Intravenous immunoglobulin in the treatment of neurologic disorders

Abstract

Intravenous immunoglobulins (IVIGs) are often used in the treatment of autoimmune disorders and immunodeficiencies, and it has been estimated that neurologic indications can account for up to 43% of IVIG used in clinical practice. In neurologic clinical practice, IVIG is used for acute therapy of newly diagnosed autoimmune disorders or exacerbations of pre-existing conditions, or as long-term maintenance treatment for chronic disorders. IVIG exerts its effects on humoral and cell-based immunity through multiple pathways, without a single dominant mechanism. Clinical use of IVIG has been supported by guidelines from American Academy of Neurology and European Federation of Neurologic Societies. IVIG is generally recommended for the treatment of Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy in adults, multifocal motor neuropathy and myasthenia gravis, and should be considered as a treatment option for dermatomyositis in adults and Lambert-Eaton myasthenic syndrome. Additional potential indications include stiff person syndrome, multiple sclerosis during pregnancy or while breastfeeding, refractory autoimmune epilepsy, and paraneoplastic disorders. Clinical use of IVIG is mostly safe but few adverse effects may still occur with potentially severe complications, including aseptic meningitis and thromboembolism. In addition to intravenous route (IVIG), subcutaneous immunoglobulins have been used as an alternative treatment option, especially in patients with limited intravenous access. Treatment with IVIG is effective in various autoimmune diseases, but its broader use is constrained by limited supply. This review evaluates the use of immunoglobulins in treatment of neurologic diseases.

Consenso Mexicano para la prescripción de inmunoglobulina G como tratamiento de reemplazo e inmunomodulación

“…Se desconoce su efecto en pacientes con anticuerpos anti-GAD negativos (GRADE B1). 144,[173][174][175][187][188][189] Encefalitis límbica autoinmunitaria R. Los pacientes pediátricos y adultos con alto índice de sospecha de encefalitis límbica autoinmunitaria deben recibir tratamiento inmunomodulador con IgG intravenosa (en dosis de 400 mg/kg/d por 5 días) y corticosteroides, plasmaféresis, o ambos. El 50% de los casos reacciona satisfactoriamente con la inmunoterapia de primera línea (IgG intravenosa, corticosteroides o plasmaféresis) y el 50% restante requiere tratamiento de segunda línea, con rituximab o la combinación de rituximab y ciclofosfamida.…”

“…La dosis de 2 g/kg entre 2 y 5 días consecutivos ha reportado resultados variables (GRADE D2). [185][186][187][188][189][190][191][192][199][200][201][202][203][204][205][206] Esclerosis múltiple R. La indicación de IgG por vía intravenosa en pacientes con esclerosis múltiple se considerada un tratamiento de segunda línea en algunos países; sin embargo, en general no se encuentra indicada. No obstante, es el único inmunomodulador disponible durante el embarazo (GRADE C2).…”

Guía colombiana de práctica clínica para el uso de inmonuglobulinas en el tratamiento de reemplazo e inmunomodulación

“…La dosis recomendada es de 2 g/kg a lo largo de 2 días. (GRADE B2) 158,181,182,183,184,185,186,187,188,189,190,191 R El uso de IgIV puede considerarse en pacientes pediátricos con esclerosis múltiple si los corticoides están contraindicados o cuando se intenta reducir la dosis de los mismos en pacientes con frecuentes exacerbaciones. (GRADE B2) 192 Algunos estudios sugieren que la IgIV puede disminuir la actividad de la enfermedad en los adultos, incluyendo la frecuencia de recaídas.…”

Intravenous immunoglobulin in paediatric neurology: safety, adherence to guidelines, and long‐term outcome

“…1 Supplementation of the immune system with IVIG broadens the spectrum of a recipient's immune response and attenuates autoimmune reactivity, 1 although the precise mechanisms of anti-inflammatory and immunoregulatory action are not completely understood, and are thought to be diverse according to the underlying pathophysiology. [1][2][3] The demand for IVIG has increased over the years, resulting in high cost to health providers and IVIG shortages. 2 Guidelines regulating IVIG use according to the evidence base have been created in different countries, to ensure its availability for patients who are most likely to benefit from the therapy.…”

“…Intravenous immunoglobulin (IVIG) is a fractionated blood product made from pooled human plasma, that has been used in the treatment of immune deficiencies and autoimmune disorders for almost four decades. 1 Supplementation of the immune system with IVIG broadens the spectrum of a recipient's immune response and attenuates autoimmune reactivity, 1 although the precise mechanisms of anti-inflammatory and immunoregulatory action are not completely understood, and are thought to be diverse according to the underlying pathophysiology. [1][2][3] The demand for IVIG has increased over the years, resulting in high cost to health providers and IVIG shortages.…”