Myasthenia Gravis in an HIV-Infected Woman
Myasthenia Gravis in an HIV-Infected Woman
A 33-year-old woman was found to be HIV-infected and diagnosed with myasthenia gravis by a neurologist in 1999. She was treated with pyridostigmine (60 mg 5 times daily) but had a myasthenia crisis in May 2001 precipitated by pneumonia. She survived the crisis on prednisolone (short course), pyridostigmine, mechanical ventilation, antibiotics, and physiotherapy. She is currently taking pyridostigmine (640 mg/day in divided doses) but her fatigue is worsening even with dose reduction. She has no thymoma on CT scan and has never had highly active antiretroviral therapy (HAART). Her CD4+ cell count and viral load have not been measured recently. Please advise regarding her management. I am considering long-term prednisolone but will I then need to initiate HAART?
The first consideration in the management of any patient with suspected neurologic disease is confirmation of the diagnosis. I will assume that the diagnosis of myasthenia gravis in this patient is supported by appropriate clinical and electrophysiologic (ie, repetitive stimulation and single-fiber EMG) features and antiacetylcholine receptor antibody assay results.
There are only a few case reports of the association of myasthenia gravis and HIV infection. Given their infrequency, it is likely that these cases represent chance co-occurrence of common diseases, rather than a true association. In one of these cases, the clinical course of myasthenia gravis improved over time, associated with reductions in the CD4+ cell count and antiacetylcholine receptor antibody levels. This led the authors to speculate that the immunologic changes resulting from HIV infection may have affected the natural history of myasthenia gravis. It would be of interest to note the relationship between immune status and the clinical course of myasthenia in the patient under discussion.
The management of myasthenia gravis in the setting of HIV infection is not clearly different from that in an HIV-uninfected patient. While the immunologic compromise in HIV infection necessitates caution in the use of immunosuppressant therapies, corticosteroids have been used relatively safely in HIV-infected subjects in several diseases. These include Pneumocystis carinii pneumonia (relatively short-course corticosteroids), and several neurologic disorders including HIV myopathy and inflammatory demyelinating polyneuropathy. The safety of other approaches typically employed in myasthenia gravis, such as thymectomy or cytotoxic immunosuppressants (ie, azathioprine), is less clear and they should probably be avoided.
There have been several reports suggesting that the use of corticosteroids may slightly increase the risk of opportunistic infections in HIV-infected subjects. Thus, while CD4+ cell count and HIV-1 RNA levels are not available for this patient, the use of immunosuppressant therapy such as corticosteroids would add further weight to the use of a HAART regimen.
A 33-year-old woman was found to be HIV-infected and diagnosed with myasthenia gravis by a neurologist in 1999. She was treated with pyridostigmine (60 mg 5 times daily) but had a myasthenia crisis in May 2001 precipitated by pneumonia. She survived the crisis on prednisolone (short course), pyridostigmine, mechanical ventilation, antibiotics, and physiotherapy. She is currently taking pyridostigmine (640 mg/day in divided doses) but her fatigue is worsening even with dose reduction. She has no thymoma on CT scan and has never had highly active antiretroviral therapy (HAART). Her CD4+ cell count and viral load have not been measured recently. Please advise regarding her management. I am considering long-term prednisolone but will I then need to initiate HAART?
The first consideration in the management of any patient with suspected neurologic disease is confirmation of the diagnosis. I will assume that the diagnosis of myasthenia gravis in this patient is supported by appropriate clinical and electrophysiologic (ie, repetitive stimulation and single-fiber EMG) features and antiacetylcholine receptor antibody assay results.
There are only a few case reports of the association of myasthenia gravis and HIV infection. Given their infrequency, it is likely that these cases represent chance co-occurrence of common diseases, rather than a true association. In one of these cases, the clinical course of myasthenia gravis improved over time, associated with reductions in the CD4+ cell count and antiacetylcholine receptor antibody levels. This led the authors to speculate that the immunologic changes resulting from HIV infection may have affected the natural history of myasthenia gravis. It would be of interest to note the relationship between immune status and the clinical course of myasthenia in the patient under discussion.
The management of myasthenia gravis in the setting of HIV infection is not clearly different from that in an HIV-uninfected patient. While the immunologic compromise in HIV infection necessitates caution in the use of immunosuppressant therapies, corticosteroids have been used relatively safely in HIV-infected subjects in several diseases. These include Pneumocystis carinii pneumonia (relatively short-course corticosteroids), and several neurologic disorders including HIV myopathy and inflammatory demyelinating polyneuropathy. The safety of other approaches typically employed in myasthenia gravis, such as thymectomy or cytotoxic immunosuppressants (ie, azathioprine), is less clear and they should probably be avoided.
There have been several reports suggesting that the use of corticosteroids may slightly increase the risk of opportunistic infections in HIV-infected subjects. Thus, while CD4+ cell count and HIV-1 RNA levels are not available for this patient, the use of immunosuppressant therapy such as corticosteroids would add further weight to the use of a HAART regimen.
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