Atypical onset as predictor of poor outcome in Pediatric Systemic Lupus Erythematosus (pSLE)

Purpose

Pediatric Systemic Lupus Erythematosus (pSLE) is a multisystem, inflammatory, autoimmune disease. This study is based on the observation that there are limited data on the prognostic factors of children affected by SLE and weak data correlating the course of the disease with its onset. The primary aim of this study is to assess if atypical onset influence the severity of organ damage in pSLE.

Methods

This is a multicenter IRB-approved chart review. We enrolled all patients affected by pSLE. Medical records were reviewed focusing on clinical features at onset, intended as date of diagnosis and the following 15 days. As atypical onset we meant organ involvement present at onset of pediatric SLE described in literature http://www.pubmed.com/index, but not included in ACR criteria. The primary outcome was established to be the presence of at least 1 score of System Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for Systemic Lupus Erythematosus (SLICC/ACR). Data on 100 patients were analyzed with multivariate analysis.

Results

Our population consisted in: 68 Caucasians, 24 Afro-Americans, 5 Latin-Americans and 2 Asians. There were 79 females and 21 males. They were followed up for an average of 5.3 years. 24% of patients presented aypical clinical features at onset. At multivariate analysis a significant association with outcome variables was showed for the presence at onset of atypical manifestations (p = 0.004) and renal involvement (p = 0.027).

Conclusion

Our data suggest that the presence of renal involvement and atypical manifestations at onset influence the prognosis of patients affected from pSLE.