Despite the critical role of IgA in protecting the human organism from various pathogens, the signs and symptoms in patients suffering from selective IgA immunodeficiency are interestingly absent in up to 90% of cases; the reason for the lack of symptoms is not known [1] [2]. Nevertheless, predisposition toward recurrent infections, autoimmune diseases, and allergies may be present [1] [2] [3] [4] [5] [6]. Infections of the respiratory tract and sinuses (mainly caused by bacterial pathogens such as Haemophilus influenza or Streptococcus pneumoniae) are the most common finding in selective IgA immunodeficiency. More severe forms in which bronchiectasis or other complications occur have also been recognized [1]. The gastrointestinal system, because of its abundant mucosal surface that interacts with numerous pathogens and potentially harmful substances, is another important site where various abnormalities are observed [1] [3]. In addition to infections (bacteria, but also protozoa, an example being Giardia lamblia), malabsorption and lactose intolerance are often encountered in symptomatic patients [1]. Furthermore, gastrointestinal conditions that have been strongly linked to selective IgA immunodeficiency are celiac disease and nodular lymphoid hyperplasia[1]. Allergies and autoimmune disorders are found in a significant number of patients, some of the most common being Graves disease, idiopathic thrombocytopenic purpura (ITP), systemic lupus erythematosus (SLE), and hemolytic anemia [1] [2].

• Plethora

  A plethora of abnormalities in lymphocyte subpopulations and expression of activation markers were repeatedly documented in CVID patients, while almost no data are available about lymphocyte subpopulations in IgAD patients. [ncbi.nlm.nih.gov]

• Pediatric Disease

  Despite the wide-spread belief that PIDD is a pediatric disease, 50% of new diagnoses are made in patients over 18 years of age. There are more than 300 PIDD genotypes that sort into a variety of overlapping clinical presentations. [clinicaladvisor.com]

• Recurrent Urinary Tract Infection

  Secretory IgA in recurrent urinary tract infections in childhood. Editorial. Lancet 2:433–434, 1988 Google Scholar 14. Tuttle JP, Sarvas H, Koistinen J: The role of vaginal immunoglobulin A in girls with recurrent urinary tract infections. [doi.org]

• Chronic Productive Cough

  Harrison, Causes of chronic productive cough: An approach to management, Respiratory Medicine, 109, 9, (1105), (2015). [doi.org]

  Pulmonary and sinus disease in primary humoral immunodeficiencies with chronic productive cough. Arch Dis Child. 2003 Dec. 88(12):1101-5. [Medline]. Hermans PE, Diaz-Buxo JA, Stobo JD. Idiopathic late-onset immunoglobulin deficiency. [emedicine.medscape.com]

• Diarrhea

  Treatment Ongoing or recurrent infections, allergies, autoimmune diseases, and chronic diarrhea—or a combination of these issues—can lead doctors to suspect selective IgA deficiency. [omicsonline.org]

  For patients with chronic diarrhea repeated courses of antibiotics such as quinolones or metronidazole had been reported. [medcraveonline.com]

  Gastrointestinal infections or chronic diarrhea are also common. [bundoo.com]

  Some other patients with selective IgA deficiency have gastrointestinal infections and ongoing diarrhea. These common infections affect areas with mucosal surfaces that IgA would protect, and may become chronic. [immunedisease.com]

  However, some people who have IgA deficiency experience pneumonia, ear infections, sinus infections, allergies, asthma and diarrhea. [mayoclinic.org]

• Chronic Diarrhea

  Gastrointestinal infections or chronic diarrhea are also common. [bundoo.com]

  For patients with chronic diarrhea repeated courses of antibiotics such as quinolones or metronidazole had been reported. [medcraveonline.com]

  Selective IgA Deficiency Symptoms & Diagnosis The most common symptom of Selective IgA Deficiency is susceptibility to infections including: • Pneumonia • Sinusitis • Ear infections • Chronic diarrhea caused by gastrointestinal infections IgA deficiency [aaaai.org]

  Treatment Ongoing or recurrent infections, allergies, autoimmune diseases, and chronic diarrhea—or a combination of these issues—can lead doctors to suspect selective IgA deficiency. [omicsonline.org]

  Diagnosis of Selective IgA Deficiency Ongoing or recurrent infections, allergies, autoimmune diseases, and chronic diarrhea—or a combination of these issues—can lead doctors to suspect selective IgA deficiency. [immunedisease.com]

• Oligoarthritis

  Camilleri JP, Moore RH, Griffiths DFR, Williams BD: Selective IgA deficiency associated with glomerulonephritis and oligoarthritis. Ann Rheum Dis 51:123–125, 1992 PubMed Google Scholar 28. [doi.org]

Because of the frequently asymptomatic nature of the disorder, selective IgA immunodeficiency may be difficult to diagnose in a previously healthy individual. But whenever recurrent infections appear, especially with additional gastrointestinal or systemic signs, selective IgA immunodeficiency must be included in the differential [1] [3]. The primary reason is the relatively high prevalence of this condition, with approximately 1 in 400-3000 individuals being affected according to various reports [2]. Thus, the physician should obtain a detailed patient history and conduct a meticulous physical examination during which all important information about the course of symptoms and their progression should be covered. A history of medication use is recommended, as certain drugs may induce low IgA levels [1]. To make a definitive diagnosis, it is necessary to measure levels of immunoglobulins in serum, when the diagnostic criteria are met if levels of IgA are < 0.07 g/L (or < 7 mg/dL) with other serum immunoglobulins being within physiological limits [2] [6]. Further testing of antigliadin and transglutaminase IgG antibodies is mandatory in order to confirm possible celiac disease, as their IgA analogs that are normally identified are absent in these individuals [1].

• Immunoglobulin A Decreased

  Appropriate treatment with immunoglobulin dramatically decreases infection frequency and severity; preventing hospitalization. For most patients, the need for IgG replacement will be lifelong. [clinicaladvisor.com]

• Microcytosis

  Review of the peripheral blood smear revealed mild hypochromia and microcytosis, but was otherwise unremarkable. The mean corpuscular volume was 73 fl (reference, 80–96 fl). The ferritin concentration was 5 ng/mL (reference, 10–150 ng/mL). [jalm.aaccjnls.org]

No specific treatment is available. Some people gradually develop normal levels of IgA without treatment. Treatment involves taking steps to reduce the number and severity of infections. Antibiotics are often needed to treat bacterial infections. [nlm.nih.gov]

Management • patients who do not have any symptoms do not need any treatment. • prevent anaphylactic reaction secondary to blood transfusion. • treatment of associated diseases. 27. Treatment • No specific treatment is available. [slideshare.net]

Antibiotics as needed for treatment and, in severe cases, for prophylaxis Avoidance of blood products that contain IgA Allergic manifestations are treated. [merck.com]

Prognosis is worse if an autoimmune disorder develops. Antibiotics as needed for treatment and, in severe cases, for prophylaxis Avoidance of blood products that contain IgA Allergic manifestations are treated. [merck.com]

Aghamohammadi, Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management, Scandinavian Journal of Immunology, 85, 1, (3-12), (2017). [doi.org]

Prognosis is excellent, although there is an association with autoimmune disease. [en.wikipedia.org]

DLNH is a rare benign condition of unknown etiology that may occur in any age group. 1 It usually regresses in children and it was thought to be associated with delayed type of food hypersensitivity. [medcraveonline.com]

Bruton agammaglobulinemia ( X-linked agammaglobulinemia ) Definition : x-linked recessive disease that causes a complete deficiency of B lymphocytes Epidemiology : appears in boys only Etiology : defect of Bruton's tyrosine kinase expressed in B cells [amboss.com]

Elevated IgM can occur in light of chronic active liver disease, but in this patient it may have been the etiology of his primary immunodeficiency. [acphospitalist.org]

Yong, Michael Tarzi, Ignatius Chua, Bodo Grimbacher and Ronnie Chee, Common Variable Immunodeficiency: An Update on Etiology and Management, Immunology and Allergy Clinics of North America, 28, 2, (367), (2008). [doi.org]

The diagnosis should be directed toward primary and secondary etiologies that are consistent with the clinical presentation and pattern of infections. [worldallergy.org]

Aghamohammadi, Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management, Scandinavian Journal of Immunology, 85, 1, (3-12), (2017). [doi.org]

Bruton agammaglobulinemia ( X-linked agammaglobulinemia ) Definition : x-linked recessive disease that causes a complete deficiency of B lymphocytes Epidemiology : appears in boys only Etiology : defect of Bruton's tyrosine kinase expressed in B cells [amboss.com]

PATHOPHYSIOLOGY “B lymphocytes are unable to produce Ig A” 18. [slideshare.net]

Treatment Antibiotic treatment and prophylaxis IV immunoglobulin therapy IL-12 receptor deficiency Definition : impaired Th response due to IL-12 receptors Etiology : autosomal recessive ; salmonella infections and mycobacterial infections most common Pathophysiology [amboss.com]

There is an increasing role for hospitalists in the care of PIDD patients, given the inherent complexity of the pathophysiology, diagnosis, and treatment. [acphospitalist.org]

Denver Mooney, David Edgar, Gisli Einarsson, Damian Downey, Stuart Elborn and Michael Tunney, Chronic lung disease in common variable immune deficiency (CVID): A pathophysiological role for microbial and non-B cell immune factors, Critical Reviews in [doi.org]

Several lines of evidence suggest that, in many cases, IgAD and common variable immunodeficiency (CVID) have a common pathogenesis, which is discussed further in Pathophysiology. Other data indicate different genetic risk factors. [emedicine.medscape.com]

Prevention There is no means of prevention of Selective IgA Deficiency. • Because IgA Deficiency does not become detectable until approximately six months of age, prenatal and neonatal detection of this disorder is currently not possible. • But genetic [slideshare.net]

Patients with selective IgA deficiency are advised to wear an identification bracelet to prevent inadvertent plasma or immune globulin administration, which could lead to anaphylaxis. [merck.com]

In cases where recurrent infections are a problem, preventative antibiotics may be used to help diminish the frequency of infections. Individuals with IgA deficiency often require a longer course of antibiotics for infections to clear up. [aaaai.org]

While no specific treatment is available for selective deficiency of IgA, it’s important to treat any bacterial infections with antibiotics to prevent them from recurring. [thechart.blogs.cnn.com]

Certain patients who have chronic sinusitis or chronic bronchitis may need to stay on long-term preventive antibiotic therapy (antibiotic prophylaxis). [primaryimmune.org]

1. Yel L. Selective IgA Deficiency. J Clin Immunol. 2010;30(1):10-16.
2. Azzi L, Croveri F, Vinci R, et al. Oral manifestations of selective IgA-deficiency: review and case-report. J Biol Regul Homeost Agents. 2017;31(2 Suppl 1):113-117.
3. Hammarström L, Vorechovsky I, Webster D. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID) Clin Exp Immunol. 2000;120:225–231.
4. Geha RS, Notarangelo LD, Casanova JL, et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol. 2007;120:776–794.
5. Abolhassani H, Gharib B, Shahinpour S, et al. Autoimmunity in patients with selective IgA deficiency. J Investig Allergol Clin Immunol. 2015;25(2):112-119.
6. Aghamohammadi A1, Mohammadi J, Parvaneh N, et al. Progression of selective IgA deficiency to common variable immunodeficiency. Int Arch Allergy Immunol. 2008;147(2):87-92.