Recurrent AOM associated with selective IgA deficiency

2019 ◽  
Vol 11 (4) ◽  
pp. 181-182
Author(s):  
Silvia Muriño
Keyword(s):  
Autoimmune Diseases ◽  
Primary Immunodeficiency ◽  
Allergic Diseases ◽  
Iga Deficiency ◽  
Older Age ◽  
Selective Iga Deficiency

Selective IgA deficiency is the most common primary immunodeficiency. A small percentage presents pathology, but at older age can associate deficiency of some subclass of IgG and greater susceptibility to infections, allergic diseases, autoimmune diseases and neoplasms.

Autoimmune Inner Ear Disease among Patients with Selective IgA Deficiency

2020 ◽  
pp. 1-8
Author(s):  
Eli Magen ◽  
Idan Blum ◽  
Dan Andrey Waitman ◽  
Natan Kahan ◽  
Boaz Forer
Keyword(s):  
Autoimmune Diseases ◽  
Inner Ear ◽  
Acute Otitis Media ◽  
Allergic Diseases ◽  
Healthcare Services ◽  
Iga Deficiency ◽  
Control Group ◽  
Selective Iga Deficiency ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

<b><i>Objectives:</i></b> Autoimmune inner ear disease (AIED) is a distinct clinical entity from sudden sensorineural hearing loss. The purpose of this study was to investigate the clinical characteristics of AIED in patients with selective IgA deficiency (sIgAD). <b><i>Materials and Methods:</i></b> This retrospective observational study was based on data from the Leumit Healthcare Services database in Israel. We searched all subjects aged ≥12 years who had undergone serum total IgA measurements during 2004–2014 for any reason. The sIgAD patients included all subjects with serum IgA of ≤7 mg/dL (0.07 g/L). A control group was randomly sampled from the full study population (<i>n</i> ≈ 730,000) with a case-control ratio of 10 controls for each case (1:10). <b><i>Results:</i></b> Among 347 subjects with sIgAD, we identified 9 patients with concomitant AIED (sIgAD + AIED group). This group was characterized by a higher prevalence of allergic diseases (8 patients; 88.9%) than sIgAD patients without AEID (sIgAD + AIED group; 153 patients; 45.2%; <i>p</i> = 0.014). Both systemic diseases (3 patients; 33.3%) and organ-specific autoimmune diseases (7 patients; 77.8%) were more prevalent in the sIgAD + AIED group (sIgAD + AIED group: 19 patients 5.5%, <i>p</i> = 0.015; sIgAD − AEID group: 76 patients, 21.9%, <i>p</i> &#x3c; 0.001), with an OR of 8.39 (1.94–36.19; <i>p</i> = 0.004). sIgAD patients with and without AIED were characterized by a higher prevalence of documented episodes of acute otitis media, allergic diseases, and autoimmune diseases than the control group. <b><i>Conclusion:</i></b> The study exposes a significant association between AIED and sIgAD. We believe that sIgAD has to be excluded in AIED patients.

Profile of Autoantibodies Against Phosphorylcholine and Cross-reactivity to Oxidation-Specific Neoantigens in Selective IgA Deficiency With or Without Autoimmune Diseases

2010 ◽  
Vol 30 (6) ◽  
pp. 872-880 ◽  
Author(s):  
Ana Elisa Fusaro ◽  
Kristine Fahl ◽  
Elaine Cristina Cardoso ◽  
Cyro Alves de Brito ◽  
Cristina M. A. Jacob ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Iga Deficiency ◽  
Cross Reactivity ◽  
Selective Iga Deficiency

Selective IgA deficiency associated with three organspecific autoimmune diseases, anti-IgA antibodies and erythrocyte-binding IgA

1987 ◽  
Vol 10 (3) ◽  
pp. 309-317
Author(s):  
Yukinobu Ichikawa ◽  
Mitsuaki Uchiyama ◽  
Shigeru Arimori ◽  
Junichi Ogawa ◽  
Hiroshi Inoue ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Iga Antibodies ◽  
Erythrocyte Binding

scholarly journals POS1374 RHEUMATOLOGIC DISEASES AS PRESENTATIONS OF PRIMARY IMMUNODEFICIENCY DISEASES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 969.1-969
Author(s):  
S. Kudryashov ◽  
L. Karzakova ◽  
N. Zhuravleva ◽  
I. Sidorov ◽  
T. Lutkova
Keyword(s):  
Rheumatoid Arthritis ◽  
Primary Immunodeficiency ◽  
Clinical Picture ◽  
Iga Deficiency ◽  
Primary Immunodeficiency Diseases ◽  
Adequate Treatment ◽  
Selective Iga Deficiency ◽  
Immunodeficiency Diseases ◽  
Rheumatologic Diseases ◽  
Rheumatic Symptoms

Background:Primary immunodeficiency diseases (PID) occur on average with a frequency of 1: 100 000 of the population. In accordance with the classification of PIDS of the International Union of Immunological Societies (IUIS) 9 different groups of innate errors of immunity are distinguished [1]. The polymorphism of the clinical picture, difficulties in recognizing PID cause their late diagnosis and the associated development of irreversible organ damage, complications and high mortality. According to the traditional view, the clinical marker of PID is an infectious syndrome. However, in some cases, PID is detected as a rheumatologic disease.Objectives:The aim of the study is to analyze the frequency of rheumatologic diseases in adult patients with PID living in one of the subjects of Russia –Chuvashia.Methods:The material of the study was patient data obtained during a retrospective analysis of 49 outpatient records included in the Republican Register of PID. Diagnosis of various forms of PIDS was carried out in accordance with the criteria developed by the IUIS [1].Results:During the period from 1993 to January 2020, 49 cases of PID were registered in the adult population of Chuvashia. According to the frequency of PID, сommon variable immunodeficiency (CVID) is the most common in Chuvashia (26 people). In the second place there is selective IgA deficiency (10 people); in third place there are X-linked agammaglobulinemia (4 people) and hereditary angioedema (4 people). The remaining forms of PID account for 5 cases: 2 cases of Louis-Bar syndrome, 1 case of DiGeorge syndrome, 1 case of Wiskott-Aldrich syndrome and 1 case of Hyper IgE syndrome. The main symptom of PID in 35 (71.4%) patients was heightened susceptibility to infection. In 14 (28.6%) patients, the clinical picture was dominated by non-infectious presentations (autoimmune, lymphoproliferative and oncological diseases). In 9 patients (18.3%), it was manifested by rheumatologic diseases (rheumatoid arthritis, psoriatic arthritis, scleroderma-like syndrome). These symptoms were more characteristic of CVID. With selective IgA deficiency and X-linked agammaglobulinemia, rheumatic symptoms were observed only in isolated cases. CVID debuted in 2 cases as rheumatoid arthritis, resulting in the delay in the diagnosis of an average of 5.2 years. Only the detailed immunological and genetic study made it possible to diagnose PID and prescribe adequate treatment – replacement immunoglobulin therapy. This treatment reduced the frequency of infectious manifestations of the disease and it did not significantly improve the course of rheumatological diseases. Therefore, the use of methotrexate and targeted therapy was continued.Conclusion:In the clinical picture of PID, rheumatic symptoms predominate in 28.6% of cases, which requires a thorough immunological and genetic examination of patients with rheumatic diseases in order to diagnose PID in a timely manner.References:[1]Picard C, Bobby Gaspar H, Al-Herz W, et al. J Clin Immunol. 2018;38(1):96-128.Disclosure of Interests:None declared

scholarly journals Retrospective Analysis of Autoimmune Diseases and Immunologic Characteristics of the Adult Primary Immune Deficiency Cohort: 17 Years Experience of the Tertiary Referral Immunology Center in Turkey

2021 ◽  
Vol 19 (1) ◽  
pp. 12-23
Author(s):  
Ceyda TUNAKAN DALGIÇ ◽  
Aytül Zerrin Sin ◽  
Fatma Ömür Ardeniz
Keyword(s):  
Immune Deficiency ◽  
Autoimmune Diseases ◽  
Iga Deficiency ◽  
Cross Sectional Study ◽  
Dyskeratosis Congenita ◽  
Primary Immune Deficiency ◽  
Laboratory Findings ◽  
Cross Sectional ◽  
Selective Iga Deficiency ◽  
Significant Difference

ABSTRACT Objective: Primary immunodeficiencies (PIDs) consist of genetically heterogeneous disorders. The spectrum can include infectious diseases, malignancy, allergy, and autoimmunity. We aimed to analyze the frequency and variety of autoimmune diseases (ADs) in PIDs and describe their clinical and laboratory features. Materials and Methods: Ninety-two patients with PID followed by Ege University Medical Faculty between 2000 and 2017 were enrolled in this retrospective, cross-sectional study. All patients’ medical records were reviewed using the demographic information, type of PIDs and ADs, ADs-related autoantibodies, and basic and immunologic laboratory findings. ADs were diagnosed using clinical and complementary paraclinical findings by an immunologist and/or a subspecialist related to the affected organ or system. Results: We evaluated 50 male and 42 female PID patients with a mean age of 40.92 (18-86). Twenty-nine (32 %) patients (15 females/14 males) with a mean age of 43.8 (19-78) had ADs. In our study group, the most commonly detected type of PID with AD is common variable immune deficiency (CVID) (n=17); followed by combined immune deficiency (CID) (n=3), CTLA4 deficiency (n=2), selective IgA deficiency (sIgAD) (n=2), specific IgG subgroup deficiency (n=1), autoimmune polyglandular syndrome (APS) with hypogammaglobulinemia (n=1), dyskeratosis congenita (DC) (n=1), Osler-Rendu-Weber (ORW) syndrome with CVID-like PID (n=1), and cartilage-hair hypoplasia (CHH) (n=1). According to systematic assessments, ADs resulted in endocrinologic 14%, dermatologic 10.8%, rheumatologic 9.7%, gastroenterological 9.7%, hematological 8.6%, and neurologic disorders 1%. The frequency of ADs was higher in CVID cases than other types of PIDs (p <0.05). Basic and immunologic laboratory findings of the PIDs with and without ADs were analyzed and compared; however, no statical significant difference was obtained between the groups. Conclusion: We have analyzed the frequency and variety of ADs in a adult PID cohort in Turkey. Patients presenting with multiple ADs should be screened for having an underlying PID. Keywords: Primary immune deficiency, autoimmunity, autoantibody, immunologic parameters, frequency

scholarly journals Selective IgA Deficiency in Autoimmune Diseases

2011 ◽  
Vol 17 (11-12) ◽  
pp. 1383-1396 ◽  
Author(s):  
Ning Wang ◽  
Nan Shen ◽  
Timothy J. Vyse ◽  
Vidya Anand ◽  
Iva Gunnarson ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Iga Deficiency ◽  
Selective Iga Deficiency
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