Background/Purpose: We evaluated  the clinical features, treatment modalities, treatment responses and prognosis of patients diagnosed with systemic lupus erythematosus (SLE). We also estimated the prevalence and incidence of SLE in Thrace region of Turkey.

Methods:

Twohundred-and-seventynine patients (260F, 19M, mean age: 38.7±11.6 years) diagnosed with SLE between 2003-2013 at our center were ret­rospectively evaluated. Clinical features, treatments and responses to various treatment modalities were recorded. In addition, the incidence and prevalence of SLE in our population were calculated. Our hospital has been the only tertiary referral center for rheumatological diseases for a mixed rural and urban population of 616000 people for >16 years (316000M, 300000F).  Our city, Edirne, is located in Thrace region in northwestern Turkey and makes borders with Greece and Bulgaria.

Results:

The mean annual incidence of SLE was 4.5/100000 (8.7/100000 in females, 0.6/100000 in males). The  overall prevalence of SLE was 45.3/100000 (86.7/100000 for females, 6.1/100000 for males). Malar rash was present in 37.9% of patients; photosensitivity, 72.4%; discoid rash, 15.6%; alopecia, 9.2%; raynaud phenomenon, 32.1%; pericarditis, 8%; pleural involvement, 16.1%; and livedo reticularis in 8.1%. The percentages for major organ involvement were as follows: neurologic involvement, 20.1%; renal involvement, 28.2%; autoimmune hemolytic anemia, 9.6%; and trombocytopenia, 14.7%. Results of positivity for autoimmune tests were ANA, 98.2%; anti-dsDNA, 46%; anti-Sm, 15.7%; anti-Ro, 31.3%; anti-nucleosome, 10.4%; anti-ribosomal P, 4.8%; RF, 15%; ANCA, 8.4%; and  antiphospholipid antibody, 17.6%.

At a median follow-up of 49 months, 13 SLE patients (10F, 3M) died. The 5-year survival was 95%, and the 10-year survival was 92%. According to Kaplan-Meier survival analysis, being male (5-year survival 77% vs. 95%, p=0.015); having pleural involvement (5-year survival 95.4% vs. 87%, p=0.011); having renal involvement (5-year survival 88.4% vs. 98.5%, p=0.014); usage of cyclophosphamide (5-year survival 87.3% vs. 96.6%, p=0.004); and an initially high SLEDAI score (>6) (5-year survival 89% vs 97.8%, p=0.005) were poor prognostic factors.

Smoking (5-year survival 89% vs. 95.4%, p=0.07) and autoimmune hemolytic anemia (5-year survival 96.2%, 85%,  p=0.058) tended to be associated with poor prognosis.

According to Cox regression analysis, pleural involvement (OR: 6.25, p=0.018), hemolytic anemia (OR: 4.1, p=0.05), renal involvement (OR: 4.8, p=0.045), and low C3 level at the time of initial diagnosis (OR: 5.1, p=0.038) were independent poor prognostic factors which influenced survival.

Conclusion: Our results revealed that the annual incidence and prevalence of SLE in northwestern Turkey were quite similar to western data. In our series, majority of patients were females. Survival was similar to data from western countries. Poor prognostic factors were renal involvement, pleural involvement, initially active disease and autoimmune hemolytic anemia.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-epidemiology-of-systemic-lupus-erythematosus-and-clinical-features-of-patients-at-a-single-center-in-northwestern-turkey/