Autoimmunity in patients with selective IgA deficiency.

BACKGROUND AND OBJECTIVE: Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deticiency. Patients with SIgAD have a greater risk of concomitant autoimmune disorders than healthy individuals. The exact mechanism underlying the relationship between autoimmunity and SIgAD is not fully understood. The aim of this study was to evaluate potential associations between autoimmunity and specific clinical or immunological findings in patients with SIgAD. METHODS: The study population comprised 57 symptomatic patients (65% males) with confirmed SIgAD who were referred to our center. Demographic data and history of autoimmunity were recorded both for patients and for their relatives. Comprehensive clinical and laboratory examinations were performed to investigate autoimmune complications in all the patients. RESULTS: Autoimmune disorders were documented in 17 cases (29.8%; 9 males and 8 females). The most common manifestations were thyroiditis, vitiligo, and hemolytic anemia (3 cases each). Ten patients (17.5%) had a family history of autoimmunity. Significant associations were detected between autoimmunity and increased duration of follow-up (P = .003), serum level of IgM (P = .01), regulatory T-cell count (P = .03), and class-switched memory B-cell count (P = .01). Four cases of autoimmune SIgAD (23.5%) progressed to common variable immunodeficiency during the follow-up period (P = .006). CONCLUSIONS: Autoimmune disorders, autoimmune cytopenia, and Ig subclass deficiency can lead to severe clinical manifestations in patients with SIgAD. Therefore, immunologists and pediatricians should be aware of these conditions.

Primary Antibody Deficiency in a Tertiary Referral Hospital: A 30-Year Experiment.

BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. OBJECTIVES: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. MATERIALS AND METHODS: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children's Medical Center, Tehran, Iran. RESULTS: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. CONCLUSIONS: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively.

Nasopharyngeal Carriage, Antibiotic Resistance and Serotype Distribution of Streptococcus Pneumoniae among Healthy Adolescents in Zahedan.

BACKGROUND: Colonization of nasopharynx by Streptococcus pneumoniae can lead to pneumococcal diseases. This study was performed to determine the carriage rate of nasopharyngeal S. pneumoniae in adolescents, antibiotic susceptibility and serotype prevalence in Zahedan, Iran. METHODS: Nasopharyngeal specimens from 865 adolescents (age range: 10-19 years old) attending eight schools in Zahedan, Iran, were collected and assessed by standard procedures to recover S. pneumoniae. The serotyping was carried out by latex agglutination test and the minimum inhibitory concentrations (MIC) of penicillin as well as other commonly used antibiotics were determined by a broth dilution method. RESULTS: Pneumococci were recovered from 15.7% (136/865, 95% confidence interval (CI) 12.3-18.9) of total samples which 119 isolates were typable with the available antisera. 1, 19A, 15C, 9V, 11A and 19F were found as the most frequent serotypes. Ninety three pneumococcal isolates were sensitive to penicillin. The MIC values of antibiotics tested were (µg/ml): penicillin 0.01-4, cefotaxime 0.01-4, ceftriaxone 0.02-128, chloramphenicol 0.08-32, ciprofloxacin 0.06-16, erythromycin 0.01-128, tetracycline 0.08-128 and vancomycin 0.02-1. CONCLUSION: A clear diversity was seen in the serotype distribution of the S. pneumoniae isolates and most of the antibiotic resistant strains belonged to few serotypes. Healthy adolescents in Zahedan, Iran commonly show pneumococcal carriage and antibiotic resistance.

Major histocompatibility complex class I and II antigens frequencies in Baloch ethnic group living in the southeast region of Iran.

BACKGROUND: Human leukocyte antigens have become key components to investigate the genetic relationships between populations. OBJECTIVE: The aim of this study was to determine the genetic diversity of HLA class I and II alleles among the Baloch ethnic group of southeastern in comparison with the Parsi ethnic group, thereby establishing a database for further investigations on the ancestry and genetic factors contributing to complex diseases in this region. MATERIALS AND METHODS: Thirty unrelated Balochi individuals from southeast Iran and 108 Parsi unrelated individuals were serologically typed using standard microcytotoxicity assays with commercial and local trays. RESULTS: The most frequent class I alleles of the Baloch ethnic group were HLA-A01 (53.3%), -A02 (30%), -A09 (23.3%), -A11 (20%), -A23 (23.3%), -B05 (53.3%), and -B08 (26.7%). The class II alleles more frequently observed were HLA-DR1 (26.7%), -DR2 (43.3%), -DR3 (20%), -DR4 (33.3%), -DR7 (26.7%), -DR11 (33.7%), -DRw52 (83.3%), -DRw53 (36.7%), -DQ1 (46.7%), -DQ2 (20%), and -DQ3 (40%). In contrast with the Parsi ethnic group, the frequencies for the following alleles were significantly higher in Baloch than Parsi. HLA-A01, -A23, -A25, -B05, -B08, -B16, -B17, -B60, -DR14, -DQ4, and -DQ7. In contrast, the frequencies of HLA-Cw4 and -DQ3 alleles in the Parsi were significantly higher than the Baloch ethnic group. CONCLUSIONS: This first study of HLA in widely dispersed areas of Iran represents an important resource for investigators in the fields of transplantation immunology and population genetics.

The study of type 1 allergy prevalence among people of South-East of iran by skin prick test using common allergens.

The prevalence of allergic disorders has increased markedly throughout the world over the past three decades. It seems probable that the increased prevalence is real because longitudinal studies have shown a rapid exponential increase in allergic disorders. In order to determine the prevalence of type 1 allergy in patients with allergic disorders in Sistan-Blouchestan province, in the Southeast of Iran, patients referred to Immunology and Allergy Medical Center of Khatam Hospital during a 7-year period were studied. One thousand two hundred and eighty-six patients (554 males and 732 females), aged 2-79 years, had allergic disorders. The most frequent allergic disease was rhinitis, seen in 959 patients ( 74.57%). Also, 939 patients had a history of sinusitis (73.02%). Among the skin prick test(SPT)-positive patients, a positive prick test reaction to the house dust mites (89.74%), feathers (70.29%), and Aspergillus (65.08%) was most common, followed by Alternaria (57.39%), Cladosporium (47.12%), grasses (43.39%), trees (41.29%), Penicillium (39.19%), fruits (38.41%), and weeds (32.50%). Also, 30.33% of the subjects were sensitized to Hen's egg in the prick test, walnut (29.16%), cow's milk (21.46%), beef meat (19.21%), and hazel-nut (15.32%) accounting for other positive reactions. The prevalence of SPT-positive to common allergens is high among Iranian patients with allergic disorders. So, it should be recognized in the diagnosis of allergic diseases as well as in allergen-reduction programs.

CD4 T cells in the rheumatoid joint are oligoclonally activated and change during the course of disease.

OBJECTIVE: To assess the nature of T cell receptor (TCR) utilisation by CD4 T cells in the rheumatoid joint. METHODS: Sequencing of the joining (NDJ) region of TCR beta chain mRNA isolated from synovial fluid CD4 T cells was performed in three patients in order to determine if oligoclonal expansion of particular sequences was present. Two patients were studied longitudinally to determine if these sequences changed over time. RESULTS: A number of dominant clonotypes were found within the TCR transcripts sequenced in each patient. In the two patients who were studied longitudinally, different dominant clonotypes were detected over time. No single clonotype was persistently dominant during the period of study. CONCLUSIONS: The pattern of TCR usage showed multiple oligoclonally expanded CD4 T cells within the rheumatoid joint. The change in clonotypes within the joint over time suggests that different antigens may be able to elicit synovial inflammation during the course of rheumatoid disease.