Article Text
Abstract
Objectives To study the nailfold capillaroscopic patterns and microangiopathy evolution score (MES) and correlate with the severity of organ damage in Chinese patients with systemic sclerosis (SSc)
Methods Patients who fulfilled the 2013 ACR criteria for SSc were studied. A full physical examination was performed. Blood was taken for SSc autoantibodies, along with a full lung function test and echocardiogram. The extent of skin involvement was assessed by the modified Rodnan skin score (mRSS). Organ damage of SSc was assessed by the Medsger disease severity scale. A Nailfold capillaroscopic examination was performed by a trained nurse blinded to the medical history of the patients. The following parameters were obtained: (1) capillaroscopy patterns (early, active and late); (2) Degree of enlarged capillaries, giant capillaries, capillary haemorrhages, capillary density, disorganization of vascular array and capillary ramification assessed by a semi-quantitative method; and (3) MES score (sum of capillary density, disorganization of vascular array and capillary ramification). Correlation among the capillaroscopic patterns, individual capillaroscopic parameters and the MES with organ damage was performed by the Spearman's rank correlation test.
Results A total of 138 Chinese patients were studied (91.3% women; age 56.36±11.81 years). The median disease duration was 8.14±6.21 years. 39 (28.3%) patients had DcSSc and 99 (71.7%) had LcSSc. Anti-centromere, anti-Scl 70 and anti-RNA polymerase III antibodies were present in 28.6%, 28.5% and 5.6% of the patients respectively. Organ damage was present in all patients, most common being skin (84%), lung (79%), peripheral vascular (74%) and GI tract (46%). The median mRSS was 6 (IQR 2–12). A total of 27 patients (19.7%) had early SSc pattern on capillaroscopy, 40 (29.2%) had active pattern and 68 (49.6%) had late pattern. The median MES score was 3.02 (IQR 1.76–5.25). Patients with late SSc pattern on capillaroscopy had significantly longer disease duration and were more likely to have organ damage in the general, peripheral vascular and lung domains compared to those not having late SSc patterns. The total MES score correlated significantly with organ damage scores in the muscle (Rho 0.188; p=0.029), GI tract (Rho 0.169; p=0.048) and lung (Rho 0.265; p=0.006) domains. Regarding individual components of the MES score, capillary density correlated significantly with scores in the peripheral vascular (Rho 0.460; p<0.001), skin (Rho 0.343; p<0.001), joint/tendon (Rho 0.220; p=0.011), muscle (Rho 0.295; p=0.001), GI tract (Rho 0.188; p=0.028) and lung (Rho 0.238; p=0.015) damage domains. Enlarged capillaries correlated significantly with scores in the muscles (Rho -0.205; p=0.017) and lung (Rho -0.213; p=0.029) damage domains. Giant capillaries and microhaemorrhages correlated significantly with scores in the peripheral vascular (Rho 0.239; p=0.005 and Rho 0.228; p=0.007 respectively) damage domains. Disorganization of capillary array correlated significantly with scores in the lung (Rho 0.253; p=0.009) damage domain. Capillary ramifications correlated significantly with the scores in the kidney (Rho 0.171; p=0.048) damage domains.
Conclusions In Chinese patients with SSc, capillaroscopic patterns and components of the microangiopathy evolution score were associated with severity of organ damage.
Disclosure of Interest None declared