Clinical course and outcomes of Iranian children with juvenile dermatomyositis and polymyositis.

This study evaluated the clinical features, course, and outcomes of Iranian children with juvenile dermatomyositis (JDM), juvenile polymyositis (JPM), and other uncommon connective tissue disorders. A chart review of 85 Iranian children with JDM and JPM was performed during a 10-year period from 2003 to 2013. The patients' clinical signs and symptoms, laboratory data, and other factors affecting clinical outcomes were recorded using questionnaires. Statistical analysis was performed using SPSS software version 20. In all, 40 boys and 45 girls were included in the study (F/M, 1.1:1). Disease frequency was significantly higher in boys aged <5 years (F/M, 0.4:1) and girls aged >5 years (F/M, 1.6:1). The combined mean age at diagnosis was 7.5 years. Muscle weakness, particularly in the proximal muscles of lower extremities (96 %); fatigue (83 %); and heliotrope rash (71 %) were the most frequently recorded symptoms. Elevated lactate dehydrogenase level was the most common enzyme disturbance (98 %). Monocyclic course was seen in 60 % of patients. The mean treatment duration was 3 years. The incidence rate of complications such as calcinosis, lipodystrophy, and growth disturbances was 20, 9, and 30 %, respectively. The occurrence of these complications in patients with monocyclic disease was significantly lower. Vital organ involvement led to the death of four patients. The incidence of calcinosis was significantly lower in patients having a shorter interval between disease onset and treatment. Two important complications, failure to thrive and lipodystrophy, were significantly higher in patients having antinuclear antibodies. The incidence of the above three complications was higher in patients with polycyclic or continuous chronic disease. Respiratory failure was the most common cause of patient mortality.

Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome in Iranian Children First Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR).

OBJECTIVE: The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a nonhereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. No longtime sequel was reported in this disease. Early diagnosis can lead physicians to treatment of this disorder with a short course steroid application and provide satisfaction of the patient's family. METHODS: This study is a prospective review of patients diagnosed with PFAPA syndrome who were registered in Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR) through periodic fever clinic in the Children's Medical Center, Pediatric Center of Excellence in Tehran, Iran from January 2013 to March 2014. FINDINGS: One hundred thirty patients were registered in our databases. Twenty-one (16.1%) patients including 15 males and 6 females had PFAPA. Normal growth was seen in all patients. The median age at onset was 18 months. The mean duration of fever was 4 days and the mean duration of intervals between fever episodes 21 days. Along with fever, all patients had characteristic symptoms. All patients were asymptomatic between fever episodes. Steroid was used in all patients and causing immediate reduction by 84.61%. Two patients received both steroid and colchicine because of their clinical feature and positive laboratory tests for PFAPA and familial Mediterranean fever. No patient received biological therapy or a tonsillectomy. CONCLUSION: The long diagnostic delay of PFAPA gives cause to concern indicating a need for greater awareness of the disease so that the diagnosis may be made timely.

Sensitivity and specificity of adenosine deaminase in diagnosis of juvenile idiopathic arthritis.

BACKGROUND: Juvenile Idiopathic Arthritis (JIA) is one of the most common chronic rheumatic diseases inchildren with unknown etiology and pathogenesis. It also has no diagnostic test and its clinical diagnosis ismade through ruling out other types of arthritis. The aim of this study was to evaluate the level of ADA (AdenosineDeaminase) in the serum of JIA patients and to compare it with that of patients with Reactive Arthritis(RA). Evaluation of sensitivity and specificity of serum ADA level in JIA was another objective. METHODS: The study included 120 children with JIA (mean age= 7.6 ± 4.3 years) and 40 children with RA(mean age= 5.5 ± 3.1 years). The ADA was measured in the active phase of both diseases. RESULTS: The mean ADA serum level was obtained as 15.8 ± 11.8 U/l in JIA patients and 14.3 ± 7.5 U/l in RApatients. The difference was statistically insignificant (p= 0.4). Another finding of this study was the significantspecificity (77.5%) of this laboratory parameter for JIA in comparison with its low sensitivity (36.7%). Positivepredictive value was 83% and negative predictive value 29%. CONCLUSION: Determination of ADA serum levels is a noninvasive reliable and easy biomarker for diagnosis ofJIA and it can be used as alternative parameters representing disease activity.

Evaluation of 10-year experience of Wegener's granulomatosis in Iranian children.

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%).

The effect of positive family history of autoimmunity in juvenile idiopathic arthritis characteristics; a case control study.

OBJECTIVE: To compare Juvenile Idiopathic Arthritis (JIA) patients with and without family history of autoimmune disease with respect to clinical features and laboratory data. METHODS: Sixteen JIA patients with family history of autoimmune disease were identified during study, 32 patients were chosen for comparative group from referred patients to the rheumatology clinic according to the date of referral. Two groups were compared with respect to age of onset, sex, subtype, disease activity, duration of active disease and laboratory variables. FINDINGS: The age of onset was significantly lower in JIA patients with family history of autoimmunity (4.7 years vs. 7.0 years; P=0.02), polyarthicular subtype was more frequent in patients with positive family history (50% vs.25%; P=0.04) most of JIA patients with positive family history were in the active phase at the time of study (64% vs 25%; P=0.02) and had a longer duration of active disease (21.0 months vs 12.3 months; P=0.04). Patients with positive family history had more positive ANA (43.5%% vs 12.5%; P=0.01) and also more positive ADA (75% vs 20.8%; P=0.002). Two groups were similar according to sex, and other laboratory variables. CONCLUSION: JIA patients with family history of autoimmune disease seem to have a more severe disease than patients without such family history, they are younger at the onset, and have mostly poyarthicular subtype. They also have more ANA and ADA positivity. These findings are different from familial JIA case-control studies according to active disease duration, subtype, and ANA positivity.