Morbidity and mortality in Iranian children with juvenile systemic lupus erythematosus

Juvenile systemic lupus erythematosus [JSLE] is a common rheumatologic disorder that involves multi organ systems. Prognosis of lupus in children may be poorer than in adults. In this study, we determined mortality and morbidity rates in the pediatric SLE in Iran. In a cross sectional study, we evaluated 120 children with SLE who had registered in our pediatric rheumatology database within 2004-2010. Data including sex, age, remission, age at the time of diagnosis, age at the time of study, various types of organ involvement, mortality and remission were extracted from this database. From 120 cases, 77% [92 cases] were females and 23% [28 cases] males [F:M=3.3]. Mean follow up period was 56 +/- 32 months and mean age at the time of manifesting disease 10.34 +/- 2.9 years. Mortality rate was 10% [12 cases] in our investigation. Musculoskeletal involvement showed significant difference between various age groups [P<0.01], that was more frequent in group of 7 years and older. Frequency of oral ulcer and ophthalmic involvement in boys was significantly higher than in girls [P<0.05]. Frequency of cardiovascular involvement [P<0.01] and renal involvement [P<0.01] was significantly higher in the patients who had no remission. There was a significant association between mortality rate and cardiac [P<0.02, OR=4.9], pulmonary [P<0.01, OR=10.167] and liver [P<0.05, OR=1.19] involvement. In our investigation 1-year survival rate was 97% and 5-year survival rate 89%. Liver, cardiac and pulmonary involvements have an association with higher mortality in JSLE patients

Neonatal lupus erythematosus following rheumatoid arthritis: case report and literature review

Neonatal lupus erythematosus [NLE] is a transient autoimmune disease of developing fetus and neonate in mothers with systemic lupus erythematosus [SLE]. In this report we introduce an infant with NLE whose mother had rheumatoid arthritis. Our case was a 40 day old male infant with discoid-like and annular skin lesions over forehead and neck, irritability and low grade fever. There was a history of prematurity due to preeclampsia. There was no cytopenia or cardiac involvement but liver enzymes were more than 5-fold increased. FANA, Anti Ro and La were negative. The mother had a history of un-controlled rheumatoid arthritis for 12 years with deformity in metacarpal and PIP and ulnar deviation in hands. FANA=1/640 and anti-SSB/La was positive in the mother but there was no other clinical and paraclinical sign of SLE. Without any treatment and during months, the skin and mucosal lesions gradually disappeared without any scar and liver enzymes reached the normal level. After 6 months follow up, he was symptom free with normal growth and development. We recommend to check anti SSA/Ro and anti SSB/La antibodies in all pregnant women with connective tissue diseases to prevent life-threatening involvement of the infant

Periodic fever: a review on clinical, management and guideline for Iranian patients - part II

Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. In the first part of this paper, we presented a guideline for approaching patients with periodic fever and reviewed two common disorders with periodic fever in Iranian patients including familial Mediterranean fever [FMF] and periodic fever syndromes except for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis [PFAPA]. In this part, we review other autoinflammatory disorders including hyper IgD, tumor necrosis factor receptor-associated periodic syndrome [TRAPS], cryopyrin associated periodic syndromes, autoinflammatory bone disorders and some other rare autoinflammatory disorders such as Sweet's and Blau syndromes. In cryopyrin associated periodic syndromes group, we discussed chronic infantile neurologic cutaneous and articular [CINCA] syndrome, Muckle-Wells syndrome and familial cold autoinflammatory syndrome. Autoinflammatory bone disorders are categorized to monogenic disorders such as pyogenic arthritis, pyoderma ;gangraenosum and acne [PAPA] syndrome, the deficiency of interleukine-1 receptor antagonist [DIRA] and Majeed syndrome and polygenic background or sporadic group such as chronic recurrent multifocal osteomyelitis [CRMO] or synovitis, acne, pustulosis, hyperostosis and osteitis [SAPHO] syndrome are classified in sporadic group. Other autoinflammatory syndromes are rare causes of periodic fever in Iranian system registry

Juvenile Churg-Strauss syndrome as an etiology of myocarditis and ischemic stroke in adolescents; a case report

Churg-Strauss syndrome [CSS], a systemic vasculitis accompanied by asthma and eosinophilia, almost invariably affects the lung and is frequently associated with cutaneous involvement It rarely has cardiac involvement. We report an unusual case of CSS with myocardial. A 16-year old female suffered of allergic asthma for 4 years. She was under treatment with oral prednisolone and seretide inhalation. After CSS diagnosis, she developed paroxysmal atrial tachycardia. Serum levels of Troponin I and Troponin T were increased indicating massive myocardial damage probably due to myocarditis. After 5 months she developed acute hemiparesis without any evidence of ischemic or hemorrhagic event. She was treated with IVIg, intravenous pulses of methylprednisone and cyclophosphamide for each complication. Myocarditis and stroke may also complicate CSS which should be taken in consideration for better management

Pleuritic chest pain; where should we search for?

Pleuritic pain is not an unusual problem in children. Other concomitant symptoms should be considered for diagnostic approach in a child with pleuritic chest pain. In this report we discuss chest pain in a 6-year-old child with regard to other signs and symptoms. Finally, we found a rare life-threatening complication of juvenile systemic lupus erythematosus [JSLE] in our patient

Acute hemorrhagic edema of infancy; a report of five Iranian infants and review of the literature

Acute hemorrhagic edema of infancy [AHEI] is a benign self limiting leukocytoclastic vasculitis in young children. Serious complications, e.g. renal and gastrointestinal involvement, are not usually detected in AHEI patients. We report five patients with AHEI. Our patients were 17 to 21 months old. One patient presented with gastrointestinal bleeding due to this syndrome, the other one experienced second attack and scrotal edema due to epididymo-orchitis, while the third patient had renal involvement as hematuria and the other one had bilateral auricular chondritis. One of our cases was a typical case of AHEI without any complications, so a skin biopsy was not necessary. In this study, we describe the symptoms, probable triggering factors and treatment of choice for each patient. Although AHEI is a childhood vasculitis with no impairment of the general condition, some organ involvements such as gastrointestinal, renal or scrotal lesions and rarely chondritis are probable in these patients

Report on takayasu arteritis in three iranian children

Takayasu arteritis [TA] or giant cell vasculitis is the third common vasculitis after Henoch-Schoenline purpura and Kawasaki disease in children. This vasculitis usually affects large vessels and is more common in females during the childhood. Aim of this report is to present three Iranian children [two boys, one girl] with TA. Mean age of our cases on admission was 10 years. Patients most commonly presented with episodes of systemic symptoms including, fever, headache, increased arterial blood pressure in one limb and convulsion. Other related symptoms were myalgia, limb pain, chest pain, and abdominal pain. Supra sternal, and abdominal bruit was noted in all patients. Angiography was performed in all cases. This revealed stenosis of the left subclavian artery and common carotid artery in one patient, generalized aortitis in one case and stenotic right renal artery in one patient. One boy was diagnosed as having systemic onset of juvenile idiopathic arthritis. One case was referred with hand pain and headache, and one case for control of hypertension. Follow-up ranged from 2 to 7 years since diagnosis. All patients were treated with prednisone, azathioprine, aspirin, and antihypertensive drugs. Each patient received either methotrexate, or hydroxychloroquine or mycofenolate mofetil. Although TA is uncommon before 10th year of life, it should be considered in patients presenting with hypertension and systemic symptoms such as fever, limb pain and pulseless limb

Pleuropulmonary manifestations in juvenile systemic lupus erythematosus; a review and descriptive study in 64 cases

Juvenile systemic lupus erythematosus [JSLE] is a multisystemic autoimmune disease that can involve multiple organs such as: skin, kidney, musculoskeletal system, brain, and others as well as lung. Pulmonary manifestations may be an initial and/or life-threatening complication of SLE in children. The aim of this report is to describe the first pleuropulmonary manifestation of childhood lupus erythematosus. We studied retrospectively 64 children with JSLE, diagnosed as JSLE at the Children's Medical Center Hospital between 1995 and 2005. All met the American College of Rheumatology [formerly American Rheumatism Association, ARA] revised criteria for SLE. They were evaluated for evidence of pleuro-pulmonary involvement. During the 10-yr study period, 64 patients were diagnosed as childhood-onset JSLE, who had the disease at or before the age of 16 [3-16 years]. Fifty five patients [86%] were females and 9 patients [14%] were males [female: male ratio=6/1]. Mean age of this group at the onset of the disease was 10 years [range 3-16]. Eighteen cases [28%] had pulmonary involvement. Pulmonary complications include: infectious pneumonia in 38%, pleuritis in 33%, pulmonary vasculitis in 11%, and acute lupus pneumonitis, chronic interstitial pneumonitis and pulmonary embolism [so-called lupus anticoagulant] each one in 5.5%. The prevalence of pulmonary involvement in patients with JSLE varies according to the method used, but clinically significant pulmonary involvement in our series occurs in approximately 28%. There is few data regarding the treatment for most of the immune mediated pulmonary manifestations of JSLE

B-cell lineage study in patients with juvenile idiopathic arthritis

Juvenile idiopathic arthritis [JIA] is the most common rheumatic disease in children. The exact causes of disease are still poorly understood. It seems that B cells have several functions in JIA, including production of autoantibodies, antigen presentation, production of cytokines, and activation of T cells. Here, we aimed to evaluate B-cell lineage and its precursors in the bone marrow of patients with JIA Twenty consecutive patients with JIA were enrolled in this study. JIA is subdivided into three groups of Pauciarticular, Polyarticular, and Systemic JIA. Bone marrow mononuclear cells were separated. Then we analyzed the immunophenotype of the JIA patients by flow cytometry. After separation, the mononuclear cells were stained specific for B cell lineage [CD10, CD19 and CD20], T cell lineage [CD3] and non specific lineage [CD34, HLA-DR and TdT]. Flow cytometric study of bone marrow showed that JIA patients had low level of CD10, CD19, and CD20.Polyarticular patients had lower level of D10, CD19, and CD20 than pauciarticular JIA patients and systemic onset JIA patients had lower levels than both of them. Decreasing of B cell precursor in bone marrow is one of mechanisms for pathogenesis of JIA and the more decreased B cell precursors in bone marrow are, the worst severity of the disease is. Significant differences in CD10 content of bone marrow were detected between the polyarticular and pauciarticular groups. So, it seems that polyarticular JIA patients had lower percentage of pre B cell stage

Lupus headaches in 55 childhood-onset SLE

Although headache is a common complaint among patients with lupus, no universally accepted explanation was available until the International Headaches Society adopted Lupus headache as a Nomenclature in its classification recently. Few studies indicate that lupus patients with positive anti-nuclear antibody [ANA] and positive antiphospholipid antibodies [aPL] experience more frequent headaches. The aim of this study was to determine the correlation between headache frequencies and ANA, anti-double strand DNA [anti-ds-DNA] and aPL positivity. In this prospective multicenter study were enrolled 55 children, 45 girls and 10 boys [F/M ratio:4.5], aged 3-16 years [mean 11.5 years], with neuropsychiatric lupus complaining of headache, that where followed-up for 5 years.. Whether lupus headache is a sign of progressive nature of the disease and how it should be treated is not clear yet. Those with active disease, hypertension, or tension headache were not included in this study. We studied 55 children with definite lupus. Twenty three [43%] of our patients developed new or significantly worse, persistent headaches that sometimes were similar to migraine in the early course of their disease. However their headaches were not accompanied with disease flare up and the headaches were not found to be related to hypertension or use of other medications either. Accordingly, we came to a diagnosis of lupus headache for these patients. Among them 19/55 cases [35%] had a positive aPL and 53/55 cases [96%] had a positive ANA. Lupus headaches are most likely multifactorial, and probably only a small proportion of them truly represent active lupus. The above data highlights probable correlation between aPL, ANA, an anti-ds-DNA and lupus headache. However, more research is required to find better treatments and to establish a definitive correlation among them

Kawasaki disease in 159 Iranian children

Kawasaki disease [KD] is an intense life-threatening vasculitis. The diagnosis of Kawasaki disease is made by clinical criteria. This disease is a common rheomatologic disease in Iran. The aim of this stody was describe the demographics and clinical features of KD in Iranian patients. A review was conducted for all cases of KD treated at Pediatric rheumatology department in Children's Medical Center between January 1994 and July 2004. The diagnostic criteria for typical Kawasaki were based on the criteria of the Japan Kawasaki Disease Research Committee. Atypical or incomplete KD has been described in which patients not strictly meeting the diagnostic criteria but have coronary artery changes. Color doppler echocardiograms were done at the time of diagnosis, 14 to 21 days, 60 days, and 1 year after treatment. One hundred fifty nine patients were identified. One hundred twenty five children [78.6%] fulfilled criteria for typical KD. Echocardiographic abnormalities were found in 30 cases [18.9%], including 9.6% with typical and 46.1% with atypical Kawasaki. The incidence of atypical Kawasaki in our study was about 22%. Coronary arteries aneurysms found in 66.7% and 33.3% was other abnormalities. Male to female ratio was more in patients with cardiac complication [2.3:1]. Kawasaki disease should be considered in any infants or child [especially less than 2 years old] with a prolonged febrile illness. Demographic features of our patients were similar to reports from other country. The incidence of atypical Kawasaki in our study about 22%