Complications
A serious idiosyncratic allergic reaction that includes rash, fever, nausea, vomiting, and abdominal pain occurs in 10% to 15% of patients within the first 3 weeks of treatment.[45][46] The reaction resolves within 24 hours of stopping azathioprine and recurs with rechallenge.
Cytopenias occur commonly and an FBC should be obtained 2 weeks after a dose increase, and then every 4-6 weeks when on a stable dose.
Drug-induced hepatitis may also occur, and monitoring of liver enzymes every 2 months is reasonable.
Oligospermia and infertility: may be seen in men, and is generally reversible on discontinuing therapy.
Seizures may occur with doses >100 mg/day; patients with known seizures should not receive amifampridine.[54] If a seizure occurs, amifampridine should be discontinued, or the dose should be reduced.
All patients taking supraphysiological doses of corticosteroids are at increased risk for developing osteoporosis and should be evaluated for other risk factors.
Supplemental calcium and vitamin D, and consideration of bisphosphonate therapy may be appropriate.
As most patients require long-term immunosuppressive treatment, and corticosteroids are a mainstay of treatment, corticosteroid-related adverse effects may result in substantial morbidity. These include cataracts, insulin resistance and type 2 diabetes mellitus, and infection.
Patients with swallowing impairment are at high risk of aspiration. Impaired swallowing occurs in nearly half of patients with Lambert-Eaton myasthenic syndrome, which increases the risk of aspiration and may require admission for either intravenous immunoglobulin or plasma exchange.[1]
Subsequent respiratory infections, respiratory distress syndrome, and respiratory failure remain a major cause of morbidity and mortality.
If swallowing difficulties are reported, obtaining a swallow study and consulting a speech pathologist may be considered. Food consistency may need to be modified and thickeners may be added to liquids if thin liquids elicit choking. Eating and drinking should be done in an upright position to minimise aspiration risk.
Impaired swallowing may lead to aspiration and secondary pneumonia.
Rarely occurs in patients with Lambert-Eaton myasthenic syndrome, although may be provoked by infections, medications, surgery, or trauma. Serial measurements of FVC and negative inspiratory force should be taken using an appropriate interface for patients with severe bulbar weakness. Indication for mechanical ventilation includes FVC of ≤15 mL/kg and negative inspiratory force of ≤20 cm H₂O. Neither abnormal ABGs nor pulse oxygenation may reflect the degree of respiratory weakness because abnormalities in either occur late in the course after clinical decompensation.
Treatment focuses on providing appropriate respiratory support, rapidly improving neuromuscular function with either intravenous immunoglobulin or plasma exchange, and identifying and removing any provoking factors.
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