Lupus nephritis in Chinese children--a territory-wide cohort study in Hong Kong

We report a multicenter study of Chinese children in Hong Kong with systemic lupus erythematosus (SLE) nephritis. Children were included if: they fulfilled the ACR criteria, had significant proteinuria or casturia, were Chinese and younger than 19 years and had been diagnosed with SLE between January 1990 and December 2003. Investigators in each center retrieved data on clinical features, biopsy reports, treatment and outcome of these patients. There were 128 patients (eight boys, 120 girls; mean age: 11.9+/-2.8 years). About 50% presented with multisystem illness and 40% with nephritic/nephrotic symptoms. Negative anti-dsDNA antibodies were found in 6% of the patients. Renal biopsy revealed WHO Class II, III, IV and V nephritis in 13 (10%), 22 (17%), 69 (54%) and 13 (10%) patients, respectively. The clinical severity of the nephritis did not accurately predict renal biopsy findings. The follow-up period ranged from 1 to 16.5 years (mean+/-SD: 5.76+/-3.61 years). During the study five patients died (two from lupus flare, one from cardiomyopathy, two from infections). Four patients had endstage renal failure (ESRF) (one died during a lupus flare). All deaths and end-stage renal failure occurred in the Class IV nephritis group. Chronic organ damage was infrequent in the survivors. The actuarial patient survival rates at 5, 10 and 15 years of age were 95.3, 91.8, and 91.8%, respectively. For Class IV nephritis patients, the survival rates without ESRF at 5, 10, and 15 years were 91.5, 82.3 and 76%, respectively. The survival and chronic morbidity rates of the Chinese SLE children in the present study are comparable to those of other published studies.postprin

Skin is the most frequently damaged system in recent-onset systemic lupus erythematosus in a tropical region

The objective of this study was to evaluate the early damage as measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) in Brazilian systemic lupus erythematosus (SLE) patients with disease duration of 2 and 3 years and to evaluate the possible association between SLICC/ACR DI score and sociodemographic and clinical data. the SLICC/ACR DI was measured in 54 patients with SLE according to the ACR criteria for SLE and a mean (SD) disease duration of 29 (3.8) months. the patients were provided by outpatient clinics and hospitals of the public health network and private clinics in the city of Natal in Brazil. the SLICC/ACR DI scores for each type of organ damage, prevalence of damage within organ systems, and the association with sociodemographic variables were assessed. Disease duration was considered as the time from diagnosis until the study. Organ damage was present in 18 (33%) of the 54 patients while 36 patients (67%) had no damage. the skin (11%), renal (9%), and pulmonary (7.4%) systems were the most frequently involved, followed by the neuropsychiatric and musculoskeletal systems, premature gonadal failure, and diabetes. the most frequent individual items on the SLICC/ACR DI were scarring chronic alopecia and pleural fibrosis. No association was demonstrated between organ damage (SLICC/ACR scores) and sociodemographic and clinical variables. Early organ damage demonstrated in Brazilian patients with SLE was similar to other populations studied in the world, despite a low socioeconomic status. in contrast to reports in other studies, a cutaneous lesion was the most frequent cause of damage in our patients.Univ Fed Rio Grande Norte, BR-59020120 Tirol Natal, RN, BrazilUniversidade Federal de São Paulo, São Paulo, BrazilUniversidade Federal de São Paulo, São Paulo, BrazilWeb of Scienc

Double-blind, randomized, controlled clinical trial of clofazimine compared with chloroquine in patients with systemic lupus erythematosus

Objective. To evaluate the efficacy of clofazimine (CFZ) compared with chloroquine diphosphate (CDP) for the treatment of cutaneous involvement in systemic lupus erythematosus (SLE).Methods. A prospective, randomized, controlled, double-blind clinical trial was carried out in SLE patients with active cutaneous lesions, of whom 16 were randomized to receive CFZ at 100 mg/day and 17 received CDP at 250 mg/day for 6 months. All drugs had a similar appearance to avoid identification. Both groups received broad-spectrum sunscreens twice a day and the prednisone dose was kept stable during the study. Cutaneous lesions were evaluated by 2 blinded observers at baseline and at months 1, 2, 4, and 6.Results. Thirty-three patients were randomized to a treatment group, of whom 27 completed 6 months of treatment. the groups were homogeneous and comparable in terms of demographic and clinical characteristics. Five CFZ-treated patients and 1 CDP-treated patient (P = 0.15) dropped out due to development of severe lupus flare. At the end of the study, 12 CFZ-treated patients (75%) and 14 CDP-treated patients (82.4%) had complete or near-complete remission of skin lesions; intention-to-treat analysis showed no significant difference in the response rates between groups. Side effects, mainly skin and gastrointestinal events, were frequent in both groups, but no patients had to discontinue their treatment.Conclusion. These findings suggest that CFZ is equally as effective as CDP in controlling cutaneous lesions in SLE patients. However, we cannot exclude the possibility that the CFZ itself could be the cause of systemic lupus flare.Univ Fed Rio Grande Norte, BR-59014020 Natal, RN, BrazilUniversidade Federal de São Paulo, São Paulo, BrazilUniversidade Federal de São Paulo, São Paulo, BrazilWeb of Scienc