Selective IgA Deficiency

Classify SIgAD when serum IgA is <7 mg/dL in a patient older than 4 years, with normal serum IgG and IgM, normal vaccine responses, and exclusion of secondary hypogammaglobulinemia and T-cell defects. Do not apply this definitive label to younger children, in whom low IgA can be developmentally transient. Wiley+2WileySelective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management - Yazdani - 2017 - Scandinavian Journal of Immunology - Wiley Online LibraryPubMedSelective IgA Deficiency and COVID-19 Outcomes: A Nationwide Retrospective Cohort Study - PMCPubMedThe clinical implications of selective IgA deficiency - PMC

Repeat quantitative serum immunoglobulins when the initial result is unexpected or obtained during an acute clinical evaluation, and interpret pediatric concentrations against age-specific laboratory reference ranges. Quantify IgG and IgM concurrently: low IgG or low IgM shifts the working diagnosis away from isolated SIgAD and toward a broader humoral immunodeficiency. PubMed+1PubMedThe clinical implications of selective IgA deficiency - PMCScienceDirectImmunoglobulin A Deficiency - an overview | ScienceDirect Topics

Review medications and acquired conditions before diagnosing a primary disorder. Antiepileptic medications, microbiome disruption associated with antibiotics, infections, and malignancy have been associated with secondary IgA deficiency. ScienceDirect+1ScienceDirectThe clinical implications of selective IgA deficiency - ScienceDirectPubMedSelective IgA Deficiency - PMC

Incidentally identified, asymptomatic patients usually need confirmation of the immunoglobulin pattern and documentation rather than treatment. Approximately 85% to 90% of IgA-deficient individuals are asymptomatic, whereas symptomatic patients most often have recurrent respiratory or gastrointestinal infections, allergic disease, or autoimmunity. PubMed+1PubMedSelective IgA Deficiency - PMCPubMedSelective IgA Deficiency and COVID-19 Outcomes: A Nationwide Retrospective Cohort Study - PMC

For recurrent otitis, sinusitis, bronchitis, or pneumonia, obtain microbiologic data during clinically significant episodes when feasible and assess functional antibody responses to protein and polysaccharide antigens. Recurrent pulmonary infections are commonly associated with encapsulated bacteria, particularly Streptococcus pneumoniae and Haemophilus influenzae; risk is greater when IgG2 or IgG3 subclass deficiency coexists. PubMed+1PubMedSelective IgA Deficiency - PMCPubMedSelective IgA Deficiency - StatPearls - NCBI Bookshelf

For chronic or recurrent diarrhea, malabsorption, or unexplained weight loss, test for Giardia lamblia and evaluate for celiac disease. SIgAD has a predilection for Giardia infection, and total IgA deficiency makes IgA-based celiac testing unreliable. ScienceDirect+2ScienceDirectThe clinical implications of selective IgA deficiency - ScienceDirectnice org uk[PDF] Coeliac disease: recognition, assessment and management | NICEPubMedSelective IgA Deficiency - StatPearls - NCBI Bookshelf

For severe allergic rhinitis, asthma, eczema, urticaria, conjunctivitis, food allergy, or recurrent allergic symptoms, evaluate and treat the allergic disorder on its own merits while recognizing the association with SIgAD. For autoimmune features, use organ-directed testing based on presentation because celiac disease, thyroid disease, type 1 diabetes, systemic lupus erythematosus, autoimmune cytopenias, and inflammatory arthritis are associated conditions. PubMed+1PubMedThe clinical implications of selective IgA deficiency - PMCPubMedSelective IgA Deficiency - StatPearls - NCBI Bookshelf

For isolated SIgAD without clinically consequential infections or associated disease, do not institute immunoglobulin replacement solely because the IgA concentration is low. IVIG is not indicated when selective IgA deficiency is the only abnormality of concern. ema europa euema europa eu[PDF] Guideline on core SmPC for human normal immunoglobulin for ...

For recurrent bacterial respiratory infections, treat documented infections with pathogen-directed antimicrobial therapy and determine whether impaired specific antibody responses, an IgG subclass deficiency, or evolving CVID is contributing. Prophylactic antibiotics may be appropriate in selected symptomatic patients, particularly those with a severe infectious phenotype; agent choice and regimen should be individualized to infection site, cultures, allergy history, and local resistance patterns. ScienceDirect+1ScienceDirectThe clinical implications of selective IgA deficiency - ScienceDirectWileySelective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management - Yazdani - 2017 - Scandinavian Journal of Immunology - Wiley Online Library

Consider immunoglobulin replacement only rarely and only when there is a justified severe phenotype with an additional clinically meaningful antibody defect. If replacement is used, select an immunoglobulin product with minimal IgA and initiate and monitor treatment under an immunodeficiency-experienced physician because hypersensitivity reactions can occur in patients with anti-IgA antibodies. Wiley+1WileySelective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management - Yazdani - 2017 - Scandinavian Journal of Immunology - Wiley Online Libraryema europa eu[PDF] Guideline on core SmPC for human normal immunoglobulin for ...

Treat associated Giardia, celiac disease, allergic disease, and autoimmunity according to the confirmed disorder rather than attributing persistent symptoms to SIgAD alone. In celiac disease, use IgG-based serology for the diagnostic pathway when IgA deficient. ScienceDirect+2ScienceDirectThe clinical implications of selective IgA deficiency - ScienceDirectnice org uk[PDF] Coeliac disease: recognition, assessment and management | NICEPubMedThe clinical implications of selective IgA deficiency - PMC

A rare subset of patients with complete IgA deficiency develops anti-IgA antibodies, including IgE anti-IgA antibodies, that can cause severe hypersensitivity or anaphylaxis after IgA-containing blood products or immunoglobulin. A prior anaphylactic transfusion reaction should prompt formal evaluation and transfusion-medicine planning before future transfusion. ScienceDirect+2ScienceDirectThe clinical implications of selective IgA deficiency - ScienceDirectCDC[PDF] Guidelines for Preventing Opportunistic Infections Among ... - CDCPubMedSelective IgA Deficiency - PMC

Standard immunoglobulin preparations should not be used casually in SIgAD. IVIG labeling guidance states that true hypersensitivity reactions are rare but may occur in patients with anti-IgA antibodies, and IVIG is not indicated for isolated SIgAD. When an independent replacement indication is established, use a low-IgA product and supervise initiation and monitoring through an experienced immunodeficiency clinician. Wiley+1WileySelective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management - Yazdani - 2017 - Scandinavian Journal of Immunology - Wiley Online Libraryema europa eu[PDF] Guideline on core SmPC for human normal immunoglobulin for ...

Place IgA deficiency and any prior transfusion or immunoglobulin reaction prominently in the medical record. For patients with a concerning reaction history, coordinate product selection prospectively with transfusion medicine rather than waiting until urgent transfusion is required. CDC+1CDC[PDF] Guidelines for Preventing Opportunistic Infections Among ... - CDCPubMedSelective IgA Deficiency - PMC

Monitor symptomatic patients at regular clinical intervals for recurrent sinopulmonary infection, gastrointestinal infection, asthma or allergic rhinitis, and autoimmune disease. Infection is often the earliest manifestation in symptomatic cohorts, with allergic and autoimmune manifestations emerging later; new disease in one domain should prompt reassessment of the others. ScienceDirect+1ScienceDirectThe clinical implications of selective IgA deficiency - ScienceDirectPubMedUnderstanding the natural history of selective IgA deficiency - PMC

Repeat quantitative immunoglobulins and functional antibody assessment when infections become more frequent or severe, when new autoimmune disease develops, or when a prior isolated pattern changes. SIgAD can be an early manifestation before subsequent CVID diagnosis; falling IgG, impaired vaccine responses, or reduced memory B cells warrants reclassification rather than continued labeling as uncomplicated SIgAD. ScienceDirect+1ScienceDirectThe clinical implications of selective IgA deficiency - ScienceDirectPubMedThe clinical implications of selective IgA deficiency - PMC

Patients with recurrent lower respiratory infections require particular attention because coexisting IgG subclass deficiency and defective antibody responses can identify a higher-risk phenotype. Persisting lower respiratory disease despite standard management should prompt immunology review rather than empiric indefinite treatment as uncomplicated SIgAD. BMJ+2BMJBTS Guideline - ThoraxPubMedSelective IgA Deficiency - PMCPubMedSelective IgA Deficiency - StatPearls - NCBI Bookshelf