported to be 82.2%, 2.3%, 28.4% and 23.8%, respectively [12], and which are also present in IRIS‐MG, especially MuSK [5, 7, 13, 14]. Interestingly, our patient was found to have thymus hyperplasia, which is common in HIV‐negative patients but rarely found in HIV‐positive patients with MG [15].…”

Section: Discussionmentioning

confidence: 83%

“…Wirtz et al also described a patient who developed MG during ART with positive MuSK antibody [18]. In contrast to the lower incidence of reported MuSK antibody in HIV-negative patients, the MuSK antibody was more frequently detected in HIV-infected patients at the time of IRIS [4,6,13,14,19]. Sherpa et al proposed that patients with MuSK-related MG have a much lower frequency of thymic pathology than patients with AChR-Ab(+) MG due to no thymus enlargement in reported patients with MuSK MG [14].…”

Section: Discussionmentioning

confidence: 99%

Comorbidity of Myasthenia gravis and Graves' disease as immune reconstitution‐associated autoimmune disease in HIV infection: A case report and literature review

Lu,

Ma,

Cong

et al. 2023

HIV Medicine

View full text Add to dashboard Cite

BackgroundComorbidity of Myasthenia gravis (MG) and Graves' disease (GD) in treated HIV‐infected individuals has rarely been described and little study has been done on the link between HIV‐related immune reconstitution and autoimmune diseases occurring post antiretroviral therapy.Case PresentationHere we report on a 33‐year‐old Chinese man with HIV infection who had been virologically suppressed since 2018. The patient was diagnosed with GD and was treated in 2020. Early in 2022, he developed fluctuating weakness and fatigue involving the bilateral extraocular muscles and limbs. With a positive neostigmine test, he was considered to have MG, but showed a poor response to oral medication. After multiple failed medication attempts, a thymectomy was finally performed to resolve his symptoms. The consecutive onset of immunological events may have partially resulted from immune reconstitution after viral control.ConclusionsThis is a rare case of HIV‐related immune reconstitution‐associated autoimmune disease (IRAD) with comorbidity of MG and GD which was reported initially. Cooperation with multidisciplinary teams is essential to avoid misdiagnosis and to promote the overall health of HIV‐infected patients.

show abstract

“…Immunosuppressants and immunomodulators have been successfully used in the treatment of MuSK MG in HIV patient [4–6]. As in other cases of myasthenia gravis with worsening symptoms and impending respiratory failure, our patient was managed with IVIg, which improved her symptoms.…”

Section: Discussionmentioning

confidence: 88%

“…Very little is known about this association, mostly through case reports [3]. Comorbid HIV and MuSK MG is even rarer, with only 3 cases previously reported [4–6]; these cases are summarized in Table 1. Clinical features of MuSK resemble AChR-Ab myasthenia, but these patients may additionally have increased incidence of diplopia, ptosis, dysarthria, and prominent respiratory muscle weakness, and are less responsive to acetylcholinesterase inhibitors [7].…”

Section: Discussionmentioning

confidence: 99%

Comorbid Human Immunodeficiency Virus (HIV) and Muscle-Specific Kinase (MuSK) Myasthenia Gravis: A Case Report and Literature Review

et al. 2017

View full text Add to dashboard Cite

Patient: Female, 44Final Diagnosis: MuSK myasthenia gravisSymptoms: Difficulty swallowing • double visionMedication: —Clinical Procedure: —Specialty: NeurologyObjective:Rare co-existance of disease or pathologyBackground:HIV infections with concomitant immunologically-mediated disorders have been frequently described but there has been little research on the association between HIV and myasthenia gravis.MuSK myasthenia gravis coexisting with HIV is an even a rarer entity and can occur as a part of immune restoration disease. We report the case of a patient with asymptomatic HIV infection who presented with new-onset MuSK myasthenia gravis.Case Report:A 44-year-old African-American woman with HIV since 2004 and on highly active antiretroviral therapy (HAART) presented to the ED with complains of double vision and difficulty swallowing for 2 weeks. The patient was intermittently on HAART therapy. On examination, she had bilateral ptosis, weak orbicularis oris and orbicularis oculi, along with mild lateral gaze palsy of the left eye. Her CD4 count was 383 and the viral load was undetectable. An MRI of the brain produced normal results and a CT chest did not show thymus enlargement. Due to worsening symptoms and high suspicion for myasthenia gravis, she was started on IVIG at 0.4 mg/kg/day for 5 days, and her symptoms markedly improved. She was found to have strongly positive MuSK antibody and negative Ach receptor antibody. Repetitive nerve stimulation showed a 13% decrease in the right median nerve, which confirmed the diagnosis. She was subsequently discharged to home on pyridostigmine. Azathioprine was added at clinic follow-up. The patient continues to improve.Conclusions:As the use of antiretroviral therapy increases, immune reconstitution syndromes have become more common. Rare associations like HIV and MuSK myasthenia gravis are being increasingly reported. The use of immunosuppressants in the treatment of these conditions should be carefully evaluated.

show abstract

MuSK myasthenia gravis as a manifestation of immune restoration disease in an HIV-positive patient

Cited by 9 publications

References 5 publications

A Comparison of Clinical, Electro-Diagnostic, Laboratory, and Treatment Outcome Differences in a Cohort of HIV-Infected and HIV-Uninfected Patients With Myasthenia Gravis

A Comparison of Clinical, Electro-Diagnostic, Laboratory, and Treatment Outcome Differences in a Cohort of HIV-Infected and HIV-Uninfected Patients With Myasthenia Gravis

Comorbidity of Myasthenia gravis and Graves' disease as immune reconstitution‐associated autoimmune disease in HIV infection: A case report and literature review

Comorbid Human Immunodeficiency Virus (HIV) and Muscle-Specific Kinase (MuSK) Myasthenia Gravis: A Case Report and Literature Review

Contact Info

Product

Resources

About