Background Several studies have been carried out in systemic sclerosis (SSc) in order to correlate the microvascular changes with the clinical features and organ involvement, most of them through qualitative or semiquantitative assessment of the morphological abnormalities forming the so-called scleroderma pattern. Quantitative studies about the scleroderma pattern (SP) arecurrently scarce and none is addressed to the relationship with the characteristics of the disease.
Objectives To evaluate in a series of SSc patients the association between the type and extent of the SP with both clinical features and disease severity.
Methods Fifty-four patients (47 women, mean age 59.5 yrs, median disease duration 13.0 yrs) consecutively enrolled, underwent nailfold videocapillaroscopy. For each patient, a total of 32 images were collected (4 images were representative of the entire nailfold per each finger). All images were classified according to the following patterns: normal, nonspecific, SP (with no reduction of the capillary density, with a slight reduction of the capillary density, and with avascular areas). The videocapillaroscopic patterns were correlated with several features as sex, age, disease duration, Raynaud’s phenomenon, digital ulcers (DU). Moreover, skin, muscle, gastrointestinal, pulmonary, heart and renal involvement were considered. Finally, the overall score of disease severity was calculated.
Results A total of 1728 images were analyzed. Patients were divided into 4 groups, on the basis of the extent ofthe SP. Ingroup 1 (6 pts) the extent was less than 25%, in group 2 (5 pts) the extent was between 25-50%, in group 3 (16 pts) between 50-75%, in group 4 (27 pts) the extent was more than 75%. Group 1 and group 2 showed no significant relationship with any of the variables considered. Patients in group 3 had a significant correlation with the presence of DU (p=0.007). Group 4 showed a significant correlation with disease severity (p=0.034), history of DU (p=0.045) and the presence of active DU (p=<0.0001). The extent of SP without reduction of the capillary density was correlated with calcinosis (p=0.036) and renal involvement (p=0.025). The extent of SPwith a slight reduction of capillary density was not associated with any clinical characteristic. The extent of SP with avascular areas showed a significant relationship with disease severity (p=0.001), lung fibrosis (p=0.014), gastrointestinal involvement (p=0.014), skin involvement (p=0.037), history of DU(p=0.045), active DU (p=0.001). No association was found between patterns and disease duration.
Conclusions Disease severity in SSc patients was significantly associated with a greater extent of the SP. The correlation observed between the presence of DU and/or history of DU both with the extent of the SP and the avascular areas represents a relevant data, confirming the hypothesis of a strong relationship between the peripheral vascular involvement and the degree of nailfold vascularisation. The lack of correlation between the type and extent of the SP with the disease duration supports the concept that the severity of microangiopathy is not a characteristic of the late stages of the SSc.
Disclosure of Interest None Declared