A High Prevalence of Gastrointestinal Manifestations in Common Variable Immunodeficiency

Pikkarainen, Sampsa; Martelius, Timi; Ristimäki, Ari; Siitonen, Sanna; Seppänen, Mikko; Färkkilä, Martti

doi:10.14309/ajg.0000000000000140

Cited by 62 publications

(53 citation statements)

References 23 publications

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“…CrA, instead, seems to have little usefulness in this setting. The presence of additional risk factors, including family history of gastric cancer and common variable immunodeficiency, prompt endoscopic surveillance every 1‐2 years. In all cases, high‐definition endoscopy with chromoendoscopy should be offered, as it may increase the probability to detect small lesions.…”

Section: Discussionmentioning

confidence: 99%

Natural history of autoimmune atrophic gastritis: a prospective, single centre, long‐term experience

Miceli

Vanoli

Lenti

et al. 2019

Aliment Pharmacol Ther

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Summary Background Autoimmune atrophic gastritis (AAG) is an immune‐mediated disorder characterised by destruction of gastric oxyntic mucosa Aim To explore gastric histopathological evolution in a cohort of AAG patients over a prolonged follow‐up Methods Single centre prospective study enrolling consecutive patients with histologically confirmed AAG between 2000 and 2018. All AAG patients undergoing endoscopic follow‐up every 1‐3 years were classified as having stages 1, 2 or 3 according to atrophy severity (mild, moderate and severe). AAG patients with either glandular or neuroendocrine dysplasia/neoplasia were classified as having stage 4. Disease stage progression, and changes in serum anti‐parietal cell antibody (PCA), chromogranin A and gastrin‐17 were assessed. Results In total, 282 AAG patients (mean age 60.3 years; F:M ratio 2.4:1; median follow‐up 3 years, interquartile range 1‐7) were enrolled. All patients with stages 1 or 2 progressed to stage 2 or 3 over time with a steady trend (P = .243) and regression from a severe to a milder stage was never noticed. Disease progression of patients with stages 1 or 2 occurred within the first 3 years. PCA positivity rate did not change over time. Stage 3 patients had higher gastrin‐17 levels compared to patients with stages 1 and 2 (median 606 vs 295 pg/mL; P < .001). In stage 3, the hazard ratio for the risk of developing stage 4 was 6.6 (95% CI 1.5‐29; P = .001). Conclusions AAG is a steadily progressive disease, in which stages 1 and 2 always progress to stage 3. The risk of developing a complicated disease stage is greater in patients with more severe gastric lesions.

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Section: Discussionmentioning

confidence: 99%

Natural history of autoimmune atrophic gastritis: a prospective, single centre, long‐term experience

Miceli

Vanoli

Lenti

et al. 2019

Aliment Pharmacol Ther

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“…Among organ specific autoimmune manifestations, hypothyroidism was the most prevalent at 3.5%, followed by alopecia areata and vitiligo at 2.7%, and type 1 diabetes at 1.6% in an ESID registry of 2700 CVID patients (9). Autoimmunity may also underlie gastrointestinal complications of CVID, including inflammatory bowel disease, autoimmune enteropathy, and autoimmune gastritis (33).…”

Section: Overview Of Autoimmune Disease In Common Variable Immunodefimentioning

confidence: 99%

Current Understanding and Recent Developments in Common Variable Immunodeficiency Associated Autoimmunity

Gereige

Maglione

2019

Front. Immunol.

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Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency and comprises a group of disorders with similar antibody deficiency but a myriad of different etiologies, most of which remain undefined. The variable aspect of CVID refers to the approximately half of patients who develop non-infectious complications in addition to heightened susceptibility to infection. The pathogenesis of these complications is poorly understood and somewhat counterintuitive because these patients that are defined by their immune futility simultaneously have elevated propensity for autoimmune disease. There are numerous aspects of immune dysregulation associated with autoimmunity in CVID that have only begun to be studied. These findings include elevations of T helper type 1 and follicular helper T cells and B cells expressing low levels of CD21 as well as reciprocal decreases in regulatory T cells and isotype-switched memory B cells. Recently, advances in genomics have furthered our understanding of the fundamental biology underlying autoimmunity in CVID and led to precision therapeutic approaches. However, these genetic etiologies are also associated with clinical heterogeneity and incomplete penetrance, highlighting the fact that continued research efforts remain necessary to optimize treatment. Additional factors, such as commensal microbial dysbiosis, remain to be better elucidated. Thus, while recent advances in our understanding of CVID-associated autoimmunity have been exciting and substantial, these current scientific advances must now serve as building blocks for the next stages of discovery.

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“…Gastrointestinal manifestations, such as autoimmune gastritis, celiac disease, chronic diarrhea, malabsorption, nodular lymphatic hyperplasia, etc., may affect patients with primary antibody deficiencies [124]. We recommend assessing specifically gastrointestinal symptoms in all patients with antibody deficiency.…”

Section: Gastrointestinal Manifestationsmentioning

confidence: 99%

Treatment and management of primary antibody deficiency: German interdisciplinary evidence‐based consensus guideline

et al. 2020

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This evidence‐based clinical guideline provides consensus‐recommendations for the treatment and care of patients with primary antibody deficiencies (PADs). The guideline group comprised 20 clinical and scientific expert associations of the German, Swiss, and Austrian healthcare system and representatives of patients. Recommendations were based on results of a systematic literature search, data extraction, and evaluation of methodology and study quality in combination with the clinical expertise of the respective representatives. Consensus‐based recommendations were determined via nominal group technique. PADs are the largest clinically relevant group of primary immunodeficiencies. Most patients with PADs present with increased susceptibility to infections, however immune dysregulation, autoimmunity, and cancer affect a significant number of patients and may precede infections. This guideline therefore covers interdisciplinary clinical and therapeutic aspects of infectious (e.g., antibiotic prophylaxis, management of bronchiectasis) and non‐infectious manifestations (e.g., management of granulomatous disease, immune cytopenia). PADs are grouped into disease entities with definitive, probable, possible, or unlikely benefit of IgG‐replacement therapy. Summary and consensus‐recommendations are provided for treatment indication, dosing, routes of administration, and adverse events of IgG‐replacement therapy. Special aspects of concomitant impaired T‐cell function are highlighted as well as clinical data on selected monogenetic inborn errors of immunity formerly classified into PADs (APDS, CTLA‐4‐, and LRBA‐deficiency).

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A High Prevalence of Gastrointestinal Manifestations in Common Variable Immunodeficiency

Cited by 62 publications

References 23 publications

Natural history of autoimmune atrophic gastritis: a prospective, single centre, long‐term experience

Natural history of autoimmune atrophic gastritis: a prospective, single centre, long‐term experience

Current Understanding and Recent Developments in Common Variable Immunodeficiency Associated Autoimmunity

Treatment and management of primary antibody deficiency: German interdisciplinary evidence‐based consensus guideline

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