Unique Characteristics of Prepubertal Onset Systemic Lupus Erythematosus.

OBJECTIVES: The aim of this study was to investigate the influence of age at disease onset on disease expression and outcomes of pediatric systemic lupus erythematosus SLE (pSLE). METHODS: A total of 103 patients with pSLE from Sultan Qaboos University Hospital, Oman, were retrospectively studied. Epidemiological, clinical phenotype, disease severity, serology, treatment, and outcome were compared among the three groups using univariate statistical tests. RESULTS: The mean disease duration of the cohort was 9.8 ± 4.7 years. The patients were divided into three groups: prepubertal onset (n=39) with mean age at diagnosis of 5.1 ± 2.0 years and pubertal disease onset (n=29) with mean age at diagnosis of 10.8 ± 1.0 years as well as postpubertal disease onset (n=35) group with mean age at diagnosis of 15.3 ± 1.6 years. The prepubertal pSLE cohort demonstrates unique characteristics with increased frequency of familial SLE (61%) of which 49% were from first-degree relatives. Similarly, this group had distinctive clinical features, which included increased renal disease in pubertal and postpubertal groups, respectively (51% vs 23% vs 20%; p=0.039). Prepubertal, similar to pubertal group, had a higher incidence of cutaneous manifestations than in the postpubertal group (74% vs 69% vs 46%; p=0.029). Laboratory features in prepubertal group were distinct with increased frequency of positive anti-cardiolipin antibodies (47%), anti-glycoprotein antibodies (42%), ANCA (62%), and low complement levels (97%) compared to pubertal and postpubertal group. The prepubertal group also has the lowest frequency of positive SSA antibodies (18%) and SSB antibodies (5.1%). The overall mean SLEDAI score in pSLE cohort was 15.6 ± 18.5. The mean SLEDAI scores among the groups showed no significance difference (p=0.110). The overall SLICC DI ≥1 was 36% with a mean damage score of 0.76 ± 1.38. No significant differences in damage index (SLICC DI ≥1) were noted among the groups. CONCLUSIONS: Distinct clinical features were identified in prepubertal onset pSLE population of Arab ethnicity. Given the high rate of consanguineous marriage and high frequency of familial SLE in this cohort, these manifestations could be explained by higher frequency of genetic factors that influence the disease pathogenesis.

Incidence, outcomes and outcome prediction of unplanned extubation in critically ill children: An 11year experience.

PURPOSE: Unplanned extubation represents loss of control in the ICU, is associated with harm and is used as a measure of quality of care. We evaluated the rates and consequences of unplanned extubation. MATERIALS AND METHODS: Eligible patients were intubated, <18years, and in ICU. Patient, care-related and environmental characteristics were compared in patients who did and did not receive positive pressure ventilation in the 24h after events. Rates are expressed per 100 intubation-days. RESULTS: The 11,310 eligible patient-admissions identified were intubated for 75,519days; 410 (3.39%) patients had 458 unplanned extubation events (0.61 events/100 intubation-days). Annual rates of unplanned extubation reduced from 0.98 in 2004 to 0.37 in 2014. Consequences occurred in 245 (53.5%) events and included cardiac arrest in 9 (2%), bradycardia 52 (11%), and stridor 63 (14%). Positive pressure was provided after 263 (57%) events, and was independently associated with pre-event sedative and muscle relaxant drugs, non-use of restraints, respiratory reason for intubation and recent care by more nurses. CONCLUSION: Unplanned extubation was associated with both significant and no morbidity. Modification of factors including more consistent nurse staffing, restraint use, and increased vigilance in patients with previous events may potentially reduce rates and adverse consequences of unplanned extubation.

Bone mineral density in juvenile onset systemic lupus erythematosus.

OBJECTIVE: To compare bone mineral density in patients with juvenile-onset Systemic lupus erythematosus and healthy controls. PARTICIPANTS: Serial bone mineral density measurements in 27 patients with juvenile-onset systemic lupus were compared to 97 healthy age-matched controls. RESULTS: All patients with juvenile-onset had low bone mineral density scores at initial assessment that progressed over disease course. Low body mass index was independently associated with a decline in bone mineral density Z scores; disease activity, use of immunosuppressive agents and vitamin D levels were not risk factors. CONCLUSIONS: Patients with juvenile-onset systemic lupus erythematosus have low bone mineral density.

Juvenile systemic lupus erythematosus in the Sultanate of Oman: clinical and immunological comparison between familial and non-familial cases.

Consanguineous marriage is quite prevalent in the Sultanate of Oman, with up to 45% of marriages being consanguineous. The aim of this study was to determine demographic, clinical and serological characteristics between familial and non-familial cases of juvenile systemic lupus erythematosus (SLE) in a highly consanguineous region such as Oman. Hospital medical records were retrospectively reviewed for 44 consecutive children with juvenile SLE seen at Sultan Qaboos University Hospital, 16 with familial SLE and 28 with non-familial SLE. All the children included in the study were Omani, diagnosed before 13 years of age, and fulfilled the 1982 revised ACR criteria. Analyses were performed using descriptive statistics. There were largely no significant differences in the clinical and serological manifestations between the two cohorts. However, the familial SLE group was associated with worse SLEDAI score at onset of diagnosis compared with the non-familial cohort (12 vs. 17; p = 0.003) suggesting a greater severity of disease in the familial group. Even though the rate of familial SLE is higher in Oman (36%) compared with the Western world (10-12%), it appears that familial and non-familial SLE cases are in fact similar disease entities in both the West and the Middle Eastern countries.

Anti-CD20 monoclonal antibody in acute life threatening haemolytic anaemia complicating childhood onset SLE.

Haematological involvement occurs frequently in childhood onset systemic lupus erythematosus (SLE) with autoimmune haemolytic anaemia occurring in 10%. Conventional treatment includes high-dose steroids. Second line agents used in refractory cases include azathioprine, mycophenolate mofetil, cyclosporine and cyclophosphamide. Rituximab, anti-CD20 monoclonal antibody, is emerging in the treatment of SLE refractory to conventional therapy. We report the successful use of anti-CD20 therapy in the treatment of acute life threatening haemolytic anaemia, complicating childhood onset SLE. We propose that earlier introduction of rituximab may reduce the side effects related to other cytotoxic therapy mainly steroids and cyclophosphamide. However, in view of controversies in the reported side effects of rituximab in childhood onset SLE, multicentred randomised controlled trials are required to determine the long-term efficacy, safety and optimal dosing of rituximab in childhood onset SLE.

Childhood systemic lupus erythematosus in Sultanate of Oman: demographics and clinical analysis.

There is a worldwide variation in the incidence of childhood manifestation of systemic lupus erythematosus, which may be related to environmental, genetic and geographical factors. The aim of the study is to describe for the first time the clinical and immunological characteristics of childhood onset systemic lupus erythematosus at diagnosis in the Sultanate of Oman. We retrospectively reviewed the records of 50 children with systemic lupus erythematosus fulfilling the American College of Rheumatology criteria between 1990 and 2006. There were 42 girls and eight boys. The mean age of disease onset was low at 8.6 years (SD 3.1). The female:male ratio was 5.3:1. The most frequent clinical manifestations at initial diagnosis were: articular (76%), cutaneous (70%), haematological (68%) and renal (64%) dominated by class IV nephritis, pulmonary (26%), neuropsychiatric (18%) and cardiac (10%). A positive antinuclear antibody (>1:80) was present in 94% at diagnosis. Positive anti-double stranded DNA was present in 82% and low complement levels were present in 84% at diagnosis. This is the first review of childhood onset systemic lupus erythematosus in the Sultanate of Oman.