THU0535 ARE THERE DISCRIMINATING FEATURES BETWEEN “SCLERODERMA” AND “SCLERODERMA-LIKE” CAPILLAROSCOPIC PATTERN?
Authors: S. Lambova, U. Müller-Ladner
Journal: Annals of the Rheumatic Diseases (2020)

Background: The “scleroderma” type capillaroscopic pattern is a diagnostic criterion of the EULAR/ACR scoring system for systemic sclerosis (SSc). In addition, the validated staging system of Cutolo et al. is used that categorizes the capillaroscopic changes into an “early”, ”active” and ”late” phase. A “scleroderma-like” capillaroscopic pattern can also be observed in a number of rheumatic diseases, i.e., dermatomyositis (DM), systemic lupus erythematosus (SLE), undifferentiated connective tissue diseases, overlap syndromes, and rheumatoid arthritis (RA). Objectives: To evaluate the categories “early”, “active” and “late” in “scleroderma-like” pattern in rheumatic diseases different from SSc and to assess the presence of discriminating features between “scleroderma” and “scleroderma-like” capillaroscopic pattern. Methods: 544 capillaroscopic images that showed a “scleroderma” and “scleroderma-like” pattern have been analysed from the following groups: 405 images from 42 SSc patients, 66 images from 4 patients with DM, 37 images from 9 RA patients and 36 images from 5 SLE patients. Results: 30 of the images obtained from SSc patients demonstrated an “early” phase capillaroscopic pattern, 284 an “active” phase, and 29 a “late” phase. In 62 images, neoangiogenesis could be observed in images from an “active” phase capillaroscopic pattern that could be classified as “active-to late stage of transition”. Among the 66 images from DM patients, 43 capillaroscopic pictures revealed an “active” phase and 23 - neoangiogenic capillaries with giant capillary loops, capillary loss and derangement (“active neoangiogenic” pattern). An “early” and ”late” phase capillaroscopic pattern was not present in this group. The images from SLE patients (n=36) could be classified into the following groups: 3 images “early” phase, 29 images “active” phase, and 4 images with neoangiogenesis during the active phase. A “late” phase capillaroscopic pattern was not observed. In the group of capillaroscopic pictures from RA patients (n=37), an “early” phase changes could be observed in 11 images (8 out of 9 patients) and an “active” phase in 3 images (2 patients). 23 of the images from RA patients demonstrated evidence of neoangiogenesis associated with mild capillary derangement, moderate capillary loss, and single giant capillaries (“advanced neoangiogenic” pattern). Conclusion: In conclusion, an “early” phase “scleroderma” pattern is present in RA and SLE patients, but obviously not in DM patients. An “active” phase “scleroderma” pattern was found in all three patients groups other that SSc i.e., DM, SLE and RA. In DM, profound neoangiogenesis is also a characteristic finding. In RA, advanced neoangiogenesis with moderate devascularization and single giant capillaries could also be documented. A classic “late” phase “scleroderma” pattern was found only in SSc patients and was not observed in other rheumatic diseases i.e., SLE, RA, DM. The results of the current study suggest presence of differences between “scleroderma” and ”scleroderma-like” capillaroscopic pattern that may reflect different pathogenic mechanisms of microvascular damage. Disclosure of Interests: Sevdalina Lambova: None declared, Ulf Müller-Ladner Speakers bureau: Biogen