Childhood-onset systemic lupus erythematosus: early disease manifestations that the paediatrician must know

Silva, Clóvis A.

doi:10.1080/1744666x.2016.1195685

Cited by 34 publications

(50 citation statements)

References 22 publications

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“…A similar observation was made by Hari et al (19) that more boys were found to be affected with stage IV nephritis. The clinical and laboratory features of lupus nephritis encountered in our study are broadly in conformity with other reports (20)(21)(22)(23). It has been the classical textbook teaching that SLE results in non-erosive arthritis; however, we found two children with deforming arthritis satisfying the rhupus syndrome designation and one with SLE and cutaneous scleroderma overlap syndrome (24,25).…”

Section: Discussionsupporting

confidence: 77%

Childhood lupus nephritis: 12 years of experience from a developing country’s perspective

Samanta¹,

Nandi²,

Mondal³

et al. 2017

Eur J Rheumatol

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Objective: To assess the long-term outcome of lupus nephritis in children with systemic lupus erythematosus followed up over 12 years at a tertiary care teaching hospital in Eastern India. Material and Methods:This is a retrospective observational study of the clinicopathological presentation, management, and outcome in 46 children with lupus nephritis over a period of 12 years at a tertiary teaching hospital in Eastern India. Mortality was compared between different lupus classes and therapy groups with Kaplan-Meier analysis and log-rank testResults: The incidence of lupus nephritis was 58.97% [95% confidence interval (CI) 48.06%-59.89%] with the mean age at presentation being 10.2±2.43 years (range 5.5-14.5) years. Majority belonged to class IV (30.43%), followed by class II (26.91%), class III (23.91), and class V (8.70%). Outcome analysis of children with lupus nephritis over 12 years revealed that 24 (52.17%) achieved complete remission of disease activity, 5 attained partial remission, 4 continued to have active disease, 5 developed end-stage renal disease (ESRD), and 8 died. Overall mortality thus observed was 17.39% with septicemia in the background of ESRD being the commonest cause. No significant difference in mortality was observed between different lupus nephritis classes or therapy arm groups. Conclusion:The study throws light on various aspects of lupus nephritis and their long-term outcome patterns in children from developing countries such as India.

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Section: Discussionsupporting

confidence: 77%

Childhood lupus nephritis: 12 years of experience from a developing country’s perspective

Samanta¹,

Nandi²,

Mondal³

et al. 2017

Eur J Rheumatol

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“…We have confirmed and extended previous observation that liver involvement is rarely observed in cSLE and adult SLE patients, with a variety of clinical and laboratorial manifestations [1,[12][13][14][15][16]. The rigorous selection criteria of AIH and cSLE patients excluding viral infections, malignancies and alcohol intake was relevant to avoid other confounding etiologies of hepatitis [1,7,12,14].…”

Section: Discussionsupporting

confidence: 85%

Autoimmune hepatitis in 847 childhood-onset systemic lupus erythematosus population: a multicentric cohort study

et al. 2018

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Objective: To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: This retrospective multicenter study included 847 patients with cSLE, performed in 10 Pediatric Rheumatology services of São Paulo state, Brazil. AIH was defined according to the International Autoimmune Hepatitis Group criteria (IAHGC). The statistical analysis was performed using the Bonferroni's correction (p < 0.0033). Results: AIH in cSLE patients confirmed by biopsy was observed in 7/847 (0.8%) and all were diagnosed during adolescence. The majority occurred before or at cSLE diagnosis [5/7 (71%)]. Antinuclear antibodies were a universal finding, 43% had concomitantly anti-smooth muscle antibodies and all were seronegative for anti-liver kidney microsomal antibodies. All patients with follow-up ≥18 months (4/7) had complete response to therapy according to IAHGC. None had severe hepatic manifestations such as hepatic failure, portal hypertension and cirrhosis at presentation or follow-up. Further comparison of 7 cSLE patients with AIH and 28 without this complication with same disease duration [0 (0-8.5) vs. 0.12 (0-8.5) years, p = 0.06] revealed that the frequency of hepatomegaly was significantly higher in cSLE patients in the former group (71% vs. 11%, p = 0.003) with a similar median SLEDAI-2 K score [6 (0-26) vs. 7 (0-41), p = 0.755]. No differences were evidenced regarding constitutional involvement, splenomegaly, serositis, musculoskeletal, neuropsychiatric and renal involvements, and treatments in cSLE patients with and without AIH (p > 0.0033). Conclusions: Overlap of AIH and cSLE was rarely observed in this large multicenter study and hepatomegaly was the distinctive clinical feature of these patients. AIH occurred during adolescence, mainly at the first years of lupus and it was associated with mild liver manifestations.

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“…The most relevant issue observed by LAPR was the nonadherence of medication, in spite of high frequencies of glucorticosteroid, antimalarials, and immunosuppressive availability. Compliance is a determinant factor on cSLE outcome [26][27][28], and Brazilian Society of Rheumatology Consensus for SLE reinforced the importance to evaluate adherence in all appointments, especially for adolescents [29]. Furthermore, alcohol intake and illicit drug were also important issues evidenced in adolescents, particularly reported by LAPR in large centers.…”

Section: Discussionmentioning

confidence: 99%

Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America

et al. 2018

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To assess epidemiology and management practices of Latin America Pediatric Rheumatologists (LAPR) about childhood-onset systemic lupus erythematosus (cSLE). A cross-sectional study was performed in 288 LAPR PANLAR members based on online survey about cSLE practices. The response rate of web-based survey by LAPR was 170/288(59%) and the majority worked in university hospitals (63%). The ACR and/or SLICC classification criteria (99%) and disease activity tools (97%) were almost universally used by LAPR, whereas damage index (70%) and CHAQ (58%) instruments were less frequently used. Laboratory exams, diagnostic imaging, and biopsies were generally available (> 75%), however low availability for densitometry (66%). Drug access was excellent for the most common prescribed medications (> 75%), except for belimumab (11%). Emerging mosquito-borne diseases were also reported: dengue (20%), chikungunya (11%), and Zika (8%). Groups were further divided in two, according to the median number of cSLE patients followed by LAPR in the last year: groups A and B (≥ 25 and < 25, respectively). Frequencies of condom in combination with other contraceptive methods were significantly higher in group A than B (p = 0.01). The frequencies of reported pregnancy (p < 0.001) and non-adherence to therapy were significantly higher in group A (p = 0.023). Alcohol intake (p = 0.004) and illicit drug use (p = 0.007) were also reported more frequently by LAPR of group A in at least one cSLE patient. This first large web-based survey demonstrated an overall excellent access for diagnosis and therapy by LAPR, probably related to their high rate of practices in tertiary care of university hospitals. Adherence to therapy, pregnancy, and substance abuse was identified as major challenges in this population, particularly in larger centers.

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Childhood-onset systemic lupus erythematosus: early disease manifestations that the paediatrician must know

Cited by 34 publications

References 22 publications

Childhood lupus nephritis: 12 years of experience from a developing country’s perspective

Childhood lupus nephritis: 12 years of experience from a developing country’s perspective

Autoimmune hepatitis in 847 childhood-onset systemic lupus erythematosus population: a multicentric cohort study

Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America

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