Both selective IgM deficiency and hyper-IgM have been highly associated with recurrent upper and lower respiratory infections. Visit the group’s website or contact them to learn about the services they offer. The prevalence of selective IgM deficiency is 0.03 –1% of the population.212 Between 60% and 80% of patients suffer from recurrent bacterial and viral infections. Pediatrics. [Full Text]. Ideura G, Agematsu K, Komatsu Y, et al. Since IgM may have a different range of specificities than placentally-transferred maternal IgG, infants can succumb to overwhelming infections such as meningitis, pneumonia, and gram-negative sepsis. Indian Pediatrics. Serum IgM levels in SLE have been reported to be increased during the early stages and during periods of disease activity. Srividya Sridhara, MD Resident Physician, Department of Internal Medicine, University of OklahomaDisclosure: Nothing to disclose. Whether a person has primary or secondary SIgMD (associated with another underlying condition) also plays a role in the types of symptoms a person has, as they may also have symptoms of their underlying condition. It is mostly found in the bloodstream, although mucosal IgM has been noted in physiologic amounts. Whether this mechanism is relevant to human SLE remains to be established.79, Francisco P. 2008. The role of IgM can also be studied in mutant mice that cannot produce the AID protein, which is required both for class switch recombination and somatic hypermutation. Survival half-life of IgG in SLE patients is decreased at an average of 8.2 days compared with 18 days in normal controls with an average of 10.1% of total body IgG catabolized daily compared with a mean of 3.9% in normal controls.115. [Medline]. 29(7):811-815. The survival half-life of IgG in these patients is decreased at an average of 8.2 days, compared with 18 days in normal patients with an average of 10.1% of total body IgG catabolized daily compared with a mean of 3.9% in normal patients.64. Laboratory studies disclose selective IgM deficiency (<20–30 mg/dl in adults, and <10–15 mg/dl in infants and children), when measured at least twice on separate occasions. Over 80% of IgM-deficient patients present with recurrent infections, usually in the respiratory tract. September 1, 2020, NIH-Supported Research Survey to Examine Impact of COVID-19 on Rare Diseases Community  Reported IgM concentrations in SIgM deficiency vary from 40 mg/dL (though some sources say 20 mg/dL) to undetectable levels (reference range 45-150 mg/dL in adults). There are no other underlying immunodeficiencies. Yel L, Ramanuja S, Gupta S. Clinical and immunological features in IgM deficiency. SIgM deficiency may occur as a primary or secondary condition. Selective IgM patients are managed in the same fashion as for other antibody deficiencies. 57(1):99-105. Few PIDs have been described as consistently associated with SLE or lupus-like manifestations (Table 49.9): Homozygous deficiencies of early components of the classical complement pathway; Selective or partial immunoglobulin deficiencies, particularly isolated IgA and IgM deficiencies; and, CGD, in both patients and female carriers of the X-linked CGD allele (Table 49.2).13,14,21,38–41, Complete deficiency of any of the early components of the classical complement pathway represents the strongest single genetic risk factor for the development of SLE identified to date. Mutant (sIgM−/−) mice that cannot produce secreted IgM have increased susceptibility to bacterial peritonitis, as demonstrated in the cecal ligation and puncture model (Boes et al., 1998a). Selective IgA deficiency has been described in a number of breeds of dogs, including the German shepherd, Shar-Pei, Irish setter, and beagle.  Recent series report IgM levels of 29.7±8.7 mg/dL (mean±SD) for adults and 16.5±13.8 (mean±SD) in children. The presence of these antibodies can cause a severe anaphylactic blood transfusion reaction.126,128,129, Serum IgE levels are higher in SLE patients with active disease, including those with nephritis compared with patients in remission.130 IgE ANAs specific for dsDNA, Sm, Ro/SSA, and La/SSB are prevalent in two large SLE cohorts and appeared to be highly associated with disease activity similar to IgG ANA specific for these autoantigens.131 Immune complexes of IgE anti-dsDNA and DNA antigen activate plasmacytoid dendritic cells leading to the secretion of IFN-α supports a pathogenic role for autoreactive IgE in SLE.132 Other investigators reported significantly higher total serum IgE levels in SLE patients with quiescent disease than in those with active disease. In Handbook of Vertebrate Immunology, 1998. [10, 11, 12] They may also have autoimmune disease including glomerulonephritis and osteomyelitis from which organisms are not recoverable, [Medline]. Resistance was restored by transfer of IgM from unimmunized mice, indicating a role for natural IgM. QuismorioJr., Karina D. Torralba, in Dubois' Lupus Erythematosus and Related Syndromes (Eigth Edition), 2013, Measurement of baseline serum immunoglobulin levels is useful to diagnose primary immunodeficiencies associated with SLE, including combined variable immunodeficiency and selective immunoglobulin A (IgA) deficiency, and to identify hypogammaglobulinemia that can occur as a result of treatment or as part of the disease. Hong R, Gupta S. Selective immunoglobulin M deficiency in an adult with Streptococcus pneumoniae sepsis and invasive aspergillosis. J Clin Immunol. Gradually, current state-of-the-art laboratory technology is being applied in studying patients with SIgM deficiency, though much remains to be learned. Serum total IgE levels were not associated with the presence of antinuclear IgE.133. Patients may develop prolonged or life-threatening infections caused by both encapsulated bacteria and viruses, especially in infancy. Selective Immunoglobulin M Deficiency Presenting with Recurrent Impetigo: A Case Report and Review of the Literature. CVID should be suspected in SLE patients with quiescent disease activity and not on immunosuppressive treatment but with recurrent sinopulmonary infections.112,128,132,134 Table 46.11 lists Ig abnormalities and their association with SLE. In some infants, selective IgM deficiency may be transient.220 For other patients insufficient data are available for a prognosis. (1977), and is now an established clinical entity (McClure et al., 1996). Makay B, Unsal E, Anal O, Gunes D, Men S, Cakmakci H, et al. In selective IgM deficiency, the genetics is not well known, although it has been hypothesized that there is a functional defect in B cell differentiation prior to the secretion of IgM. IgG deficiency has been noted to occur in SLE patients and may be associated with infections. In most instances there are normal numbers of IgA-producing plasma cells, suggesting an inability to synthesize or secrete IgA. Belgemen T, Suskan E, Dogu F, Ikinciogullari A. Some horses reach adulthood before they show clinical signs of selective IgM deficiency. Int Arch Allergy Immunol. Copyright © 2020 Elsevier B.V. or its licensors or contributors. Approximately 14% of reported patients with selective IgM deficiency have autoimmune conditions including a few patients with SLE.69, IgA, the second most abundant immunoglobulin, exists in two isotypes—IgA1 and IgA2—with the former as the predominant isotype in the serum. 62(6):547-52. De la Concha EG, Garcia-Rodriguez MC, Zabay JM. Patients with SIgM deficiency are susceptible to recurrent sepsis and overwhelming infection with encapsulated bacteria (eg, Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae). 2008 Aug. 12(4):300-4. In contrast, however, all four IgG subclasses were detected in the renal glomerular deposits.66, Ten percent of serum immunoglobulin is IgM, which has a half-life of 5 to 10 days. IgG deficiency has been noted to occur in patients with SLE and may be associated with infections. Common presentations include otitis media, bronchitis, chronic rhinosinusitis, pneumonia, and sepsis.204 Selective IgM deficiency may also be asymptomatic. The most common forms are selective IgM deficiency and selective IgA deficiency, characterized by a serum level of IgM or IgA, respectively, that is at least two standard deviations below normal. 103:1971-2. Monomeric IgA1 has antiinflammatory functions theorized to work via FcαRI-inhibitory signaling.70. 2014 Apr. We want to hear from you. [Medline]. May 22, 2020, NCATS Translational Approach Addresses COVID-19  Recent series report IgM levels of 29.7±8.7 mg/dL (mean±SD) for adults and 16.5±13.8 (mean±SD) in children. Inoue T, Okumara Y, Shirahama M, et al. ADA deficiency should be suspected in all patients who show recurrent infections during the first year of life. Low Immunoglobulin M. Low levels of IgM may mean your immune system is not working optimally. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. 46(2):104-11. When … 137693-overview If you have problems viewing PDF files, download the latest version of Adobe Reader, For language access assistance, contact the NCATS Public Information Officer, Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311. IgG deficiencies can occur at any age. SLE itself is typified by high levels of serum Igs and circulating autoantibodies. Clin Exp Immunol. Clin J Sport Med. 6(2):130-5. [Medline]. However, a reasonable normal range is 45-250mg/dl. 150(3):291-8. http://www.uptodate.com/contents/selective-igm-deficiency, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5591887/, http://kidshealth.org/en/parents/test-immunoglobulins.html, https://emedicine.medscape.com/article/137693-overview, http://va8ef7lf8s.search.serialssolutions.com/?sid=Entrez:PubMed&id=pmid:26928000, https://www.ncbi.nlm.nih.gov/pubmed/28730517. In subjects with partial ADA deficiency, the enzyme is deficient in red blood cells, but sufficient residual ADA activity is present in their lymphocytes. Prophylactic antibiotics and prompt treatment of febrile illness are crucial. Pediatr Clin North Am. We remove all identifying information when posting a question to protect your privacy.
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