(FDA approved in bold)

• Chronic inflammatory demyelinating polyneuropathy (CIDP)
  
• Primary humoral immunodeficiency
  
• Idiopathic thrombocytopenic purpura
  
• B-cell chronic lymphocytic leukemia
  
• Kawasaki syndrome
  
• Kidney transplant with a high antibody recipient or with an ABO incompatible donor
  
• Human immunodeficiency virus infection (pediatric only)
  
• Guillain Barre syndrome (GBS)
  
• Myasthenia gravis (MG)
  
• Multifocal motor neuropathy (MMN)
  
• Inflammatory myopathies: dermatomyositis (DM) and polymyositis (PM)
  
• Stiff-person syndrome
  
• Adrenoleukodystrophy
  
• Paraneoplastic syndromes
  
• Paraproteinemic immunoglobulin M demyelinating polyneuropathy
  
• Intractable childhood epilepsy including West syndrome, Lennox-Gastaut and Rasmussen syndrome
  
• Acute demyelinating encephalomyelitis (ADEM)
  
• Optic neuritis and multiple sclerosis (MS)
  
• Central pontine myelinolysis
  
• Diabetic amyotrophy
  
• Peripheral polyneuropathy
  
• Myelopathy associated with human T-cell lymphotropic virus-1 infection (HTLV-1)
  
• Wegener’s granulomatosis
  
• Churg-Strauss syndrome
  
• Amyotrophic lateral sclerosis (ALS)
  
• Alzheimer dementia

• IGIV preparations are derived from a pool of at least 1000 donors. They contain anti-idiotypic antibodies that bind to and neutralize pathogenic autoantibodies. The infused IG may downregulate production of endogenous IG. The IG from IGIV may block Fc receptors on immune cells
  
• IGIV contains high-affinity neutralizing antibodies against interleukin (IL)-1a, IL-6 and TNFα, which may downregulate synthesis of cytokines by T cells
  
• It forms complexes with products of complement activation, preventing the formation and deposition of attack complexes on target cells
  
• IGIV also causes transient lymphopenia and reduces the number of natural killer cells

• Days. Usually there is some effect within a week

• CIDP and GBS: Improves strength and sensory symptoms. In GBS improves prognosis and prevents residual disability
  
• MG: May allow improvement in symptoms and prevent acute deterioration. Often initiated at the time of a clinical flare, before starting long-term disease-modifying therapy
  
• MMN: Symptoms and conduction block improve, but GM1 antibody titers may remain elevated

• CIDP and GBS: Consider corticosteroids for CIDP. In GBS, plasma exchange is an alternative
  
• MG: Start or change disease-modifying therapies (usually at the same time as initiating IGIV)
  
• MMN: If no effect, reconsider the diagnosis, as treatment options for MMN are limited. If the effect of IGIV wears off, consider a short course of plasma exchange before repeating dose

• CIDP and GBS: Most patients require corticosteroids for long-term treatment of CIDP. There are small studies that suggest a combination with 500 mg methylprednisolone improves outcomes in GBS
  
• MG: Usually used with symptomatic treatment such as pyridostigmine. Often combined with other disease-modifying agents such as prednisone, azathioprine, cyclophosphamide, mycophenolate mofetil, or cyclosporine
  
• MMN: Cyclophosphamide may be useful in refractory cases

• Check renal function, CBC before starting treatment and monitor renal function periodically during therapy