Crohn's Disease Associated With Selective Immunoglobulin a Deficiency

Iizuka, Masahiro; Itou, Hiroaki; Sato, Michiko; Yukawa, Michihiro; Shirasaka, Tomoyuki; Chiba, Mitsuro; Watanabe, Sumio

doi:10.1046/j.1440-1746.2001.t01-5-02379.x

Cited by 18 publications

(6 citation statements)

References 3 publications

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“…The 2 cases reported here warrant careful evaluation of every patient with a suspected, concurrent combination of these conditions, since their combination may predispose to a complicated and possibly fatal outcome. IgA deficiency may be underestimated, due to the known immune activation which usually accompanies chronic inflammatory bowel disease (1,2). The serum IgA deficiency in our patients did not influence the positive outcome of antimicrobial therapy in our series, since both patients achieved a stable clinical and microbiological cure (in contrast to the 3 described cases of necrotizing fasciitis occurring during Chron's disease) (3 Á/5).…”

Section: Discussionmentioning

confidence: 59%

“…To the best of our knowledge only 3 cases of selective IgA deficiency have been reported in patients with Chron's disease (1,2). Two exceptional cases characterized by severe, lifethreatening bacterial complications in young males with a recently diagnosed moderate Chron's disease and an unexpected, concurrent selective IgA immunodeficiency, are described.…”

Section: Introductionmentioning

confidence: 94%

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

Manfredi

Coronado²,

Marinacci

et al. 2004

Scandinavian Journal of Infectious Diseases

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Life-threatening necrotizing fasciitis and relapsing Lemierre syndrome associated with Fusobacterium necrophorum septicaemia occurred in young adults with a moderate Chron's disease and a missed profound IgA deficiency. This unexpected association of a chronic bowel inflammatory syndrome with prominent IgA abnormalities and severe bacterial infection deserves careful attention by physicians faced with young patients with Chron's disease.

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Section: Discussionmentioning

confidence: 59%

Section: Introductionmentioning

confidence: 94%

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

Manfredi

Coronado²,

Marinacci

et al. 2004

Scandinavian Journal of Infectious Diseases

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“…Crohn's-like lesions may appear in the large and small intestine of patients with the autosomal recessive glycogen storage disease type 1b (136). Several immunodeficiency disorders, including hereditary angioedema, hypoglammaglobulinemia, and selective immunoglobulin A (IgA) deficiency are also associated with IBD (87).…”

Section: Genetic Factors Underlying Resistance and Susceptibilitymentioning

confidence: 99%

Johne's Disease, Inflammatory Bowel Disease, andMycobacterium paratuberculosis

Chacón

Bermudez

Barletta³

2004

Annu. Rev. Microbiol.

209

148

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Johne's disease is a chronic diarrhea affecting all ruminants. Mycobacterium avium subsp. paratuberculosis (MAP), a slowly growing mycobacteria, is the etiologic agent. There is also a concern that MAP might be a causative agent of some cases of inflammatory bowel disease in humans, especially Crohn's disease. Food products including pasteurized bovine milk have been suggested as potential sources of human infection. This review addresses microbial factors that may contribute to its pathogenicity. In addition, the experimental evidence defining MAP as the cause of Johne's disease and the issues and controversies surrounding its potential pathogenic role in humans are discussed.

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“…Other associations between IgA deficiency and gastrointestinal diseases include lymphomas, pernicious anemia, 85,86 Crohn disease, 87,88 ulcerative colitis, 89 chronic hepatitis, 90 and biliary cirrhosis, 91 although the actual prevalence of each is not well defined in the literature.…”

Section: Xlamentioning

confidence: 99%

Pathogenesis and treatment of gastrointestinal disease in antibody deficiency syndromes

Agarwal

Mayer

2009

Journal of Allergy and Clinical Immunology

131

154

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Primary humoral immune deficiencies are characterized by limited antibody responses secondary to either impaired B-lymphocyte development or B-cell responses to T-lymphocyte signals. Given that the gastrointestinal tract is the largest lymphoid organ in the body, it is not surprising that intestinal diseases are common in immunodeficiency. These gastrointestinal diseases can be classified into one of 4 groups, infection, malignancy, inflammatory, and autoimmune, and can mimic other known disease processes, such as inflammatory bowel disease and celiac sprue. The exact pathogenesis of these gastrointestinal disorders in the setting of systemic immunodeficiency is still under investigation. However, studies suggest that defects in antibody deficiency alone do not result in gastrointestinal disease but rather that defects in cellular immunity are also involved. Treatment is difficult given an already immunocompromised state, and often therapy with immunomodulators is required for more severe processes.

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Crohn's Disease Associated With Selective Immunoglobulin a Deficiency

Cited by 18 publications

References 3 publications

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

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

Johne's Disease, Inflammatory Bowel Disease, andMycobacterium paratuberculosis

Pathogenesis and treatment of gastrointestinal disease in antibody deficiency syndromes

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