At present, a national consensus or guideline for diagnosing and managing patients suspected of having predominantly antibody deficiencies (PADs) is lacking. This consensus is written based on a combination of scientific literature and comments from the expert panel of Iranian immunologists. A group of clinical immunologists reviewed the current consensus, presented their comments at a meeting titled 'First Meeting on the Diagnosis of Inborn Errors of Immunity (IEI) by IEI Experts,' and agreed on this consensus. This consensus guideline provides recommendations on the diagnosis, antimicrobial prophylaxis, management of clinical manifestations, and immunoglobulin replacement therapy (IgRT) for patients with PAD.

Background: Asthma and Allergic rhinitis (AR) are frequently concurrent diseases. Epidemiological,
immunological, and clinical studies reported increasing evidence of the links between AR and asthma.
Epidemiologically, up to 40% of patients with AR also have asthma, and up to 80% of patients with asthma
experience nasal symptoms. Furthermore, patients with AR are at three times the risk of developing asthma
compared with those without AR.

Method: This cross-sectional study was carried out during one year from all patients between 0-16 years
who were referred to allergy and ENT clinics with symptoms of chronic cough (more than four weeks) or
upper respiratory tract symptoms such as sneezing, runny nose, and nasal congestion. Data and diagnosis
were classified according to the Global Initiative for Asthma (GINA) and the AR criteria and its Impact on
Asthma guidelines (ARIA), respectively.

Results: 190 (120 individuals with asthma and 70 individuals with AR) were enrolled in the study. The mean
age of patients with asthma was 9.14±3.13, and 43.3% were female. The severity of asthma was classified as
mild (20%), moderate (70.8%), and severe (9.2%). The mean age of patients with rhinitis was 10.13±3.45 years,
and 35.7% were female. Rhinitis was classified as intermittent (40%) and persistent (60%). The prevalence of
AR in the asthma group was 42 individuals (35%). Twenty-nine cases of them (24.2%) had intermittent AR,
and 13 (10.8%) cases had persistent AR. The prevalence of asthma in AR was 18 cases (25.7%) 3 cases (4.3%)
had mild asthma, 12 cases (17/1%) had moderate asthma, and 3 cases (4.3%) had severe asthma. A significant
correlation was found ( P<0.0001) between the severity of AR and asthma.

Conclusion: Based on the findings of our study, there is a significant relationship between the severity of
asthma and AR in patients with asthma and AR. The results show that asthma prevalence is high in patients
with AR. Also, the frequency of AR in patients with asthma is significantly higher.

Background: Systemic juvenile idiopathic arthritis (sJIA) is determined by prominent multisystemic involvements with onset before the age of 16 years of which IL-6 has a significant role in inflammatory process. Tocilizumab (TCZ) is a human anti-IL-6 receptor monoclonal antibody, which modulates IL-6 activity by blocking its binding to the soluble and membrane-bound IL-6 receptor. No comprehensive study has been done regarding the use of IL-6 inhibitor in the treatment of SJIA patients in Iran. This study was conducted with the aim of investigating the effectiveness and outcome of IL-6 inhibitor treatment in Iranian children with sJIA.
Methods and Materials: This is a case-control study including twenty patients diagnosed with sJIA divided equally in two groups of case and control who received TCZ and corticosteroids respectively. Subsequently, various clinical and laboratory features were compared amongst these two groups.
Results: A significant difference before and after TCZ has been seen in terms of height increase, weight gain, and decrease in leukocytosis, thrombocytosis and ESR, CRP negativity and arthritis improvement.
Conclusions: Considering the favorable effects of TCZ in the control of sJIA, by producing this drug in the country and making it available to patients at a lower cost, it is possible to better control the disease and reduce the harmful effects caused by the disease and reduce the long-term use of corticosteroids.

Background: Recurrent aphthous stomatitis (RAS) is an inflammatory nature, while interleukin 17 (IL-17) has a pre-inflammatory role. In the present study, serum level of IL-17 was measured in a group of individuals with RAS, compared to healthy individuals.

Methods: This case-control study was done on 36 patients with RAS as cases and 36 healthy subjects as controls. The level of IL-17 was measured, using ELISA method.

Results: The mean serum level of IL-17 in cases was 0.14±0.21 pg/ml, which was insignificantly lower than 0.21±0.17 pg/ml in control group (p=0.12). Meanwhile there was a significant reverse correlation between age and serum level of IL-17 (r=-0.32, p=0.006).

Conclusion: Although the serum level of IL-17 in RAS patients was lower than the controls, the difference was not significant. However, significantly lower serum level of IL-17 in older subjects.

Background: Griscelli syndrome type II is a primary immunodeficiency disorder caused by RAB27A gene mutation and is inherited in an autosomal recessive manner and characterized by oculocutaneous hypopigmentation and various cellular immune system deficiencies.

Case Presentation: Herein, we report a 5-year-old girl with silvery-gray hair, eyebrows, and eyelashes who were referred to our primary immune deficiency clinic because of recurrent oral thrush. Further investigations were performed to uncover the probable underlying genetic disorder. Whole-exome sequencing revealed a novel mutation in the RAB27A gene (c.137T>G) and confirmed the diagnosis of Griscelli syndrome type 2.

Conclusions: Due to the poor prognosis nature of this disorder and also its need for differential diagnosis with some other conditions with hypopigmentation, prompt diagnosis, genetic analysis, and proper treatment are necessary for avoiding serious complications.