Autoimmunity in Primary Antibody Deficiencies

Azizi, Gholamreza; Ahmadi, Moslem; Abolhassani, Hassan; Yazdani, Reza; Mohammadi, Hamed; Mirshafiey, Abbas; Rezaei, Nima; Aghamohammadi, Asghar

doi:10.1159/000453263

Cited by 45 publications

(36 citation statements)

References 120 publications

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“…Autoimmunity has been reported to be more frequent among patients with CVID . The CVID typically can present in childhood and early adulthood, but the likelihood of autoimmune diseases in patients with CVID increases by age .…”

Section: Discussionmentioning

confidence: 99%

Rheumatologic complications in a cohort of 227 patients with common variable immunodeficiency

et al. 2018

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Common variable immunodeficiency (CVID) is the most prevalent symptomatic type of human primary immunodeficiency diseases (PID). Clinically, CVID is characterized by increased susceptibility to infections and a wide variety of autoimmune and rheumatologic disorders. All patients with CVID registered in Iranian PID Registry (IPIDR) were enrolled in this retrospective cohort study. We investigated the frequency of rheumatologic diseases and its association with immunological and clinical phenotypes in patients with CVID. A total of 227 patients with CVID were enrolled in this study. The prevalence of rheumatologic disorders was 10.1% with a higher frequency in women than men. Most common rheumatologic manifestations were juvenile idiopathic arthritis (JIA) and adult rheumatoid arthritis (RA) followed by juvenile spondyloarthritis (JSpA) and undifferentiated inflammatory arthritis (UIA). Septic arthritis in patients with CVID with a history of RA and JIA was higher than patients without rheumatologic complication. Patients with CVID with a history of autoimmunity (both rheumatologic and non-rheumatologic autoimmunity) had lower regulatory T cells counts in comparison with patients without autoimmune disorders. There was an association between defect in specific antibody responses and negative serologic test results in patients with rheumatologic manifestations. JIA, RA, JSpA and UIA are the most frequent rheumatologic disorders in patients with CVID. Due to antibody deficiency, serologic tests may be negative in these patients. Therefore, these conditions pose significant diagnostic and therapeutic challenges for immunologists and rheumatologists in charge of the care for these patients.

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Section: Discussionmentioning

confidence: 99%

Rheumatologic complications in a cohort of 227 patients with common variable immunodeficiency

et al. 2018

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“…Autoimmunity refers to a pathologic condition, in which the immune system recognizes self‐antigens and tissues, which results in an immune response against an individual's own cells and tissues by different immune constituents, such as autoreactive antibodies and T cells (Azizi, Ahmadi, et al, ; Azizi, Hafezi, et al, ; Azizi, Ziaee, et al, ; Salmaninejad et al, ; Singh et al, ). Modifications in miRNA regulation appear to be highly associated with the progression of immune dysfunctions and autoimmunity.…”

Section: Biogenesis Of Mirnasmentioning

confidence: 99%

MicroRNA implications in the etiopathogenesis of ankylosing spondylitis

Mohammadi

Hemmatzadeh

Babaie

et al. 2018

Journal Cellular Physiology

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Ankylosing spondylitis (AS) is a chronic immune-mediated inflammatory disease that affects both axial and peripheral skeletons as well as soft tissues. Recent investigations offer that disease pathogenesis is ascribed to a complex interplay of genetic, environmental, and immunological factors. Until now, there is no appropriate method for early diagnosis of AS and the successful available therapy for AS patients stay largely undefined. MicroRNAs (miRNAs), endogenous small noncoding RNAs controlling the functions of target mRNAs and cellular processes, are present in human plasma in a stable form and have appeared as possible biomarkers for activity, pathogenesis, and prognosis of the disease. In the present review, we have tried to summarize the recent findings related to miRNAs in AS development and discuss the possible utilization of these molecules as prognostic biomarkers or important therapeutic strategies for AS. Further examinations are needed to determine the unique miRNAs signatures in AS and characterize the mechanisms mediated by miRNAs in the pathology of this disease.

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“…Several studies have reported that abnormalities in T helper (Th) subsets, primarily Th1, Th17, and Th22 cells, and regulatory T (Treg) cells are associated with organ‐specific autoimmunity and inflammatory bowel disease (IBD; Azizi, Yazdani, & Mirshafiey, ; Globig et al, ; Leung et al, ). Moreover, an inappropriate balance between proinflammatory (e.g., interferon‐γ [IFN‐γ], interleukin [IL]‐17, and IL‐22) and anti‐inflammatory cytokines (e.g., IL‐10) could result in the dysregulation of immune homeostasis, and autoimmune and inflammatory diseases (Azizi, Ahmadi, et al, ; Azizi, Rezari, et al, ). Although the abnormality of CD4 + T cell has been reported in patients with LRBA deficiency (Alangari et al, ; Alkhairy et al, ; Charbonnier et al, ; Gamez‐Diaz et al, ; Revel‐Vilk et al, ), no study has evaluated frequencies and functional statuses of Th subsets in these patients.…”

Section: Introductionmentioning

confidence: 99%

“…[IL]-17, and IL-22) and anti-inflammatory cytokines (e.g., IL-10) could result in the dysregulation of immune homeostasis, and autoimmune and inflammatory diseases (Azizi, Ahmadi, et al, 2016;Azizi, Rezari, et al, 2016). Although the abnormality of CD4 + T cell has been reported in patients with LRBA deficiency (Alangari et al, 2012;Alkhairy et al, 2016;Charbonnier et al, 2015;Gamez-Diaz et al, 2016;Revel-Vilk et al, 2015), no study has evaluated frequencies and functional statuses of Th subsets in these patients.…”

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confidence: 99%

The imbalance of circulating T helper subsets and regulatory T cells in patients with LRBA deficiency: Correlation with disease severity

Azizi

Mirshafiey

Abolhassani

et al. 2018

Journal Cellular Physiology

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Patients with lipopolysaccharides responsive beige-like anchor protein (LRBA) deficiency suffer from a variety of immunological abnormalities. In the current study, we investigated the role of T helper (Th) cell subsets and regulatory T (Treg) cells and their related cytokines and transcription factors in the immune dysregulation of LRBA deficiency. The study population comprised of 13 LRBA-deficient patients and 13 age- and sex-matched healthy controls (HCs). Th subsets and Treg were examined by flow cytometry. The expression of determinant cytokines (interferon-γ [IFN-γ], interleukin [IL]-17, IL-22, and IL-10), and cell subset-specific transcription factors were evaluated before and after proliferation and activation stimuli. The frequencies of Th1, Th1-like Th17 and Th22 cells along with the expression of T-box transcription factor (TBET) and runt-related transcription factor 1 (RUNX1) were significantly increased in patients with LRBA. Moreover, IFN-γ and IL-22 production in LRBA-deﬁcient CD4 T cells were elevated after lymphocyte stimulation, particularly in patients with enteropathy. However, CD4 CD25 FoxP3 CD127 cells were significantly decreased in LRBA-deficient patients compared with those of HCs, particularly in patients with autoimmunity. There was a negative correlation between the frequencies of CD4 CD25 FoxP3 CD127 cells and Th1-like Th17 cells in LRBA-deficient patients, and an overlapping phenotype of autoimmunity and enteropathy were observed in ~70% of patients. The frequency of Th17 cells was lower in patients with enteropathy, while Th1-like Th17 cells were higher than in those without enteropathy. Our findings demonstrated an imbalance in Th subsets, mainly in Th1-like Th17 and Treg cells and their corresponding cytokines in LRBA deficiency, which might be important in the immunopathogenesis of autoimmunity and enteropathy.

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Autoimmunity in Primary Antibody Deficiencies

Cited by 45 publications

References 120 publications

Rheumatologic complications in a cohort of 227 patients with common variable immunodeficiency

Rheumatologic complications in a cohort of 227 patients with common variable immunodeficiency

MicroRNA implications in the etiopathogenesis of ankylosing spondylitis

The imbalance of circulating T helper subsets and regulatory T cells in patients with LRBA deficiency: Correlation with disease severity

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