Purpose To provide an updated, comprehensive assessment of the burden and severity of SLE manifestations at the community level.
Methods Data were retrieved from the Cretan Lupus Registry, which includes adult patients with SLE who are regularly followed in Crete. SLE is defined as mild, moderate or severe, based on the BILAG glossary of the severity of disease manifestations and the use of potent immunosuppressive drugs. Organ damage was assessed through the SLICC/ACR Damage Index [SDI].
Results A total 737 SLE patients (98% with ≥4 ACR-1997 criteria, 74% fulfilling both ACR-1997 and SLICC-2012 criteria) were included in the present analysis, with a median (interquartile range) age at diagnosis of 43 (21) years and disease duration of 8 (7) years. Regarding disease severity, 49% of the patients had mild, 32% moderate and 19% severe lupus. Within the severe cases, most frequently afflicted systems were the haematological (4.6%), renal (3.6%), cardiovascular system (2.8%), and neurological (2.3%). Mycophenolate was administered in 0%, 2.7% and 8.5%, and rituximab in 0%, 7.1% and 15.3% of patients with mild, moderate and severe disease, respectively (p<0.01 for both). Disease severity did not differ according to age of SLE diagnosis (before or after 50 years), whereas female predominance was more pronounced in mild cases (31:1) as compared to moderate (12:1) or severe (5:1) (p<0.001). Notably, more severe disease correlated with shorter time interval from symptoms onset to SLE diagnosis (delay >12 months: 56% in mild, 39% in moderate, 27% in severe, p<0.001). Unemployment and smoking status (n=399 patients) tended to be higher in the moderate/severe group (54% versus 44% and 34% versus 28%, respectively). Regression analysis showed that moderate/severe as compared to mild disease is strongly associated (odds ratio 2.5, p<0.001) with organ damage accrual (SDI>0).
Conclusions At the community level, more than half of SLE patients present with moderate or severe manifestations which may contribute to irreversible organ damage.
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