Autoimmunity in IgA Deficiency: Revisiting the Role of IgA as a Silent Housekeeper

Jacob, Cristina Miuki Abe; Pastorino, Antônio Carlos; Fahl, Kristine; Carneiro‐Sampaio, Magda; Monteiro, Renato C.

doi:10.1007/s10875-007-9163-2

Cited by 148 publications

(129 citation statements)

References 25 publications

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“…IgA deficiency, the most common primary immunodeficiency with a prevalence of 1 per 500-700 among Europeans, is associated with both systemic and organspecific autoimmune diseases (33). The prevalence of IgA deficiency in our study was one of 59 in contrast to one of eight in two previous reports (9,18), which might be influenced by their selection of patients.…”

Section: Discussioncontrasting

confidence: 66%

Hypoparathyroidism and autoimmunity in the 22q11.2 deletion syndrome

Lima

Abrahamsen

Wolff

et al. 2011

European Journal of Endocrinology

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Objective: To characterize the endocrine and autoimmune disturbances with emphasis on parathyroid dysfunction in patients with 22q11.2 deletion syndrome (22q11.2 DS). Design: In this nationwide survey; 59 patients (age 1-54 years) out of 86 invited with a 22q11.2 DS were recruited through all the genetic institutes in Norway. Methods: Data was collected from blood tests, medical records, a physical examination and a semistructured interview. We registered autoimmune diseases and measured autoantibodies, hormone levels and HLA types. Results: Twenty-eight (47%) patients had hypoparathyroidism or a history of neonatal or transient hypocalcemia. Fifteen patients had neonatal hypocalcemia. Fourteen patients had permanent hypoparathyroidism including seven (54%) of those above age 15 years. A history of neonatal hypocalcemia did not predict later occurring hypoparathyroidism. Parathyroid hormone levels were generally low indicating a low reserve capacity. Twenty-eight patients were positive for autoantibodies. Six (10%) persons had developed an autoimmune disease, and all were females (P!0.02). Hypoparathyroidism correlated with autoimmune diseases (P!0.05), however, no antibodies were detected against the parathyroid glands. Conclusions: Hypoparathyroidism and autoimmunity occur frequently in the 22q11.2 DS. Neonatal hypocalcemia is not associated with later development of permanent hypoparathyroidism. Hypoparathyroidism may present at any age, also in adults, and warrants regular measurement of calcium levels. Hypoparathyroidism and autoimmunity occur frequently together. Our findings of autoimmune diseases in 10% of the patients highlight the importance of stringent screening and follow-up routines.

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Section: Discussioncontrasting

confidence: 66%

Hypoparathyroidism and autoimmunity in the 22q11.2 deletion syndrome

Lima

Abrahamsen

Wolff

et al. 2011

European Journal of Endocrinology

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“…Association of selective IgA deficiency and atopy has been reported with a variable rate ranging between 13% and 58% (11,25,26). Increased aeration on lung graphy was found with a markedly higher rate in the patients with selective IgA deficiency compared to the other PIDs (44.4%).…”

Section: Discussionmentioning

confidence: 98%

Profile of the patients who present to immunology outpatient clinics because of frequent infections

et al. 2014

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Aim: We aimed to determine the rate of primary immune deficiency (PID) among children presenting to our immunology outpatient clinic with a history of frequent infections and with warning signs of primary immune deficiency. Material and Methods:The files of 232 children aged between 1 and 18 years with warning signs of primary immune deficiency who were referred to our pediatric immunology outpatient clinic with a complaint of frequent infections were selected and evaluated retrospectively. Results: Thirty-six percent of the subjects were female (n=84) and 64% were male (n=148). PID was found in 72.4% (n=164). The most common diagnosis was selective IgA deficiency (26.3%, n=61). The most common diseases other than primary immune deficiency included reactive airway disease and/or atopy (34.4%, n=22), adenoid vegetation (12.3%, n=8), chronic disease (6.3%, n=4) and periodic fever, aphtous stomatitis and adenopathy (4.6%, n=3). The majortiy of the subjects (90.5%, n=210) presented with a complaint of recurrent upper respiratory tract infection. PID was found in all subjects who had bronchiectasis. The rates of the diagnoses of variable immune deficiency and Bruton agammaglubulinemia (XLA) were found to be significantly higher in the subjects who had lower respiratory tract infection, who were hospitalized because of infection and who had a history of severe infection compared to the subjects who did not have these properties (p<0.05 and p<0.01, respectively). Growth and developmental failure was found with a significantly higher rate in the patients who had a diagnosis of severe combined immune deficiency or hyper IgM compared to the other subjects (p<0.01). No difference was found in the rates of PID between the age groups, but the diagnosis of XLA increased as the age of presentation increased and this was considered an indicator which showed that patients with XLA were being diagnosed in a late period. Conclusions: It was found that the rate of diagnosis was considerably high (72.4%), when the subjects who had frequent infections were selected by the warning signs of PID. (Türk Ped Arş 2014; 49: 210-6)

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“…IgAD patients can also present with comorbidities such as chronic intestinal inflammatory disorders and autoimmune diseases, demonstrating the critical role of IgA in regulating immunephysiologic homeostasis of the host. [69][70][71][72] IgA-deficiency in humans is often asymptomatic because in the absence of IgA, IgM can exert a compensatory function. 73 However, in rabbits that have been treated from birth with anti-allotypic Ab to suppress Ab production, IgA-producing cells are the first escapees.…”

Section: Functions Of Igamentioning

confidence: 99%