Conditions for which IVIg use is in exceptional circumstances only

Acute leukaemia in children

[Includes acute lymphoblastic or lymphoid leukaemia (ALL) and acute myeloblastic leukaemia (AML)].

IVIg may be considered in cases of ALL or AML with neutropenic sepsis in patients aged ≤15 years in whom conventional antimicrobial therapy has been ineffective and who have life-threatening infection.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

HIV in children

The need for IVIg in paediatric HIV has been substantially reduced with the advent of highly active antiretroviral therapy (HAART). A trial of therapy may however be considered in children with significant recurrent bacterial infections despite HAART.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Orange, JS, Hossny, EM, Weiler, CR, et al 2006, ‘Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology’, Journal of Allergy and Clinical Immunology, vol. 117, no. 4, pp. S525–53.

Autoimmune congenital heart block (neonatal lupus)

IVIg therapy may be indicated during pregnancy when there is a history of autoimmune congenital heart block in at least one previous pregnancy and maternal SS-A and/or SS-B antibodies are present.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Buyon, JP, Kim, MY, Copel, JA, et al 2001, ‘Anti-Ro/SSA antibodies and congenital heart block: necessary but not sufficient’, Arthritis & Rheumatism, vol. 44, no. 8, pp. 1723–7.

Kaaja, R & Julkunen, H 2003, ‘Prevention of recurrence of congenital heart block with intravenous immunoglobulin and corticosteroid therapy: comment on the editorial by Buyon et al’, Arthritis & Rheumatism, vol. 48, no. 1, pp. 280–1.

Tran, HB, Cavill, D, Buyon, JP, et al 2004, ‘Intravenous immunoglobulin and placental transport of anti-Ro/La antibodies: comment on the letter by Kaaja and Julkunen’, Arthritis & Rheumatism, vol. 50, no. 1, pp. 337–8.

Villain, E, Coastedoat-Chalumeau, N, Marijon, E, et al 2006, ‘Presentation and prognosis of complete atrioventricular block in childhood, according to maternal antibody status’, Journal of the American College of Cardiology, vol. 48, no. 8, pp. 1682–7.

Wong, JP, Kwek, KY, Tan, JY, et al 2001, ‘Fetal congenital complete heart block: prophylaxis with intravenous gammaglobulin and treatment with dexamethasone’, Australia New Zealand Journal of Obstetrics and Gynaecology, vol. 41, no. 3, pp. 339–41.

Autoimmune neutropenia

Autoimmune neutropenia is a rare disorder caused by peripheral destruction of antibody-sensitised neutrophils by cells of the reticuloendothelial system. IVIg may be considered among treatment options in rare circumstances when the standard treatment of G-CSF fails.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Anderson, D, Ali, K, Blanchette, V, et al 2007, ‘Guidelines on the use of intravenous immune globulin for hematologic conditions’, Transfusion Medicine Reviews, vol. 21, no. 2, suppl. 1, pp. S9–56.

Autoimmune uveitis

Uveitis refers to inflammation of the uvea of the eye and can be caused by infection, exposure to toxins or autoimmune disorders. Symptoms may include redness of the eye, blurred vision, unusual sensitivity to light, dark floating spots in the vision and eye pain. Ocular inflammation of this kind may threaten sight and be resistant to standard immunosuppression.

IVIg therapy may be considered for immune-mediated, sight- threatening uveitis with persistent activity despite both oral corticosteroid and systemic immunosuppressive therapy. Uveitis of non-immune origin is not indicated.

Recommended dose is 1.5 g/kg/month for three months, with further maintenance dependent upon evidence of significant improvement in visual acuity and ocular inflammation.

Dosing above 1 g/kg per day is contraindicated for some IVIg products.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Lim, LL, Suhler, EB & Smith, JR 2006, ‘Biologic therapies for inflammatory eye disease’, Clinical and Experimental Ophthalmology, vol. 34, pp. 365–74.

Orange, JS, Hossny, EM, Weiler, CR, et al 2006, ‘Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology’, Journal of Allergy and Clinical Immunology, vol. 117, no. 4, pp. S525–53.

Rosenbaum, JT, George, RK & Gordon, C 1999, ‘The treatment of refractory uveitis with intravenous immunoglobulin’, American Journal of Ophthalmology, vol. 127, no. 5, pp. 545–9.

Catastrophic antiphospholipid syndrome

IVIg may be appropriate therapy for catastrophic antiphospholipid syndrome, a term that describes the accelerated form of antiphospholipid syndrome characterised by widespread small vessel thrombosis leading to multiorgan failure. It is not indicated for the treatment of antiphospholipid syndrome in other cases. Please see Antiphospholipid syndrome (non-obstetric) in Chapter 8 (page 214) and Recurrent foetal loss (with or without antiphospholipid syndrome) in Chapter 8 (page 216).

Qualifying criteria for IVIg therapy

A patient will qualify for IVIg when all the following criteria are met:

1. Evidence of rapidly evolving thrombosis involving two or more organs;
2. Unequivocal laboratory evidence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies and/or beta 2 glycoprotein I antibodies); and
3. Other causes of thrombotic microangiopathy are considered less likely.

Confirmation by histopathology of thrombotic small vessel occlusion in at least one organ or tissue is desirable but should not delay IVIg therapy if indicated.

A single treatment is usually sufficient, based on a dose of 2 g/ kg divided over 2–5 days. The potential pro-thrombotic effect of IVIg should be considered in this indication.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Asherson, RA, Cervera, R, de Groot, PG, Erkan, D, Boffa, M-C, Piette, J-C, et al 2003, ‘Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines’, Lupus, vol. 12, pp. 530–34.

Asherson RA, et al 2002, ‘Catastrophic antiphospholipid syndrome: proposed guidelines for diagnosis and treatment’, Journal of Clinical Rheumatology, vol. 8, no. 3, pp. 157–65.

Cervera, R, Asherson, RA & Font, J 2006, ‘Catastrophic antiphospholipid syndrome’, Rheumatic Disease Clinics of North America, vol. 32, no. 3, pp. 575–90.

Erkan, D 2006, ‘Therapeutic and prognostic considerations in catastrophic antiphospholipid syndrome’, Autoimmunity Reviews, vol. 6, no. 2, pp. 98–103.

Coagulation factor inhibitors (alloantibodies and autoantibodies), including acquired haemophilia, acquired von Willebrand syndrome, inhibitors to factor VIII in haemophilia A, and inhibitors to factor IX in haemophilia B

Management of these rare and severe bleeding disorders should be undertaken only by or in consultation with haemophilia treatment centres. When indicated, IVIg only forms part of the management of these complex patients, with additional haemostatic support required.

IVIg may be considered in the following circumstances:

1. Inhibitors to factor VIII (FVIII) in haemophilia A and inhibitors to factor IX (FIX) in haemophilia B, especially in cases where there has been failure of immune tolerisation and poor response to recombinant factor VIIa or factor eight inhibitor bypassing activity (FEIBA) — only as part of the Bonn–Malmö protocol for immune tolerance induction.
2. Autoimmune acquired von Willebrand syndrome — correction of FVIII and von Willebrand factor levels for the management of bleeding and before invasive procedures, except cases associated with IgM paraprotein where response is unlikely. Use is indicated in failure to respond to chemotherapy/immunosuppressants or where there is insufficient time for chemotherapy/immunosuppressants to be given. Initial therapy either 0.4 g/kg for 5 days or 1 g/kg for 2 days. Continued therapy 1 g/kg once every 3–4 weeks.
3. Acquired haemophilia A for:

1. Support of correction of FVIII level for the management of bleeding, and before invasive procedures in individuals in whom steroid or immunosuppressive therapy is contraindicated or has failed to eradicate the inhibitor (2 g/kg over 2–5 days); or
2. Support of correction of FVIII level following failure of first line therapies (steroids and immunosuppressants) and poor response to recombinant factor VIIa or FEIBA when used as part of the Bonn–Malmö protocol.

4. Other acquired (autoimmune) coagulation inhibitors (e.g. acquired Factor V inhibitors) to correct factor level for the management of bleeding and before invasive procedures in cases where other therapeutic approaches have failed or are contraindicated (2 g/kg over 2 to 5 days).

Dosing above 1 g/kg per day is contraindicated for some IVIg products.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Hay, CR, Brown, S, Collins, PW, et al 2006, ‘The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation’, British Journal of Haematology, vol. 133, no. 6, pp. 591–605.

Devic disease (neuromyelitis optica)

Devic disease is an idiopathic inflammatory demyelinating disorder of the central nervous system characterised by recurrent bouts of optic neuritis and myelitis. It is distinct from multiple sclerosis and evidence of B-cell autoimmunity has been found. A circulatory antibody to aquaporin-4 is found in many patients providing further evidence of B-cell autoimmunity in its pathogenesis and suggestive of a role for IVIg therapy. Single case reports of various therapies, including IVIg, have shown variable benefit in this otherwise devastating disorder.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Lennon, VA, Wingerchuk, DM, Kryzer, TJ, et al 2004, ‘A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis’, Lancet, vol. 364, no. 9451, pp. 2106–12.

Lucchinetti, CF, Mandler, RN, McGavern, D, et al 2002, ‘A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica’, Brain, vol. 125, pp. 1450–61.

Minagar, A, Alexander, JS, Fowler, MR, et al 2002, ‘Devic disease: clinical course, pathophysiology, and management’, Pathophysiology, vol. 9, no. 1, p. 33.

Diabetic amyotrophy (diabetic proximal neuropathy or diabetic lumbosacral radiculoplexus neuropathy)

IVIg may be considered in exceptional circumstances for intractable pain or progressive muscle weakness in patients in whom steroids are ineffective or cannot be tolerated. This condition is monophasic and usually self-limiting. A single treatment may be sufficient, although monthly infusions for up to six months may be required for recurrent pain.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Epidermolysis bullosa acquisita

IVIg should be considered for severe cases refractory to conventional immunosuppressive therapy.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Epilepsy

• Landau–Kleffner syndrome
• Lennox–Gastaut syndrome

IVIg should be considered in childhood cases only after failure of all conventional therapies and full assessment by a paediatric neurologist.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Graves ophthalmopathy

IVIg may be indicated in select cases. Tagami et al (1996) have shown that IVIg is effective in this condition. Other studies have shown IVIg to be as effective as corticosteroids with fewer side effects. May be indicated where steroids have failed or are contraindicated.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Tagami, T, Tanaka, K, Sugawa, H, et al 1996, ‘High-dose intravenous steroid pulse therapy in thyroid associated ophthalmopathy’, Endocrinology Journal, vol. 43, no. 6, pp. 689–99.

Haemolytic disease of the newborn (HDN)

HDN arises from foetomaternal antigen incompatibility and can result in clinically significant foetal/neonatal haemolysis, severe anaemia and hyperbilirubinaemia.

Although prophylaxis programs have reduced the frequency of Rhesus (Rh) D HDN, antibodies to RhD remain the most common cause in Australia. Antibodies to other antigens in the Rh system (e.g. Rhc, E), ABO and other antigens (e.g. K) may also cause disease ranging from mild to life-threatening.

IVIg may be used in selected cases in consultation with experts in foetomaternal medicine and transfusion medicine.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Gottstein, R & Cooke, RW 2003, ‘Systematic review of intravenous immunoglobulin in haemolytic disease of the newborn’, Archives of Disease in Childhood – Fetal Neonatal Edition, vol. 88, no. 1, pp. F6–10.

Kaplan, M, Vreman, HJ, Hammerman, C, et al 1996, ‘Intravenous immune globulin in neonatal ABO isoimmunisation: factors associated with clinical efficacy’, Biology of the Neonate, vol. 70, pp. 69–72.

Miqdad, AM, Abdelbasit, OB, Shaheed, MM, et al 2004, ‘Intravenous immunoglobulin G therapy for significant hyperbilirubinaemia in ABO haemolytic disease of the newborn’, Journal of Maternal-Fetal and Neonatal Medicine, vol. 16, no. 3, pp. 163–6.

Haemolytic transfusion reaction

IVIg may be considered in the management or prevention of severe haemolytic transfusion reaction not responding to other interventions (e.g. corticosteroids).

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Win, N, Madan, B, Gale, R, et al 2005, ‘Intravenous immunoglobulin given to lymphoma patients with recurrent haemolytic transfusion reactions after transfusion of compatible blood’, Hematology, vol. 10, no. 5, pp. 375–8.

Hashimoto encephalopathy (steroid-responsive encephalopathy associated with autoimmune thyroiditis)

IVIg is not supported as first-line treatment, because preferable alternative treatments are available.

IVIg may be considered in exceptional circumstances where there is progressive neurologic decline despite appropriate steroid therapy.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Jacob, S & Rajabally, YA 2005, ‘Hashimoto’s encephalopathy: steroid resistance and response to intravenous immunoglobulins’, Journal of Neurology, Neurosurgery and Psychiatry, vol. 76, no. 3, pp. 455–6.

Limbic encephalitis — nonparaneoplastic

There appears to be a role for IVIg in nonparaneoplastic limbic encephalitis associated with neuronal antibodies to cell surface antigens. This includes VGKC antibodies, as well as NMDA receptor antibodies and AMPA receptor antibodies.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Myocarditis in children

There is some evidence that IVIg improves cardiac function in children with proven or likely viral myocarditis.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Drucker, NA, Colan, SD, Lewis, AB, et al 1994, ‘Gamma- globulin treatment of acute myocarditis in the pediatric population’, Circulation, vol. 89, pp. 252–7.

Robinson, J, Hartling, L, Vandermeer, B, et al 2005, ‘Intravenous immunoglobulin for presumed viral myocarditis in children and adults (Cochrane Review)’, in The Cochrane Library, Issue 1, John Wiley & Sons, Ltd, Chichester, UK.

Paediatric autoimmune neuropsychiatric disorder associated with streptococcal infection (PANDAS)

PANDAS was first described in the early 1990s. PANDAS is characterised by rapid-onset tics associated with obsessive- compulsive disorder (OCD) in the context of recovery from streptococcal infection. Molecular mimicry between streptococcal antigens and the central nervous system is thought to underlie the cause. Symptomatic therapy is used with variable response.

A single randomised placebo-controlled trial using IVIg for PANDAS showed very prolonged and significant improvement in obsessive-compulsive symptoms, anxiety, depression, emotional lability and overall function compared with placebo. Improvements in symptoms were still evident at one-year follow-up.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Singer, HS 1999, ‘PANDAS and immunomodulatory therapy’, Lancet, vol. 354, no. 9185, pp. 1137–8.

Perlmutter, SJ, Leitman, SF, Garvey, MA, et al 1999, ‘Therapeutic plasma exchange and intravenous immunoglobulin for obsessive-compulsive disorder and tic disorders in childhood’, Lancet, vol. 354, no. 9185, pp. 1153–8.

Paraneoplastic neurological syndromes

   Paraneoplastic subacute sensory neuropathy

IVIg may be indicated in select cases, in combination with tumour therapy (tumour resection and/or oncological treatment) where the latter has not led to an improvement in the neurologic syndrome; where other immunomodulatory therapies are contraindicated or have failed; or if the neurologic features warrant urgent intervention.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

   Paraneoplastic cerebellar degeneration

IVIg may be indicated in select cases, in combination with tumour therapy (tumour resection and/or oncological treatment) where the latter has not led to an improvement in the neurologic syndrome; where other immunomodulatory therapies are contraindicated or have failed; or if the neurologic features warrant urgent intervention.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

   Limbic encephalitis — paraneoplastic

IVIg may be indicated in select cases, in combination with tumour therapy (tumour resection and/or oncological treatment) where the latter has not led to an improvement in the neurologic syndrome; where other immunomodulatory therapies are contraindicated or have failed; or if the neurologic features warrant urgent intervention.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Bataller, L, Galiano, R, Garcia-Escrig, M, Martinez, B, Sevilla, T, Blasco, R, et al 2010, ‘Reversible paraneoplastic limbic encephalitis associated with antibodies to the AMPA receptor’, Neurology, vol. 74, no. 3, pp. 265–7.

Henry, C, Husson, H, de Broucker, T 2009, ‘Autoimmune limbic encephalitis with anti-NMDA receptor antibodies and ovarian teratoma: a treatable form of paraneoplastic limbic encephalitis’ (in French), Revue neurologique (Société de neurologie de Paris), vol. 165, no. 1, pp. 70–5.

Potassium channel antibody-associated encephalopathy

Potassium channel antibody-associated neurologic syndromes include limbic encephalitis/subacute amnesic encephalopathy, Morvan syndrome, peripheral nerve hyperexcitability and autonomic ganglionopathy.

Potassium channel antibody-associated encephalopathy is considered to be an autoimmune, nonparaneoplastic, potentially treatable syndrome, but may respond to a variety of immunomodulatory agents, including IVIg.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Vincent, A, Buckley, C, Schott, JM, et al 2004, ‘Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis’, Brain, vol. 127, pt 3, pp. 701–12.

Hudson, LA, et al 2008, ‘Reduplicative paramnesia in Morvan’s syndrome’, Journal of the Neurological Sciences, vol. 267, no. 1–2, pp. 154–7.

Pure red cell aplasia (PRCA)

PRCA is a rare syndrome of severe anaemia, reticulocytopenia and a selective deficiency of erythroid progenitors. IVIg should be considered as first-line therapy for viral PRCA associated with parvovirus B19 in immunocompromised patients. IVIg is a reasonable option for patients with immunological PRCA who have failed other therapies (e.g. prednisone or cyclosporine).

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Anderson, D, Ali, K, Blanchette, V, et al 2007, ‘Guidelines on the use of intravenous immune globulin for hematologic conditions’, Transfusion Medicine Reviews, vol. 21, no. 2, suppl. 1, pp. S9–56.

Pure white cell aplasia (PWCA)

PWCA is a rare syndrome of severe neutropenia and a selective deficiency of granulocyte progenitors. IVIg is a reasonable option for patients with immunological PWCA who have failed other therapies (e.g. prednisone or cyclosporine).

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Anderson, D, Ali, K, Blanchette, V, et al 2007, ‘Guidelines on the use of intravenous immune globulin for hematologic conditions’, Transfusion Medicine Reviews, vol. 21, no. 2, suppl. 1, pp. S9–56.

Pyoderma gangrenosum

Use of IVIg is limited to patients with significant pyoderma gangrenosum, diagnosed by a dermatologist, unresponsive to corticosteroids and other immunosuppressive agents.

IVIg should be ceased in patients who fail to respond after three cycles.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Cummins, DL, Anhalt, GJ, Monahan, T & Meyerle, JH 2007, ‘Treatment of pyoderma gangrenosum with intravenous immunoglobulin’, British Journal of Dermatology, vol. 157, no. 6, pp. 1235–39.

Kreuter, A, Reich-Schupke, S, Stucker, M, Altmeyer, P & Gambichler T 2008, ‘Intravenous immunoglobulin for pyoderma gangrenosum‘, British Journal of Dermatology, vol. 158, no. 4, pp. 856–7.

Rasmussen syndrome

Rasmussen syndrome is a chronic, progressive, focal encephalitis that is commonly accompanied by focal seizures, hemiparesis and cognitive decline. It is generally considered to be a disease of childhood, with most cases occurring in children younger than 10 years, although adult onset cases do occur. Conventional anticonvulsant therapy is usually ineffective and hemispherectomy may be helpful in the correct setting.

Immunomodulatory therapy may be useful and, of the different therapies, IVIg may be most useful. Other therapies to consider include methylprednisolone and rituximab.

Ongoing supply of IVIg would be based on evidence of stabilisation of either seizure frequency or cognitive decline.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Scleromyxedema

IVIg may be indicated in select cases not responding to steroids, or when steroids and other alternative treatments (e.g. thalidomide) are contraindicated.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Kulczycki, A, Nelson, M, Eisen, A, et al 2003, ‘Scleromyxedema: treatment of cutaneous and systemic manifestations with high-dose intravenous immunoglobulin’, British Journal of Dermatology, vol. 149, no. 6, pp. 1276–81.

Majeski, C, Taher, M, Grewal, P, et al 2005, ‘Combination oral prednisone and intravenous immunoglobulin in the treatment of scleromyxedema’, Journal of Cutaneous Medicine and Surgery, vol. 9, no. 3, pp. 99–104.

Sjogren’s syndrome

IVIg may be indicated in certain highly selected cases where other treatments have not been effective.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Smith, A, Jackson, M, Wang, F, et al 2005, ‘Neutralisation of muscarinic receptor autoantibodies by intravenous immunoglobulin in Sjogren’s syndrome’, Human Immunology, vol. 66, no. 4, pp. 411–6.

Solid organ transplantation (other than kidney)

IVIg may be indicated in:

• highly sensitised patients awaiting transplantation;
• transplant recipients with acute antibody-mediated rejection with clinical evidence of graft dysfunction; and
• transplant recipients as treatment or prophylaxis for rejection where conventional immunosuppressive therapy is contraindicated; for example, in a patient with life-threatening infection in whom conventional immunosuppression will place the patient at greater risk, or when the transplant is at risk.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Reference

Jordan, SC, Vo, A, Bunnapradist, S, et al 2003, ‘Intravenous immune globulin treatment inhibits cross match positivity and allows for successful transplantation of incompatible organs in living-donor and cadaver recipients’, Transplantation, vol. 76, no. 4, pp. 631–6.

Susac syndrome

Susac syndrome is a rare, microangiopathic disorder characterised by encephalopathy, hearing loss and retinal artery branch occlusions. Case reports show benefit of IVIg therapy in combination with corticosteroids, with or without other immunosuppressive agents.

Dose: 1–2 g/kg/month for one year providing documented clinical improvement.

Dosing above 1 g/kg per day is contraindicated for some IVIg products.

Note: Effectiveness of IVIg therapy may be difficult to determine due to the fluctuating course of disease.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Aubart-Cohen, F, Klein, I, Alexandra, J, et al 2007, ‘Long-term outcome in Susac syndrome’, Medicine (Baltimore), vol. 86, no. 2, pp. 93–102.

Fox, R, Costello, F, Judkins, A, et al 2006, ‘Treatment of Susac syndrome with gamma globulin and corticosteroids’, Journal of the Neurological Sciences, vol. 251, no. 1–2, pp. 17–22.

Systemic capillary leak syndrome (SCLS)

SCLS is an extremely rare condition that is characterised by life-threatening attacks of reversible capillary hyperpermeability accompanied by haemoconcentration and hypoalbuminaemia.

A diagnosis by a consultant physician, emergency specialist or intensive care unit specialist is required.

Other therapies may be appropriate.

Approval will be provided for an initial period of 12 months only.

Clinicians requesting ongoing IVIg therapy after the initial 12 month period are required to confirm in writing that the patient experienced a reduced number of severe episodes requiring hospital admission when treated with IVIg.

Maximum dose of 1–2 g/kg per month.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

References

Abueguen, P, Chennebault, JM & Pichard, E 2010, ‘Immunoglobulins for the treatment of systemic capillary leak syndrome’, Americal Journal of Medicine, vol. 123, pp. e3–4.

Druey, KM & Greipp, PR 2010, ‘Narrative review: the systemic capillary leak syndrome’, Annals of Internal Medcine, vol. 153, pp. 90–8.

Gousseff, M, Arnaud, L, Lambert, M, et al 2011, ‘The systemic capillary leak syndrome: a case series of 28 patients from a European registry’, Annals of Internal Medicine, vol. 154, pp. 464–71.

Govig, BA & Javaheri, S 2010, ‘The systemic capillary leak syndrome (letter)’, Annals of Internal Medicine, vol. 153, p. 764.

Lambert, M, Launay, D, Hachulla, E, et al 2008, ‘High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome’, Critical Care Medicine, vol. 36, pp. 2184–7.

Zipponi, M, Eugster, R & Birrenbach, T 2011, ‘High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome’, BMJ Case Reports, doi:10.1136/bcr.12.2010.3599.