Treatment of Immune-Mediated Conditions

Introduction to IVIG Therapy

IVIG can be used to achieve remission in patients with vasculitic neuropathy who fail to achieve remission or have persistent disease activity, as recommended by the EULAR guidelines 1
The EULAR and American College of Rheumatology/Vasculitis Foundation guidelines include IVIG as a treatment option for various immune-mediated conditions, such as polyarteritis nodosa and relapsing, refractory or persistent disease 1, 2

IVIG can be administered at a dose of 0.4 g/kg/day, with a total dose of 2 g/kg divided over 5 days, and repeat courses considered if clinical deterioration occurs after initial improvement, with a high strength of evidence level 3, 4, 5
Standard dosing for maintenance therapy is 0.4-1g/kg every 3-4 weeks, as suggested by the European League Against Rheumatism (EULAR) 6, 7, 8, 3
Alternative treatment options, such as plasma exchange (200-250 ml plasma/kg in five sessions), can be considered if IVIG is unavailable, as per ASCO guidelines 3, 5

Frequent neurological assessments should be performed to track progression, monitoring vital capacity, negative inspiratory force, oxygen saturation, heart rate, blood pressure, bowel/bladder function, and swallowing to evaluate for bulbar dysfunction and aspiration risk, as recommended by the American Academy of Neurology 3
Autonomic dysfunction should be monitored and managed by tracking cardiac arrhythmias and blood pressure fluctuations, with preventive measures for deep vein thrombosis and pressure ulcer prevention, as recommended by the American Academy of Neurology 3
Comprehensive neurological evaluation should assess for large-fiber function and small-fiber symptoms, as recommended by the American Diabetes Association 9
Baseline neurological assessment and frequent neurological assessments during treatment are necessary to monitor response and adjust therapy as needed, with a high strength of evidence level 4, 5

Exercise intensity should be monitored to prevent fatigue and overexertion during rehabilitation, according to the American Academy of Neurology 3
Continued monitoring after ICU discharge is essential, as up to two-thirds of deaths occur during the recovery phase, as noted by the American Academy of Neurology 3
Patients should be admitted to a hospital with capability for rapid transfer to ICU-level monitoring, with frequent neurological assessments and pulmonary function monitoring implemented, as per ASCO guidelines 5

Serum IgA level must be checked before IVIG administration to minimize the risk of adverse reactions, such as fever, infusion reactions, and anaphylaxis, as recommended by the Mayo Clinic 8
IVIG carries significant risks, including thromboembolic events, renal dysfunction, headache, and aseptic meningitis, with a high cost of approximately $40,000 per treatment course, as noted by 4, 5, 10

About 80% of patients regain independent walking ability by 6 months, and mortality rate is 3-10%, most commonly due to cardiovascular and respiratory complications, according to the American Academy of Neurology 3
Treatment-related fluctuations occur in about 10% of patients, and repeating IVIg may be considered in these cases, as suggested by the American Academy of Neurology 3