1066: Capillaroscopic Findings in Autoimmune Connective Tissue Diseases: Results from 20 Years of Experience in a Training Referral Center

Elvis Hysa, Carmen Pizzorni, Silvia Sammori, Emanuele Gotelli, Andrea Pogna, andrea cere, Carlotta Schenone, Veronica Gerli, sabrina Paolino, Alberto Sulli and Maurizio Cutolo, Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy

Background/Purpose: Nailfold videocapillaroscopy (NVC) allows the safe and fast detection of different microvascular patterns in patients affected by autoimmune connective tissue diseases (CTDs). The prevalence of the morphological microcirculatory alterations was retrospectively evaluated in a wide cohort of patients with Raynaud’s phenomenon secondary to a CTD at the time of the first single NVC, independently from their current treatment.

Methods: One-thousand-one-hundred-eighty-one (1181) patients affected by CTDs (1065 females, mean age 54.1 ± 16.2, mean disease duration 4.5 years ± 3) were included from 2001 to 2021. The considered CTDs, diagnosed through the classification criteria available at the time of the diagnosis, were: systemic sclerosis (SSc), undifferentiated connective tissue disease (UCTD), mixed connective tissue disease (MCTD), dermatomyositis (DM), systemic lupus erythematosus (SLE), Sjogren’s syndrome (SS) and primary anti-phospholipid syndrome (aPS) (for distribution see Table 1). The capillaroscopic parameters were collected according to the Fast Track Algorithm and distinguished between scleroderma-pattern (specific NVC alterations) and non-scleroderma patterns (non-specific NVC alterations) [1]. The presence of specific NVC findings detectable with a progressive presentation in SSc patients (“early”, “active”, “late” patterns) have been searched also in other CTDs beyond SSc. Scleroderma-like pattern was defined if the abnormalities observed in the three SSc NVC patterns were found mixed together at the same time [2]. The statistical analysis was performed with DATAtab®.

Results: Giant capillaries, capillary dilations and microhaemorrages were significantly more frequent in SSc and DM than in other CTDs (respectively in 73%, 99% and 70% of SSc patients e in 73%, 96% e 70% of DM patients). The specific abnormalities of capillary morphology, such as the ramifications (expression of neoangiogenesis) were significantly more frequent in SSc, MCTD and APS among all the CTDs (respectively, in 48%, 41% and 36% of cases). Moreover, giant capillaries and angiogenesis were also detected in MCTD (specifically, in 61% and 41% of cases). In APS patients, the most significant prevalence of microhaemorrages (50%) was observed compared with other CTDs, to the exclusion of SSc and DM being detectable in 70% of cases.

Among CTDs, the mean capillary density (1 linear mm) was significantly lower in SSc, DM and MCTD (respectively, 7.04 ± 0.18 vs 6.5 ± 0.75 vs 7.7 ± 0.48) compared with other CTDs. “Early”, “active” and “late” scleroderma patterns were detected in 34%, 38% and 16% of SSc patients whereas scleroderma-like pattern was found significantly more frequent in DM (63 %) and MCTD (37%).

Conclusion: Our data confirm that SSc and DM NVC analysis show a microangiopathy with a high prevalence of specifically diagnostic severe alterations compared with other CTDs. However, the microvascular alterations detected in other CTDs seem to further indicate that endothelial damage play an important role in their pathophysiology, worthy of frequent NVC evaluation.

References: 1. Smith V et al. Autoimmun Rev 2019 2. Cutolo M et al. Nature Rev Rheumatol 2021
Table 1. Capillaroscopic findings in autoimmune connective tissue diseases at baseline. Abbreviations: normal (NL), non-specific alterations (NSA)

Disclosures: E. Hysa, None; C. Pizzorni, None; S. Sammori, None; E. Gotelli, None; A. Pogna, None; a. cere, None; C. Schenone, None; V. Gerli, None; s. Paolino, None; A. Sulli, LABORATORY BALDACCI SPA; M. Cutolo, Bristol-Myers Squibb(BMS), Boehringer-Ingelheim, Amgen.