Immmunodeficiencies and autoimmune diseases: Common variable immunodeficiency and Crohn-like

Dueñas, Cristina Saldaña; Iturria, Saioa Rubio

doi:10.17235/reed.2015.3872/2015

Cited by 5 publications

(8 citation statements)

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“…Treatment of non-infectious GI disease in CVID includes corticosteroids, elimination of bacterial overgrowth with antibiotics, 5-aminosalicylic acid, 6-mercaptopurine, and azathioprine [ 301 , 311 , 312 ]. Besides, several groups reported substantial efficacy of targeted biological therapies, including anti-TNF-α drugs (infliximab and adalimumab), and the anti-IL−12/IL−23 monoclonal antibody ustekinumab [ 297 , 305 , 313 , 314 , 315 , 316 , 317 ]. Vedolizumab, an inhibitor of α4β7integrin, was also used in CVID-enteropathy, although with different results [ 93 , 318 ].…”

Section: Inflammatory Bowel Disease In Common Variable Immunodefimentioning

confidence: 99%

Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults

Malesza

Krela‐Kaźmierczak

et al. 2020

IJMS

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In recent years, the incidence of immune-mediated gastrointestinal disorders, including celiac disease (CeD) and inflammatory bowel disease (IBD), is increasingly growing worldwide. This generates a need to elucidate the conditions that may compromise the diagnosis and treatment of such gastrointestinal disorders. It is well established that primary immunodeficiencies (PIDs) exhibit gastrointestinal manifestations and mimic other diseases, including CeD and IBD. PIDs are often considered pediatric ailments, whereas between 25 and 45% of PIDs are diagnosed in adults. The most common PIDs in adults are the selective immunoglobulin A deficiency (SIgAD) and the common variable immunodeficiency (CVID). A trend to autoimmunity occurs, while gastrointestinal disorders are common in both diseases. Besides, the occurrence of CeD and IBD in SIgAD/CVID patients is significantly higher than in the general population. However, some differences concerning diagnostics and management between enteropathy/colitis in PIDs, as compared to idiopathic forms of CeD/IBD, have been described. There is an ongoing discussion whether CeD and IBD in CVID patients should be considered a true CeD and IBD or just CeD-like and IBD-like diseases. This review addresses the current state of the art of the most common primary immunodeficiencies in adults and co-occurring CeD and IBD.

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Section: Inflammatory Bowel Disease In Common Variable Immunodefimentioning

confidence: 99%

Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults

Malesza

Krela‐Kaźmierczak

et al. 2020

IJMS

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“…Steroid therapy was the most frequently reported treatment regimen for granulomatous disease in CVID ( Tables 5 and 6). For CVID+EGD, steroid monotherapy was the most frequently reported regimen (21 of 53 treatment courses), with 85.7% of treatment courses scored as effective ( Table 5) (17,19,20,(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40). For CVID+PGD, steroid monotherapy also was the most frequently reported treatment regimen (15 of 64 treatment courses); 66.7% of these treatment courses were associated with remission of granulomatous disease (Table 6) (29,(41)(42)(43)(44)(45)(46)(47)(48)(49)(50).…”

Section: Administered Treatment Regimens In Granulomatous Disease In mentioning

confidence: 99%

What Works When Treating Granulomatous Disease in Genetically Undefined CVID? A Systematic Review

et al. 2020

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BackgroundGranulomatous disease is reported in at least 8–20% of patients with common variable immunodeficiency (CVID). Granulomatous disease mainly affects the lungs, and is associated with significantly higher morbidity and mortality. In half of patients with granulomatous disease, extrapulmonary manifestations are found, affecting e.g. skin, liver, and lymph nodes. In literature various therapies have been reported, with varying effects on remission of granulomas and related clinical symptoms. However, consensus recommendations for optimal management of extrapulmonary granulomatous disease are lacking.ObjectiveTo present a literature overview of the efficacy of currently described therapies for extrapulmonary granulomatous disease in CVID (CVID+EGD), compared to known treatment regimens for pulmonary granulomatous disease in CVID (CVID+PGD).MethodsThe following databases were searched: Embase, Medline (Ovid), Web-of-Science Core Collection, Cochrane Central, and Google Scholar. Inclusion criteria were 1) CVID patients with granulomatous disease, 2) treatment for granulomatous disease reported, and 3) outcome of treatment reported. Patient characteristics, localization of granuloma, treatment, and association with remission of granulomatous disease were extracted from articles.ResultsWe identified 64 articles presenting 95 CVID patients with granulomatous disease, wherein 117 different treatment courses were described. Steroid monotherapy was most frequently described in CVID+EGD (21 out of 53 treatment courses) and resulted in remission in 85.7% of cases. In CVID+PGD steroid monotherapy was described in 15 out of 64 treatment courses, and was associated with remission in 66.7% of cases. Infliximab was reported in CVID+EGD in six out of 53 treatment courses and was mostly used in granulomatous disease affecting the skin (four out of six cases). All patients (n = 9) treated with anti-TNF-α therapies (infliximab and etanercept) showed remission of extrapulmonary granulomatous disease. Rituximab with or without azathioprine was rarely used for CVID+EGD, but frequently used in CVID+PGD where it was associated with remission of granulomatous disease in 94.4% (17 of 18 treatment courses).ConclusionAlthough the number of CVID+EGD patients was limited, data indicate that steroid monotherapy often results in remission, and that anti-TNF-α treatment is effective for granulomatous disease affecting the skin. Also, rituximab with or without azathioprine was mainly described in CVID+PGD, and only in few cases of CVID+EGD.

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“…De acordo com a literatura, 2 a 13% dos pacientes com ICV apresentam DII associada, tendo como principais manifestações clínicas diarreia crônica, má absorção intestinal e perda de peso, sintomas apresentados pelo paciente deste relato. Além disso, o diagnóstico de DC geralmente é realizado anos após o diagnóstico de ICV, o que foi observado neste caso e em oito dos 11 artigos revisados [9]. Entre os 11 pacientes da literatura que apresentavam ICV e DC, 10 eram igualmente do sexo masculino, sendo necessários estudos adicionais que busquem a justificativa para essa distribuição epidemiológica.…”

Section: /7unclassified

“…Quanto ao tratamento da DC, somente dois dos 11 pacientes da literatura tiveram melhora dos sintomas gastrointestinais com uso isolado de corticoide sistêmico, sendo a prednisona o fármaco de escolha [13,14]. Um paciente faleceu antes do início do tratamento [15] e os outros oito pacientes só obtiveram melhora clínica após associações diversas de corticoide, aminossalicilatos (sulfassalazina ou mesalazina) [12], antineoplásico (6-mercaptopurina) [11], imunomoduladores (azatioprina) [16] e anti-TNF alfa (infliximabe ou adalimumabe) [3,9,17], sendo que a associação de infliximabe e azatioprina foi benéfica em dois de oito casos, sem necessidade de uso concomitante de corticoide sistêmico [4,18].…”

Section: /7unclassified

“…É rara a associação entre ICV e DC; na literatura estima-se que 2-13% dos pacientes com ICV apresentam DII associada, sendo o diagnóstico de DC geralmente realizado anos após o diagnóstico de ICV [9]. A abordagem da associação dessas afecções também é justificada pelo fato de que o quadro clínico e os exames complementares de ICV e de DC apresentam semelhanças consideráveis entre si e com muitas doenças infecciosas e autoimunes, dificultando um diagnóstico preciso e postergando um tratamento específico.…”

Section: Introductionunclassified

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Imunodeficiência comum variável associada à doença de Crohn-like: relato de caso e revisão da literatura

Calamita

2019

Sci Med

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Este artigo está licenciado sob forma de uma licença Creative Commons Atribuição 4.0 Internacional, que permite uso irrestrito, distribuição e reprodução em qualquer meio, desde que a publicação original seja corretamente citada. Como citar este artigo (How to cite this article):de Castro LCT, Calamita Z. Imunodeficiência comum variável associada à doença de Crohn-like: relato de caso e revisão da literatura (Common variable immunodeficiency associated with Crohn-like disease: case report and literature review). Sci Med. 2019;29(1):e32159. https:// RESUMO OBJETIVOS: relatar o caso de um paciente diagnosticado com imunodeficiência comum variável e doença de Crohn-like, descrevendo o quadro clínico, o processo de investigação diagnóstica, as abordagens terapêuticas e a evolução clínica do paciente. Realizar revisão bibliográfica de relatos de caso que abordem pacientes com a associação imunodeficiência comum variável e doença de Crohn-like. Enfatizar a importância do diagnóstico e tratamento precoces. DESCRIÇÃO DO CASO: Um paciente masculino foi diagnosticado com imunodeficiência comum variável aos nove anos de idade e doença inflamatória intestinal inespecífica aos 10 anos, após realização de colonoscopia e biópsia colônica indicando moderado infiltrado linfoplasmocitário, eosinofílico e alguns neutrófilos em lâmina própria, com ausência de granulomas. Aos 14 anos, foi confirmado o diagnóstico de doença de Crohn-like por especialista após correlação entre história clínica e exames complementares. Atualmente com 18 anos, o paciente está em tratamento com imunoglobulina humana endovenosa, infliximabe e azatioprina, com estabilização do quadro clínico. CONCLUSÕES: A revisão da literatura identificou 11 relatos de caso sobre a associação imunodeficiência comum variável e doença de Crohnlike, inexistindo trabalhos brasileiros, o que ressalta a raridade de tal associação. Neste relato, o paciente recebeu tratamentos amplos e empíricos devido à dificuldade em se chegar a um diagnóstico específico, o qual somente foi realizado aos 14 anos, quando se iniciou tratamento mais direcionado e individualizado. Apesar de atualmente estável, o paciente apresentou diversas complicações durante a investigação diagnóstica, o que ressalta a importância de diagnóstico precoce e tratamento mais preciso e dirigido às necessidades de saúde desses pacientes. DESCRITORES: imunodeficiência de variável comum; doença de Crohn; imunidade; doenças do sistema imune; doença inflamatória intestinal.

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Immmunodeficiencies and autoimmune diseases: Common variable immunodeficiency and Crohn-like

Cited by 5 publications

References 9 publications

Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults

Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults

What Works When Treating Granulomatous Disease in Genetically Undefined CVID? A Systematic Review

Imunodeficiência comum variável associada à doença de Crohn-like: relato de caso e revisão da literatura

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