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OBJECTIVES: To assess the prevalence and types of cardiovascular complications in Saudi patients with paediatric-onset systemic lupus erythematosus (pSLE). METHODS: Retrospective record review of pSLE patients following from January 2014 to September 2015 at the rheumatology clinic of King Abdul-Aziz University Hospital, Jeddah. Laboratory data such as C-reactive protein (CRP), antinuclear antibodies (ANA), anti-double stranded DNA antibody (anti-dsDNA), C3 and C4 complements, were collected. Cardiac evaluation included chest x-ray, electrocardiogram (ECG), and echocardiography, along with estimation of SLE activity by calculating the SLE Disease Activity Index (SLEDAI) score according to SELENA modification. RESULTS: Forty-six cases of pSLE were included (91.3% females, mean±SD age at diagnosis=10.53±2.28 years). Prevalence of cardiac manifestations was 47.8%; the most frequent of which were valvular heart diseases diagnosed in 16 (34.8%) cases, followed by pericarditis in 6 (13%) cases. Of the 16 valvular diseases, tricuspid and pulmonary valves were involved in 9 and 8 cases, respectively. Cardiac involvement was silent in 36.4% and occurred as an initial presenting symptom of SLE in 9.1% cases. Biologically, patients with cardiac involvement had higher levels of CRP and anti dsDNA, and lower levels of complement C3 compared to patients with no cardiac involvement; while high SLE activity was the only significant predictor for cardiac involvement (beta=0.654; p=0.020). CONCLUSIONS: Cardiac complications are common in Saudi children with pSLE and are asymptomatic in 1 out of 3 times. They are predicted by high SLE activity and associate with high anti-dsDNA and CRP and low C3 levels.
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Enfermedades de las Válvulas Cardíacas/epidemiología , Lupus Eritematoso Sistémico/epidemiología , Pericarditis/epidemiología , Adolescente , Edad de Inicio , Autoanticuerpos/sangre , Biomarcadores/sangre , Proteína C-Reactiva/análisis , Niño , Complemento C3/análisis , Complemento C4/análisis , Ecocardiografía , Electrocardiografía , Femenino , Enfermedades de las Válvulas Cardíacas/sangre , Enfermedades de las Válvulas Cardíacas/diagnóstico , Humanos , Mediadores de Inflamación/sangre , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/diagnóstico , Masculino , Pericarditis/sangre , Pericarditis/diagnóstico , Prevalencia , Estudios Retrospectivos , Arabia Saudita/epidemiologíaRESUMEN
Introduction. Juvenile Idiopathic Arthritis (JIA) is the most common chronic arthritis in children. Our aim is to describe demographic, clinical, and laboratory characteristics and treatment of JIA patients followed up in Pediatric Rheumatology clinic in a tertiary center in Saudi Arabia. Methods. Medical records of all patients who are followed up between January 2007 and January 2015 were retrospectively reviewed. Data were collected about demographic, clinical, and laboratory features and treatment. Results. Total patients were 82, males were 31 (37.8%), and mean age of JIA onset was 7.1 ± 3.6 yr. Mean follow-up duration was 2.67±1.6 yr. Systemic onset JIA (SoJIA) was the commonest (36.5%), followed by polyarticular in 29.2% and oligoarticular in 28%. Large and small joints are involved in 76 (92%) and 30 (36.6%), respectively. Main extra-articular feature was fever in 34 (41.4%). Uveitis was diagnosed in 7 (8.5%) and in 5 (21.7%) of oligoarticular JIA. Anemia was found in 49 (59.7%), high ESR in 45 (54.8%), and leukocytosis and thrombocytosis in 33 (40.2%). Positive ANA was found in 30 (36.5%) mainly in oligoarticular subtype as 12 (52%) patients (out of 23) had this positive test. 9 patients (10.9%) required NSAIDs only, 6 patients (7.3%) required NSAIDs and intra-articular steroids only, and 19 (23%) required NSAIDs, methotrexate, steroids, and biologics. Conclusion. SoJIA is the most common JIA subtype in our study. A population based rather than a single center study will give more details about JIA characteristics in Saudi Arabia.
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To study the demographic, clinical, laboratory features, treatment and outcome of childhood systemic lupus erythematosus (SLE) in western province of Saudi Arabia. Children with SLE who were diagnosed at King Abdulaziz University Hospital, Jeddah, between March 1998 and October 2008 were included. Charts of all patients were reviewed retrospectively for clinical and laboratory features, treatment and outcome. There were 28 girls and 2 boys, with a mean age of 10.5 years (range 5-18). The female:male ratio was 14:1. Constitutional symptoms represent significant symptoms. Hematological manifestations were the most frequent finding (86.7%) at the time of diagnosis followed by arthritis and nephritis (73.3%). The malar rash represents the most common skin manifestation (46.7%). Discoid lupus was very rare. Neurological symptoms were seen in 30%, while cardiac and pulmonary involvement was uncommon. All patients had positive ANA and 90% of them had high anti-ds DNA. All patients were treated with steroids and hydroxychloroquine and 26 patients received immunosuppressive therapy. Three patients died due to severe infection; massive brain infraction and severe lung disease (one in each). Twenty-seven patients are alive in stable condition. Clinical manifestations and laboratory abnormalities were similar to previously reported series. This report confirms that SLE has comparable findings among children from different ethnicities.
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Riñón/fisiopatología , Lupus Eritematoso Sistémico/diagnóstico , Adolescente , Artritis/complicaciones , Artritis/fisiopatología , Encéfalo/fisiopatología , Niño , Preescolar , Fatiga/complicaciones , Fatiga/fisiopatología , Femenino , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Masculino , Arabia Saudita , Piel/fisiopatologíaRESUMEN
OBJECTIVE: To determine whether levels of soluble tumor necrosis factor receptor 55 (sTNFR55), sTNFR75, and interleukin 1 receptor antagonist (IL-1Ra) can differentiate different subtypes of juvenile rheumatoid arthritis (JRA), and to determine if the levels of these proteins correlate with disease activity. METHODS: Serum sTNFR (55 and 75) and IL-1Ra levels were measured by ELISA in 34 patients with JRA and these values were correlated with disease subtype and activity. RESULTS: Serum sTNFR55 levels were significantly elevated in patients with systemic onset JRA (SoJRA) (mean +/- 2 SD, 2.9 +/- 1.8 ng/ml) (p < or = 0.05) compared to rheumatoid factor positive (RF+) polyarticular JRA (2.1 +/- 0.6), RF-polyarticular JRA (1.5 +/- 0.6), and pauciarticular JRA (1.4 +/- 0.4). There was a trend for elevation of sTNFR75 levels in patients with SoJRA compared to other subtypes (p = 0.08). More patients had elevated levels of sTNFR75 than sTNFR55 (15 vs 7). This was true for all subsets (SoJRA 7 vs 5; polyarticular JRA 4 vs 2; and pauciarticular JRA 4 vs 0). In contrast to sTNFR, IL-1Ra levels were significantly elevated in RF+ polyarticular JRA compared to the other subgroups (p < or = 0.001). We found statistically significant Pearson correlations between (1) sTNFR75 and hemoglobin concentration: and (2) IL-1Ra and number of active joints and number of joints with effusions. CONCLUSION: The increased serum level of sTNF receptors in SoJRA suggests that TNF is likely more important than IL-1 in systemic inflammation and in particular in SoJRA. Conversely, IL-1 is likely more important in the inflammatory arthritis of JRA and in particular in the pathogenesis of RF+ polyarticular JRA. Our results suggest that cytokines have differing roles in JRA subtypes and likely reflect JRA subtype heterogeneity.
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Antígenos CD/sangre , Artritis Juvenil/sangre , Artritis Juvenil/inmunología , Receptores del Factor de Necrosis Tumoral/sangre , Sialoglicoproteínas/sangre , Adulto , Antígenos CD/inmunología , Artritis Juvenil/diagnóstico , Niño , Preescolar , Femenino , Hemoglobinas , Humanos , Proteína Antagonista del Receptor de Interleucina 1 , Masculino , Valor Predictivo de las Pruebas , Receptores del Factor de Necrosis Tumoral/inmunología , Receptores Tipo I de Factores de Necrosis Tumoral , Receptores Tipo II del Factor de Necrosis Tumoral , Índice de Severidad de la Enfermedad , Sialoglicoproteínas/inmunología , SolubilidadRESUMEN
OBJECTIVE: To study the pattern of clinical features, complications, and outcome of Kawasaki disease among Saudi children. METHODS: Medical records and referral letters of all children (1997 through to 2001) diagnosed with Kawasaki disease were reviewed. This study was carried out at King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia. Collected data included clinical features, laboratory results, echocardiogram findings, therapy, complications and outcome. RESULTS: Thirteen children (10 boys, 3 girls) were reviewed, age range from 0.3 to 7 years (mean 3.4 years). Nine patients met 5 out of 6 criteria for the diagnoses of Kawasaki disease and 4 met 4 out of 6 criteria and coronary aneurysm. Thirteen patients had fever and skin rash for more than 5 days, 12 had extremity and oral mucus changes, 9 had bilateral conjunctivitis, and 7 had cervical lymph node enlargement. Other associated clinical features include diarrhea, and aseptic meningitis in 3 patients, ischemia of the fingers and toes in 2, arthritis in 2, arthralgia in one, seizure and pneumonia in one. Hepatosplenomegaly, pancytopenia and elevated liver enzymes in one, hepatomegaly and normal liver enzymes in 2 patients. Cardiac complication seen in 4 patients (30%), all of them were boys, 3 had coronary artery aneurysm (4-7 mm) and one had giant aneurysm (9mm), one of the 3 patients had axillary and subclavian artery aneurysm as well. Two out 4 patients were treated with intravenous immunoglobulin and aspirin within 10 days of illness and one was treated on day 21 and one was treated with aspirin alone. Aneurysmal changes persisted in 3 patients and resolved in one patient who was treated early. Extra-cardiac complications include reaction to intravenous immunoglobulin, coagulopathy, thrombocytopenia, hemophagocytic syndrome and ischemia of peripheral extremities. CONCLUSION: Our observation showed a high percentage (30%) of coronary aneurysm and unusual complications, this is most likely due to delay in the diagnosis and initiation of treatment.