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The B lymphocyte developmental blocks agammaglobulinemia, leading to peripheral B cell depletion and plasma immunoglobulin reduction. Agammaglobulinemia is a rare yet severe disease since it is presented with recurrent sinopulmonary and skin, central nervous system, bone, and joint infections. The onset of the disease is reported to be at the age of six months. Associations have been reported between arthritis and immunodeficiency disorders, such as agammaglobulinemia and common variable immunodeficiency (CVID). This study aimed to present the case of a 3.5-year-old female with a three-month history of the swelling of the left knee, mimicking oligoarticular juvenile idiopathic arthritis. After the initiation of immunosuppressive treatment, the patient developed large, tender, erythematous lesions on the inguinal region bilaterally, which developed to ecthyma gangrenosum due to Pseudomonas. The patient's mother also reported recurrent episodes of infections since the patient was a one-year-old infant. Subsequent to the immunological examinations and laboratory tests, the patient was diagnosed with autosomal recessive agammaglobulinemia due to low serum immunoglobulin concentration and the absence of peripheral B cells. Primary immunodeficiency conditions (particularly agammaglobulinemia) and CVID should be considered in children with arthritis and recurrent infections. Moreover, immunological analysis should be performed prior to treatment in these children. [ABSTRACT FROM AUTHOR]

Objective(s): Juvenile idiopathic arthritis (JIA) is one of the most common chronic rheumatic diseases in children. The complex nature of this immune-mediated disease owes itself to several predisposing genes and environmental factors affecting its pathogenesis. Conducted in Iran, this study was originally intended to investigate every possible association between HLA DRB1 alleles and a susceptibility to JIA. Materials and Methods: In this case-control study, 45 patients with a definite diagnosis of JIA based on International League against Rheumatism (ILAR) criteria were compared against 46 healthy controls. DNA samples taken from both groups were analyzed using PCR-sequence specific primers (PCR-SSP) method. Data analysis including parametric and nonparametric test and multivariate analysis was undertaken using the SPSS 11.5 software. A P-value< 0.05 was regarded as statistically significant. Results: Mean ages in case group and healthy controls were 14.64±6.21 and 13.73±6.39, respectively with no significant difference between the two groups (P=0.515). Sex difference between JIA group and healthy controls was also not significant (P=0.068) .The frequency of HLA-DRB1*01 was found the most frequent HLA-RB1 in our patients (33.3%). No significant statistical correlation between various HLA-DRB1 alleles and clinical subtypes of the disease could be established from the data. HLA-DRB1*11 was shown to raise protection to JIA (P=0.035, OR=2.755, 95% CI=0.963-8.055) in northeastern Iran. In addition, we found that HLA-RB1*09 is nominally associated with an increased risk of JIA (P=0.56, OR=2, 05, 95% CI=0.18-23.63). Conclusion: HLA-DRB1*11 was shown to raise protection to JIA in northeastern Iran. The disparity of findings in other ethnicities prompts further investigations with larger sample sizes. [ABSTRACT FROM AUTHOR]

Background: A common type of chronic arthritis in children and adolescents is juvenile idiopathic arthritis (JIA). According to the International League of Associations for Rheumatology (ILAR) classification, JIA diagnostic criteria include age under 16 years and disease duration of six-weeks. Based on the number of involved joints in the first sixmonths of disease onset, JIA is categorized into oligoarticular or polyarticular subtypes. Age is a characteristic factor in the diagnosis of disease subsets; it is worth mentioning that cases younger than six months of age are seldom found in any of the subtypes. Case report: In this report, we present a rare case of JIA in an infant, presenting at 20 days of age. Effusion of the right hip joint was one of the primary manifestations of the disease. During hospitalization, she went through sepsis workup and a four-week antibiotic therapy for management of lower limb pseudoparalysis. In spite of antibiotic therapy, she developed effusion of a second joint. According to the course and duration of symptoms and ILAR classification for JIA, oligoarticular JIA was diagnosed and treated. Conclusion: In this case, infectious diseases, such as tuberculosis and brucellosis, and malignancies were ruled out as a cause of inflammation through bone marrow aspiration, culture, and tests; ultrasound and magnetic resonance imaging showed no lytic and sclerotic lesions or a fracture. Our experience showed a rare case of JIA and suggested that JIA must be considered in children with joint inflammation at any age. [ABSTRACT FROM AUTHOR]

Background: Juvenile Rheumatoid Arthritis (JRA) is the most common chronic pediatric rheumatologic disease. There is a wide range of variation in disease subgroup distribution and evolution according to different populations, environments and genetic predisposition. This study was designed to evaluate epidemiologic characteristics of JRA in Iran. Methods: A cross-sectional studies was performed on children suffering from JRA according to American College of Rheumatology during 10 year (2002-2011). Patients' data was recorded in hospital questionnaires; Patients' age at the first presentation and time of diagnosis, sex, ethnicity, early clinical and constitutional presentations, joint involvement pattern and presence of associated diseases were evaluated. Results: In this study, 171 children (91 boys and 80 girls) were enrolled. The mean age of patients was 5.2±3.5 years at the time of presentation and 5.7±3.6 years when the diagnosis was confirmed. The most common type of disease was polyarticular arthritis (41.9%). The prevalence of pauciarticular and systemic onset subtype were 31.6% and 19.3%, respectively. According to ethnicity, the most common incidence was seen in Fars and Turk populations respectively. The most common constitutional presentation was fever. Fever and morning stiffness was significantly higher in polyarticular patients. Extra articular manifestation was seen in 40.4% patients. Among extra-articular manifestations, skin involvement was seen more than others. There was no correlation between sex and type of chronic arthritis. Uveitis was detected in 5.8% of our cases. The mean age was significantly higher in polyarticular group (6.04 year) in comparison to pauciarticular and systemic group (4.4 year in both groups) (P=0.005). There was no correlation between sex and type of chronic arthritis. Conclusion: The prevalence of JRA subgroups was different in Iran comparing with western countries and polyarticular disease was the most common subgroup in this study. Uveitis was less common in this study in comparison to studies in other countries. There is no sex predilection in each subgroup of JRA. [ABSTRACT FROM AUTHOR]