Chron's Disease, Rare Association with Selective IgA Immunodeficiency, and Development of Life-threatening Bacterial Infections

Manfredi, Roberto; Coronado, O.; Marinacci, Ginevra; Righi, Mauro; Calza, Leonardo

doi:10.1080/00365540410020190

Cited by 10 publications

(6 citation statements)

References 5 publications

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“…We found that the concentrations of IgG, but not IgM and IgA, in the patients were significantly higher than that in the HC and correlated positively with the values of Mayo clinic score and the concentrations of serum CRP. Although the IgA deficiency, especially for the lack of secretory component in UC or CD has been reported in several UC cases, we did not observe such deficiency in those 23 UC patients in this population. The difference may stem from a small group of patients we studied.…”

Section: Discussioncontrasting

confidence: 85%

Circulating memory B cells and plasmablasts are associated with the levels of serum immunoglobulin in patients with ulcerative colitis

Wang

Jiang

Zhu

et al. 2016

J Cellular Molecular Medi

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Humoural immunity is crucial for the pathogenesis of ulcerative colitis (UC), but the precise perturbation of B cell immunity is poorly understood. This study is aimed at evaluating the numbers of different subsets of circulating memory B cells, plasmablasts, and the levels of serum immunoglobulin in UC patients. Total of 23 patients with active UC and 14 healthy controls (HC) were examined for the numbers of different subsets of circulating memory B cells and plasmablasts before and after treatment with mesalazine for 8–12 weeks by flow cytometry. Disease activity was evaluated by the Mayo clinic score. The levels of serum immunoglobulin, C‐reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were measured in individual subjects. In comparison with that in HC, significantly reduced numbers of IgG+ IgD− CD27+ CD19+ memory B cells, increased numbers of CD20− CD19+ plasmablast subsets, and higher serum IgG levels were detected in UC patients. The concentrations of serum IgG, the numbers of CD138+ CD38+ CD20− CD19+, and IgG+ CD38+ CD20− CD19+ plasmablasts were negatively associated with the numbers of IgG+ IgD− CD27+ CD19+ memory B cells. Furthermore, the values of Mayo clinic score, CRP, or ESR in UC patients were negatively correlated with the numbers of IgG+ IgD− CD27+ CD19+ memory B cells, while positively correlated with the serum IgG levels and the numbers of plasmablast subsets. Following treatment with mesalazine, the numbers of circulating IgG+ IgD− CD27+ CD19+ memory B cells were significantly increased, while the numbers of CD138+ CD38+ CD20− CD19+ and IgG+ CD38+ CD20− CD19+ plasmablasts were reduced in UC patients. These decreased IgG+ IgD− CD27+ CD19+ memory B cells and increased plasmablasts may be involved in the pathogenic process of UC.

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Section: Discussioncontrasting

confidence: 85%

Circulating memory B cells and plasmablasts are associated with the levels of serum immunoglobulin in patients with ulcerative colitis

Wang

Jiang

Zhu

et al. 2016

J Cellular Molecular Medi

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“…The question of whether IgA deficiency (and in particular lack of the free secretory component)41 predisposes to IBD has been highlighted by several case reports describing association with CD42–44 or UC 41, 45–48. Since the IgA system represents a distinct local immune mechanism in the gut, under constant exposure to bowel antigens, it was postulated that focal deficiency might translate into increased permeability to pyogenic organisms and other antigenic material 49.…”

Section: B‐cell Dysfunctionmentioning

confidence: 99%

Inflammatory bowel diseases in patients with adaptive and complement immunodeficiency disorders

Marks

Seymour

Sewell

et al. 2010

Inflammatory Bowel Diseases

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Crohn's disease and ulcerative colitis are idiopathic chronic inflammatory diseases that primarily affect the gastrointestinal tract. The underlying causes remain poorly understood, but there is a growing body of evidence advocating a likely primary pathogenic role for immunodeficiency in the development of Crohn's lesions. Concordantly, a number of congenital immunodeficiencies disrupting the cellular innate immune system strongly predispose to noninfectious, Crohn's-like inflammatory bowel disease. There are case reports and series suggesting that the same may be true for some of the congenital adaptive and complement immunodeficiencies. This review considers and critiques these potential associations.

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“…Пациентка Х., 46 [8][9][10][11][12][13]нормальный уровень IgM (0,98 г/л при норме 0,5 г/л),снижение уровня IgA (до 1,19 г/л при норме 1,1 г/л). В 2011 году при повторном анализе IgG составил 16,0 г/л,98 г/л,12 г/л,что Подведем итоги проведенного обследования.…”

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“…Причиной терминального илеита часто служит болезнь Крона. Случаи сочетания селективного дефицита IgA и болезни Крона описаны в литературе [12,13]. Вместе с тем диагноз болезни Крона вызывал сомнения, в первую очередь из-за отсутствия яркой клинической картины (не было потери веса, абдоминальной боли; длительный анамнез протекал достаточно спокойно).…”

unclassified

“…Было выявлено снижение сывороточного пепсиногена I до 2,2 мкг/л (норма 30-150), снижение PGI/ [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20], существенное повышение уровня гастрина 17 до 115,9 пмоль/л (норма < 7). Таким образом, получено дополнительное подтверждение атрофии слизистой оболочки тела желудка, что обычно является следствием аутоиммунного гастрита.…”

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A Cause of Diarrhea in a Patient with Selective Immunoglobulin A Deficiency

Gonik

Щербаха²,

Юрьева

et al. 2019

Rossijskij žurnal gastroènterologii, gepatologii, koloproktolog

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Aim. To present a clinical case with differential diagnosis of the cause of diarrhea in a patient with selective IgA deficiency.Key findings. A 46-year-old woman complained of a mushy stool without pathological impurities up to 5 times a day. An outpatient colonoscopy revealed signs of terminal ileitis. The patient’s medical history included selective IgA and vitamin B12 deficiency. Despite the absence of antibodies to parietal cells and intrinsic factor, a diagnosis of autoimmune gastritis was established on the basis of histologically confirmed atrophy of the stomach; decrease in serum pepsinogen I and the ratio of pepsinogen I to pepsinogen II, hypergastrinemia; vitamin B12 deficiency; proven autoimmune thyroiditis (antibodies to thyroglobulin and thyroid peroxidase in the diagnostic titre). Histologically, lymphoid hyperplasia of the stomach was determined. Colonoscopy revealed erosive terminal ileitis and colitis, as well as an increase in the lymphoid follicles of the sub-mucosal layer in the ileum and all parts of the colon, which gave a rough pattern to the mucosa. Histological examination revealed pronounced follicular hyperplasia of the lymphoid tissue of the ileum and colon. The clinical diagnosis was established as follows. The combined main disease: 1. Selective IgA deficiency with a nodular lymphoid hyperplasia of the stomach, small and large intestine. 2. Autoimmune gastritis with erosions in the gastric fornix. Vitamin B12 deficiency. Concomitant diseases: Autoimmune thyroiditis.Conclusion. The presented clinical observation demonstrates the importance of recognizing nodular lymphoid hyperplasia, which is established as the cause of diarrhea in a patient with selective IgA deficiency after excluding the diagnosis of Crohn’s disease. A specific feature of the presented clinical case is a combination of autoimmune gastritis and autoimmune thyroiditis with selective IgA deficiency.

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Chron's Disease, Rare Association with Selective IgA Immunodeficiency, and Development of Life-threatening Bacterial Infections

Cited by 10 publications

References 5 publications

Circulating memory B cells and plasmablasts are associated with the levels of serum immunoglobulin in patients with ulcerative colitis

Circulating memory B cells and plasmablasts are associated with the levels of serum immunoglobulin in patients with ulcerative colitis

Inflammatory bowel diseases in patients with adaptive and complement immunodeficiency disorders

A Cause of Diarrhea in a Patient with Selective Immunoglobulin A Deficiency

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