Background/Purpose

Systemic lupus erythematosus (SLE) is a multisystemic disease that may cause a broad spectrum of clinical and immunological manifestations.  Age at onset has been shown to effect the clinical course and outcome of the disease. Herein, we aimed to define the differences in clinical characteristics and organ damage between patients with juvenile-onset (jo-SLE) and adult-onset (ao-SLE)  SLE followed up in two tertiary referral centres.

Methods

This analysis included 935 patients 846 of whom attended the lupus outpatient clinic at Istanbul faculty of medicine between 1975 and May 2012 and 89 of whom were followed in the paediatric rheumatology outpatient clinic at Cerrahpaşa faculty of medicine between 2004 and 2013. At the time of recruitment, all patients fulfilled the ACR classification criteria for SLE. The data presented was the cumulative clinical and serological manifestations throughout the follow-up period. jo-SLE was defined as diagnosis at the age of 18 or younger according to the Paediatric Rheumatology International Trials Organization (PRINTO). Seven hundred nineteen (76.9 %) patients with ao-SLE and 216 (23.1 %) patients with jo-SLE  were examined. Demographic characteristics, clinical features, autoantibody profiles and damage data (SLICC damage index) were compared between the groups. Statistical analyses were performed using SPSS, version 17.

Results

Comparison of demographics revealed significant differences in age at onset (13.5 ± 3.5 vs 34 ± 11.3 years) and duration of disease (86.5 ± 96.2 vs 111.6 ± 83.9 months) between juvenile and adult groups respectively (p<0.05).  Of clinical symptoms, photosensitivity (71.6 vs 56.5 %), malar rash (73.6 vs 45.6 % and oral ulcers (23.1 % vs 15.4 %) were significantly more frequent in jo-SLE (p<0.05). As was previously reported, renal involvement was significantly more prevalent  in the jo-SLE affecting 53.2 % of the patients compared to patients with ao-SLE (28.9 %) (p<0.05). Autoimmune haemolytic anaemia (AIHA) did also occur more often in the jo-SLE (33.3 vs 9.5 %, p<0.05) whereas reverse was true for pleuritis (11.6 vs 18.4 %, p<0.05). Of the autoantibodies, a higher frequency of anti-dsDNA (78,7 vs 69 %), anticardiolipin IgG (31.9 vs 21 %) and IgM (36.6 vs 19.3 %) were observed in the jo-SLE group. However, there were significantly more patients with anti-Sm positivity in ao-SLE (19.6 vs 10.2  %, p<0.05). According to the SLICC damage index, renal damage was significantly more frequent in the jo-SLE (22.8 %) than the ao-SLE (8.4 %) (p<0.05). However, damage in musculoskeletal system, namely avascular necrosis was more prominent in the ao-SLE (14.1 vs 8.4 %, p<0.05). 

Conclusion

Our study confirms that clinical and serological differences exist between jo-SLE and ao-SLE. jo-SLE was associated with a higher frequency of renal involvement and damage. We also report a higher frequency of cutaneous symptoms, oral ulcers, AIHA and anti-dsDNA positivity in the jo-SLE. As renal involvement is a major predictor of prognosis and outcome, this study highlights the importance of awareness of the age of onset of SLE and supports the necessity of vigilant follow-up of this subgroup.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/comparison-of-disease-characteristics-and-organ-damage-in-patients-with-juvenile-and-adult-onset-systemic-lupus-erythematosus-in-large-cohort-from-turkey/