Anti-IgA antibodies in Common Variable Immunodeficiency (CVID): Diagnostic workup and therapeutic strategy

Horn, Julia; Thon, Vojtěch; Bartoňková, Dana; Salzer, Ulrich; Warnatz, Klaus; Schlesier, Michael; Peter, Hans‐Hartmut; Grimbacher, Bodo

doi:10.1016/j.clim.2006.10.002

Cited by 62 publications

(45 citation statements)

References 20 publications

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“…These products usually contain trace amounts of IgA that may precipitate anaphylaxis in severely affected patients. In fact, IgA-depleted intravenous IgG (IVIgG) preparations, which contain only trace amounts of IgA, have been repeatedly described to cause anaphylaxis [9,10,11]. This phenomenon is supported by the newly affected patients in the present study.…”

Section: Introductionsupporting

confidence: 75%

Long-Term Treatment and Transfusion of Normal Blood Components Following Tolerance Induction in Patients with Anti-IgA Anaphylactic Reactions

Salama

Kardashi

Arbach

2014

Transfus Med Hemother

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Background: In general, patients with significant anti-Ig-A do not tolerate intravenous (i.v.) administration of normal blood products. Here, we present our experiences in the induction of immune tolerance (IIT) and long-term treatment in a series of such patients affected in such a way. The question whether blood components from IgA-deficient donors are required will be discussed. Methods: Ten adult patients (4 females and 6 males; age ranging from 36 to 75 years) with anti-IgA were included in this study. All patients required long-term treatment with blood components. One patient had IgA deficiency and paroxysmal nocturnal hemoglobinuria (PNH), and all other patients had common variable immunodeficiency (CVID). The particle gel immunoassay was used for the detection of anti-IgA. Immune tolerance to IgA was induced by controlled subcutaneous (s.c.) and/or i.v. infusions of IgG preparations. Results: Prior to IIT, anti-IgA was detectable in plasma samples of all patients and significantly diminished or abolished by controlled s.c. and/or i.v. infusions of IgG. Multiple transfusions with normal blood components could be repeatedly performed with the patient suffering from PNH without any complications. As long as i.v. IgG (IVIgG) infusions were consequently administered as individually required (intervals 2-8 weeks), none of the patients developed reactions during observation (up to 10 years). However, interruption of treatment and re-exposure to IVIgG resulted in adverse reactions. Conclusion: Patients with significant anti-IgA can be safely desensitized and tolerate long-term IgG substitutions independent of the IgA concentration of the used blood component.

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Section: Introductionsupporting

confidence: 75%

Long-Term Treatment and Transfusion of Normal Blood Components Following Tolerance Induction in Patients with Anti-IgA Anaphylactic Reactions

Salama

Kardashi

Arbach

2014

Transfus Med Hemother

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“…Complete absence of IgA has been recently shown to be a significant risk factor for the development of anaphylactic reactions [94]. The IgA content of the different IVIG preparations varies and also plays a significant role in the development of severe anaphylactic reactions in the high risk patients [95].…”

Section: Intravenous Immunoglobulins (Ivig)mentioning

confidence: 99%

Treatment of Juvenile Dermatomyositis: An Update

Papadopoulou

Wedderburn

2017

Pediatr Drugs

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Abstract:The Idiopathic Inflammatory Myopathies of childhood consist a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response. The advent of biological drugs has revolutionised the management of various paediatric rheumatologic diseases, including inflammatory myopathies. There are few data from randomised controlled trials to guide management decisions, thus several algorithms for the treatment of juvenile myositis have been developed using international expert opinion. The general treatment goals now include elimination of active disease and normalization of physical function, so as to preserve normal growth and development, and to prevent long-term damage and deformities. This review summarizes the newer and possible future therapies of juvenile inflammatory myopathies, including evidence supporting their efficacy and safety.

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“…It has been demonstrated that patients with IgA antibodies can be treated more safely with SCIg [94,95]. SCIg might induce tolerance by gradual exposure to IgA owing to the slow resorption of the subcutaneous deposit of Ig [96].…”

Section: Choice Of Product and Administration Routementioning

confidence: 99%

Immunoglobulin treatment in primary antibody deficiency

Maarschalk-Ellerbroek

Hoepelman

Ellerbroek

2011

International Journal of Antimicrobial Agents

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The primary antibody deficiency syndromes are characterised by recurrent respiratory tract infections and the inability to produce effective immunoglobulin (Ig) responses. The best known primary antibody deficiencies are common variable immunodeficiency (CVID), X-linked agammaglobulinaemia (XLA), immunoglobulin G (IgG) subclass deficiency, and selective antibody deficiency with normal immunoglobulins (SADNI).Therapy in these patients consists of prophylactic antibiotics and/or Ig replacement therapy. Diagnostic delay remains common owing to limited awareness of the presenting features and may result in increased morbidity and mortality. Replacement therapy with immunoglobulins increases life expectancy and reduces the frequency and severity of infections, but the effect on end-organ damage is still unknown. Both intravenous immunoglobulin (IVIg) and subcutaneous immunoglobulin (SCIg) treatment appear to be safe, with comparable efficacy. A starting dose of 300-400 mg/kg/month in IVIg and 100 mg/week for SCIg is recommended. IgG trough levels should be >5 g/L for patients with agammaglobulinaemia and 3 g/L greater than the initial IgG level for patients with CVID; however, the clinical response should be foremost in choosing the dose and trough level. Infusion-related adverse reactions are generally mild owing to improved manufacturing processes. In this paper, aspects of Ig replacement therapy in primary antibody-deficient patients will be addressed.

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Anti-IgA antibodies in Common Variable Immunodeficiency (CVID): Diagnostic workup and therapeutic strategy

Cited by 62 publications

References 20 publications

Long-Term Treatment and Transfusion of Normal Blood Components Following Tolerance Induction in Patients with Anti-IgA Anaphylactic Reactions

Long-Term Treatment and Transfusion of Normal Blood Components Following Tolerance Induction in Patients with Anti-IgA Anaphylactic Reactions

Treatment of Juvenile Dermatomyositis: An Update

Immunoglobulin treatment in primary antibody deficiency

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