JPAC Joint United Kingdom (UK) Blood Transfusion and Tissue Transplantation Services Professional Advisory Committee

8.8: Indications for intravenous immunoglobulin (IVIg)

Human normal immunoglobulin products are manufactured from large pools of donor plasma. All the products used in the UK are imported as a variant Creutzfeldt–Jakob disease (vCJD) risk-reduction measure. The two main uses for IVIg are as replacement therapy in primary or acquired antibody deficiency disorders and as immunomodulatory agents in patients with autoimmune or inflammatory conditions. Because of limitations on supply of IVIg, the UK Departments of Health have commissioned evidence-based clinical guidelines to prioritise its use. Tables 8.8 and 8.9 are based on the 2011 update of these guidelines.

Table 8.8 High-priority (‘red’) indications for intravenous immunoglobulin – an adequate evidence base and potentially life-saving

Primary and secondary antibody deficiency states

Primary immunodeficiencies

Thymoma with immunodeficiency

HSC transplant in primary immunodeficiencies

Specific antibody deficiency

Haematology

Alloimmune thrombocytopenia
(feto-maternal/neonatal)

Haemolytic disease of the newborn

Idiopathic thrombocytopenic purpura (ITP) – acute and persistent

Neurology

Chronic inflammatory demyelinating polyradiculoneuropathy (acute)

Guillain–Barré syndrome

Paraprotein-associated demyelinating neuropathy

Others

Kawasaki disease

Toxic epidermal necrolysis

 

Table 8.9 ‘Blue’ indications for intravenous immunoglobulin – a reasonable evidence base but other treatment options are available

Primary and secondary antibody deficiency states

Secondary antibody deficiency (any cause)

Haematology

Acquired red cell aplasia

Autoimmune haemolytic anaemia

Clotting factor inhibitors

Haemophagocytic syndrome

Post-transfusion purpura

Neurology

Chronic inflammatory demyelinating polyradiculoneuropathy (chronic)

Inflammatory myelopathies

Myasthenia gravis

Multifocal motor neuropathy

Rasmussen syndrome

Stiff person syndrome

Others

Autoimmune congenital heart block

Autoimmune uveitis

Immunobullous diseases

Necrotising staphylococcal sepsis

Severe or recurrent Clostridium difficile colitis

Staphylococcal or streptococcal toxic shock syndrome

Antibody-mediated rejection after solid organ transplantation