Background Systemic sclerosis (SSc) is a progressive connective disease with the prognosis largely depending on timely start of adequate therapy. Insufficient sensitivity of 1980 ACR criteria to early identification of patients at early stage of the diseases, especially, the patients with limited disease, necessitated revision of existing SSD diagnostic standards and creation of more sensitive criteria, which allow to diagnose this disease at emergence of the earliest manifestations of illness.
Objectives To compare sensitivity of new SSc criteria (2013 ACR/EULAR) with 1980 ACR criteria when used for the diagnosis at various stages of the disease.
Methods A total 302 patients with established diagnosis of SSc by experts were enrolled to the study, which have been followed up in “Nasonova Research Institute of Rheumatology” in 2007-2013. Average age of the patients was 49±13 years (range: 18-60 years), with male/female ration 1:9 (31 vs 271), diffused and limited SSD ratio1:3 (105 and 197), and disease duration from the first non-Reynaud's syndrome 8.2±7 years (range: from 6 months to 36 years). The patients underwent physical examination, wide-field capillaroscopy, lung X-ray radiography or computed tomography, echocardiographic measurements of the pulmonary artery systolic pressure, and assay for SSc-specific antibodies (anti-RNA polymerase III antibodies were not detected).
Results Based on new criteria SSc was diagnosed in 273 (90%) of patients. Skin thickening of the fingers of bath hands extending proximal to the metcarpophalangeal joints was observed in 76/25% of cases; skin thickening of the fingers in 263/87% of cases (puffy fingers in 70/23% of patients, sclerodactyly of the fingers in 192/64% of patients), fingertip lesions in 141/47% of the patients (fingertip pitting scars in 79/26%, digital tip ulcers in 20/7%, and fingertip pitting scars plus digital tip ulcers in 42/14% of the patients), telangiectasia in 134/44%, abnormal nailfold capillaries in 276/91%, pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD in 225/78% of the patients (PAH in 15/5%, ILD in 185/61%, ILD plus PAH in 35/12% of the patients), Reynaud's phenomenon in 301/99%, SSc-related autoantibodies in 185/61% of the patients (a-Scl-70 in 138/46%, ACA in 42/14%, ACA plus a-Scl-70 in 5-1.7% of the patients).
Based on 1980 ACR criteria SSc was diagnosed in 216 (72%) of the patients, and all of them met novel criteria. The use of novel criteria allowed to diagnose SSc in 57/18% additional patients.
The table shows the rate of SSc diagnosing at various stages of the disease based on two types of criteria.
Conclusions In the Russian patient population 2013 ACR/EULAR 2013 sensitivity to SSc was as high as 0.9, thus allowing to diagnose SSs in additional 18% of the patients compared to 1980 ACR criteria. Sensitivity of novel criteria maintained at 0.9 level for all stages of the disease, including the earliest stages, while 1980 ACR criteria allowed to diagnose SSc in only half of the patient with the disease duration less than 1 year.
Disclosure of Interest None declared