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].

• Recurrent Infection

  Patients may be asymptomatic or have recurrent infections or autoimmune disorders; some develop CVID over time, but in others, selective IgA deficiency spontaneously resolves. [merck.com]

  Nevertheless, predisposition toward recurrent infections, autoimmune diseases, and allergies may be present. [symptoma.com]

• Increased Susceptibility to Infections

  Common Variable Immune Deficiency Common Variable Immune Deficiency (CVID) is a frequently diagnosed immunodeficiency, especially in adults, characterized by low levels of serum immunoglobulins and antibodies, which causes an increased susceptibility [hamad.qa]

  Immunol. (1998) [ Pubmed ] Neutrophil dysfunction and increased susceptibility to infection. Ottonello, L., Dapino, P., Pastorino, G., Dallegri, F., Sacchetti, C. Eur. J. Clin. [wikigenes.org]

  Patients with hyper-IgM experience recurrent sinopulmonary infections but also have an increased susceptibility to opportunistic infections, severe neutropenia, and complications with autoimmunity. [acphospitalist.org]

  The clinical manifestations include increased susceptibility to infection and an increased risk for autoimmune disease and malignancy. [worldallergy.org]

  Josias Brito Frazão, Paolo Ruggero Errante and Antonio Condino-Neto, Toll-Like Receptors’ Pathway Disturbances are Associated with Increased Susceptibility to Infections in Humans, Archivum Immunologiae et Therapiae Experimentalis, 10.1007/s00005-013- [doi.org]

• Recurrent Sinusitis

  Abstract A 10-year-old girl with selective IgA deficiency, recurrent sinusitis, otitis media, and juvenile rheumatoid arthritis was given plasma for replacement of IgA. [pediatrics.aappublications.org]

  Since three months she has had recurrent sinusitis infections and her blood test showed this deficiency ( IgA =6), No one in our family has this and both my husband and I are quite healthy. [thechart.blogs.cnn.com]

  The most common presentations are recurrent sinusitis, recurrent otitis and bacterial chest infections such as bronchitis and pneumonia. Any unexpected or unexpectedly complicated infection raises a question of primary immunodeficiency. [clinicaladvisor.com]

  Marshall T Wise and David D Hagaman, An immunological approach to chronic and recurrent sinusitis, Current Opinion in Otolaryngology & Head and Neck Surgery, 10.1097/MOO.0b013e328011bcbf, 15, 1, (10-17), (2007). [doi.org]

• Recurrent Bacterial Infection

  The development of severe, persistent recurrent bacterial infection is a better indicator. A common scenario is repeated episodes of sore throat or upper respiratory tract infection which lead to sinusitis, chronic otitis and bronchitis. [patient.info]

  […] or inflammation Clinical features Recurrent nonsuppurative bacterial infections (e.g., skin and mucosal), impaired wound healing Omphalitis in the newborn (delayed separation of the cord) Diagnosis Leukocytosis during infection ( neutrophils are absent [amboss.com]

  Recurrent bacterial sinus and pulmonary infections are the hallmark of antibody primary immunodeficiencies. [aafp.org]

• Recurrent Respiratory Infection

  Immunoglobulin levels and function in pre-school children with recurrent respiratory infections. D Isaacs, A D Webster, and H B Valman. Clin Exp Immunol. 1984 November; 58(2): 335–340. [allergycases.blogspot.com]

  […] to infection [ more ] 0002719 Recurrent respiratory infections Frequent respiratory infections Multiple respiratory infections respiratory infections, recurrent Susceptibility to respiratory infections [ more ] 0002205 Sporadic No previous family history [rarediseases.info.nih.gov]

  Recurrent respiratory infections are common but this is by no means pathognomonic, as every GP will be aware of the 'sickly child' who seems to acquire infections from his or her siblings frequently. [patient.info]

• Diarrhea

  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]

  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]

  Traditionally, CD was a relatively rare and extremely severe disorder seen primarily in young children, with abdominal distension, diarrhea, malabsorption, and failure to thrive. [jalm.aaccjnls.org]

  Food allergies may result in symptoms such as diarrhea or abdominal cramps. A link between Selective IgA Deficiency and allergic rhinitis or eczema is uncertain. [vaccinechoicecanada.com]

• Chronic Diarrhea

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

  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]

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

  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]

• Lactose Intolerance

  In addition to infections (bacteria, but also protozoa, an example being Giardia lamblia), malabsorption and lactose intolerance are often encountered in symptomatic patients. [symptoma.com]

• Urticaria

  Selective Immunoglobulin A Deficiency. ( 30101931 ) Harada K...Otsuka F 2018 3 Prevalence of dermatologic diseases among patients with selective immunoglobulin A deficiency. ( 28052804 ) Magen E...Vardy D 2017 4 Co-Occurrence of Chronic Spontaneous Urticaria [malacards.org]

• Hunger

  Well Photo Credit Illustration by Celia Jacobs Outrunning Hunger Intense exercise may change the way certain neurons influence our appetite and metabolism. [nytimes.com]

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]

• Helicobacter Pylori

  Diffuse intestinal nodular lymphoid hyperplasia in an immunoglobulin–A–deficient patient with Helicobacter pylori infection. Endoscopy. 2014; 46(S 01):E568–E569. ©2016 Elkholy et al. [medcraveonline.com]

  Ah‐Mee Park, Seiichi Omura, Mitsugu Fujita, Fumitaka Sato and Ikuo Tsunoda, Helicobacter pylori and gut microbiota in multiple sclerosis versus Alzheimer's disease: 10 pitfalls of microbiome studies, Clinical and Experimental Neuroimmunology, 8, 3, (215 [doi.org]

  […] ynarski W 2016 6 Role of apoptosis in common variable immunodeficiency and selective immunoglobulin A deficiency. ( 26795881 ) Yazdani R...Aghamohammadi A 2016 7 Helicobacter pylori infection in patients with selective immunoglobulin a deficiency. ( 26749258 [malacards.org]

  Staining for Helicobacter pylori was negative. Biopsies of the duodenum revealed patchy areas of villus blunting with crypt hyperplasia and increased intraepithelial lymphocytes ( Fig. 1 ). [jalm.aaccjnls.org]

• Hepatocellular Carcinoma

  Aronow, Harit Desai, Harshad Amin, Mala Sharma and Arye Rubinstein, A Case of Explosive Progression of Hepatocellular Carcinoma in a Patient with Common Variable Immunodeficiency (CVID), Journal of Gastrointestinal Cancer, 10.1007/s12029-010-9158-8, 41 [doi.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]

Immune deficiency treatment Antibiotics, virostatics, and antimycotics. [amboss.com]

Overview Symptoms & Diagnosis Treatment & Management Selective IgA Deficiency Overview Selective IgA Deficiency is the most common primary immunodeficiency disease (PIDD). [aaaai.org]

Some conditions such as selective IgAD have a good prognosis. [patient.info]

The prognosis in primary immunodeficiency disorders is variable and depends on the specific disorder. Congenital B-cell immunodeficiencies B-cell defects ( humoral immunity deficiencies ) account for 50–60% of all primary immunodeficiencies. [amboss.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]

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

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]

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]

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

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]

The epidemiology, pathogenesis, clinical phenotype, diagnosis, prognosis, management and treatment in patients with SIgAD have been reviewed. [doi.org]

Selective IgA deficiency: epidemiology, pathogenesis, clinical phenotype, diagnosis, prognosis and management. Scand J Immunol. 2017;85(1):3-12. [ Links ] 2. Lu P, Ling B, Wang N, Hammarstrom L. [scielo.org.co]

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]

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.com]

If the disorder is severe, antibiotics are given in advance to prevent infections from developing. [msdmanuals.com]

There is no known way to prevent IgA deficiency. [healthcommunities.com]

Individuals with demonstrated anti-IgA are thereafter committed to receiving IgA-depleted cellular products or IgA-deficient plasma and derivatives to prevent recurrent severe reactions. [ncbi.nlm.nih.gov]

International Symposium on Prevention of Allergic Disease 85,1-2=46; 87,1-2=47; 89,1-2=48; 91,1-2=49 usw. von: American Academy of Allergy and Immunology: Annual meeting 97,1,3=52; 103,1,2=55; 105,1,2=56; 107,2,2=57; 109,1,Suppl.=58; 111,2,Suppl.=[59] [zdb-katalog.de]

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.