Background.Systemic lupus erythematosus (SLE) is a frequent rheumatology disorder among children. Since hepatic involvement is a common systemic manifestation in lupus, the frequency and type of hepatic involvement were determined in pediatric cases of SLE admitted to Children’s Medical Hospital from 2005 to 2014.Methods and Patients.In this observational case-series study, 138 pediatric cases of SLE were admitted in Children’s Medical Center (a pediatric rheumatology referral center in Tehran, Iran) enrolled from 2005 to 2014 and the outcomes, frequency, and type of hepatic involvement were assessed among them.Results.Hepatic involvement was reported in 48.55% of total SLE patients. Aspartate aminotransferase (AST), alanine aminotransferase (ALT), and both enzymes higher than normal upper limits were detected in 8.7%, 5%, and 34.7% of lupus patients, respectively. Increased level of liver enzymes was categorized as less than 100, between 100 and 1000, and more than 1000 levels in 23.1%, 23.1%, and 2.1% of cases. The only gastrointestinal involvement in lupus patients contributing to hepatic involvement was gastrointestinal bleeding. Rising in liver enzymes was detected mostly in lupus patients without gastrointestinal bleeding (52.2% without versus 25.8% with gastrointestinal bleeding,P=0.007).Conclusion.Approximately half of the pediatric patients suffering from SLE have hepatic involvement. No significant correlation was observed between various organs involvement and abnormal level of liver enzymes.

Wegener’s granulomatosis or granulomatosis polyangiitis (GPA) is an uncommon chronic systemic vasculitis in children. The aim of this study was to describe pediatric patients with GPA in Iran. We studied records of all patients with GPA diagnosis who were referred to all Iranian Pediatric Rheumatology Division from 2002 to 2011. A total of 11 patients (5 females and 6 males) enrolled in this study. In children less than 15 years old, the prevalence of GPA is 0.6 per million. The mean age of GPA diagnosis was 11 years and average delay diagnosis was 20 months. Mortality rate was 18.1% due to pulmonary vasculitis and infection. The most common organ system involvement was upper and lower respiratory tract involvement (81.8% and 63.9%, resp.). Other common manifestations were renal (36.3), skin (27.2%), and eye involvement (18.2%).

Background: Kawasaki disease (KD) is often complicated by coronary artery lesion (CAL), including dilatation or aneurysms. Intravenous immunoglobulin (IVIG) is used with aspirin to prevent CAL in KD. Objective: Given that the primary treatment for other vasculitis is the use of corticosteroids, this study has been performed to evaluate the effect of intravenous methylprednisolone pulse (IVMP) therapy in preventing CAL in KD. Method: A randomized, single-blind clinical trial was conducted on 40 KD patients aged six months to five years. Patients were randomized into two groups according to the main treatment plan in addition to aspirin: case group (IVMP for three consecutive days and then oral prednisolone for three days) and control group (intravenous immunoglobulin 2 g/kg). Echocardiography was performed for all children at least three times, during the acute phase, two weeks, and two months later. Results: Data analysis at the end of the study was done on 40 patients (20 patients in each group). There were no significant differences in age and sex distribution, mean fever, and acute phase duration, as well as baseline echocardiography in the two groups. The frequency of CAL was 20% in the case group and 45% in the control group, after two weeks (p<0.05), but there was no significant difference between two groups in types of coronary artery lesion after two weeks and the frequency and severity of CAL after two months. Conclusion: IVMP as initial line therapy effectively control systemic and vascular inflammation and decrease coronary artery damage in KD.

Objectives. Familial Mediterranean Fever (FMF) is one of the most prevalent periodic fever syndromes; MEFV, the responsible gene for the disease, is in the short arm of chromosome16. In the considerable count of the FMF patients, only one mutation is found in the MEFV and parents, who were the obligatory carriers for that mutation, were asymptomatic. The aim of this study was to evaluate these asymptomatic parents in regard to mutation in MEFV gene and similarity between parents and offspring patients. Methods. In this cross-sectional study, asymptomatic parents of FMF patients enrolled the study were referred to periodic fever clinic or pediatric rheumatology clinic of Tehran University of Medical Sciences. The patients should have at least one mutation in MEFV gene and none of them had any family history of autoinflammatory disease. Twelve mutations in MEFV gene were assessed in the parents by Vienna Lab FMF Strip Assay kit by MAS PCR/Reverse hybridization. Results. Forty-three patients and their parents participated in the study. Sixty-three percent (27) of patients were male. Onset of disease symptoms in 31 patients (72%) was before 4 years of old. Nine (21%) of the patients had homozygote, 16 (37%) compound heterozygote, and 17(40%) heterozygote for MEFV mutation; there was a case of complex alleles mutations (2%). M694V/M694V in 4 patients (9%) was the most homozygote genotype, and M694V/R761H in 4 (9%) and E148Q in 7 (16%) were the most compound heterozygote and heterozygote genotype, respectively. M694V, M680I, and E148Q were the most mutation in the parents. Overall, 41 patients had mutations similar to their parents’ mutation, except 2 whose parents had no mutation, but a patient did. Conclusion. It seems that occurrence of new mutations in offspring is not prevalent among FMF patients and there are other reasons for different clinical presentation in similar mutation carriers. On the other hand, in ethnicities with high prevalence of FMF, new mutation in descendant may occur, infrequently.

Background: Hyper-inflammatory syndrome in children and young adult occur 2–6 weeks after COVID-19 infection or closed contact with COVID-19 persons. In this study, the laboratory data and echocardiography and abdominal ultrasonography assessments were evaluated before and after Methylprednisolone pulse as an initial treatment of hyper-inflammatory syndrome. Therefore, the aim of this study is to assessment the clinical manifestations and laboratory data and outcome after methylprednisolone pulse as an initial treatment. Method: In this retrospective study, the demographic status, clinical features, laboratory data, echocardiography, abdominal ultrasound, treatment and outcome of 31 pediatric patients under 16 years old, with inflammatory process after COVID-19 were evaluated. The clinical assessments, laboratory data, sonography and echocardiography were evaluated before and after methylprednisolone pulse. The patients were divided in two age group < and ≥ 7 years old and the clinical manifestations were compared with each other. The Mann–Whitney U test was used to assess the difference in quantitative variables between two groups. To compare pre- and post- treatment values, Wilcoxol test was used. To assess the correlation between qualitative variables chi-square test was used. The level of significant was considered 0.05. These patients with fever and hyper-inflammation features admitted to the referral pediatric rheumatology ward in Children Medical Center of Tehran University of medical sciences, from April 2020 to May 2021 were assessed. Result: The mean age ± SD were (5.94 ± 3) and 51.6% (16) patients were male and 48.4% (15) patients were female. The most documented of previous COVID infection were antibody positive in about 27 (87%) patients. Moreover, 1 (3.8%) was PCR positive, 2 (7.7%) were positive in both PCR and serology and 3(11.5%) had closed contact with COVID-19 patients. About 9(29%) of patients were admitted in Intensive Care Unit (ICU). There were significant correlation between days of delay in starting treatment and ICU admission (P-value = 0.02). The mortality rate was negative in patients and no re-hospitalization was documented. There were significant differences (P-value < 0.05) between lymphocytes, platelet, Erythrocyte Sedimentation rate, C-reactive protein, Aspartate transaminase, Alanine transaminase and ferritin before and after treatment. Skin rashes and cardiac involvement totally as carditis (myocarditis, vulvulitis and pericarditis) (33.3%) and coronary involvements (53.3%) were the most prominent initial presentation in patients. There were near significant correlation (P-value = 0.066) between ferritin level and carditis before treatment. Cervical lymphadenopathy was seen significantly more in ≥ 7 years old (P-value = 0.01). Conclusion: Multisystem inflammatory system in children as a hyperinflammatory syndrome could be treated with first step methylprednisolone pulse with decreasing inflammation in laboratory data and cardiac involvements and good outcome. Furthermore, the ferritin level may be one of the predictor of severe hyper-inflammatory syndrome leading to aggressive and urgent treatment with methylprednisolone pulse.

This study aims to identify the patterns and risk factors of uveitis in a cohort of Iranian children referred to a tertiary pediatric rheumatology clinic. A retrospective analysis was performed using the medical records of all patients with uveitis of any cause who were referred to a tertiary pediatric rheumatology clinic, along with subsequent juvenile-onset Behçet's disease and juvenile idiopathic arthritis (JIA) cases as controls. Causes of uveitis included JIA (n = 24), Behçet's disease (n = 15), idiopathic uveitis (n = 14), Sarcoidosis/Blau syndrome (n = 3), systemic lupus erythematosus (n = 2) and reactive arthritis (n = 1). Anterior and panuveitis were the most common anatomical subtypes. Retinal vasculitis was found in 28.8% of patients and 57.6% had bilateral uveitis. Complications occurred in 32% of cases, persistent visual impairment in 23% and legal blindness in none. The median "time-to-uveitis" ranged from 1 year in oligoarticular JIA to 6.5 years in the polyarticular group, with no significant difference in cumulative prevalence. Risk factors of persistent/recurrent uveitis were a lower age at onset of uveitis (p = 0.010) and retinal vasculitis (p = 0.049). This study presents a distinct pattern of uveitis among different etiological groups and describes three rare causes of uveitis. JIA-associated uveitis has a late-onset peak in the polyarticular group. This study adds to the existing evidence on the importance of long-term screening for uveitis and early treatment in pediatric rheumatic diseases.