“SCLERODERMA-LIKE” PATTERN AS A PRESENTING FEATURE OF CONNECTIVE TISSUE DISEASES

Background: The role of capillaroscopy for early diagnosis of systemic sclerosis (SSc) is well-known and pathological capillaroscopic pattern is a component of the new set of criteria for SSc (EULAR/ACR 2013). While it is also known that similar microvascular changes i.e., “scleroderma-like” could be observed in other rheumatic diseases i.e., undifferentiated connective tissue disease (UCTD), overlap syndromes, systemic lupus erythematosus (SLE), etc., the data about the time of their appearance in other rheumatic diseases different from SSc are scarce. Objectives: The aim of the study was to evaluate the prevalence of capillaroscopic features of microangiopathy in Raynaud’s phenomenon (RP) patients at the time of their first referral to rheumatology setting. Methods: 22 in- and outpatients were included in the study that were referred for consultation in our rheumatology unit in the last 6 months. Inclusion criteria were presence of RP at their first consultation or still unclear diagnosis. Presence of known rheumatic disease diagnosed at previous consultation with rheumatologist as well as signs of definite diagnosis SSc were exclusion criteria. All the patients underwent capillaroscopic examination with USB capillaroscope Dinolite (magnification 200x). Routine laboratory tests were ordered i.e., complete blood count, ESR, CRP, biochemistry as well as immunological tests. ANA test was performed in all patients while antibodies against extractable nuclear antigens, antiphospholipid antibodies or other tests were ordered depending on the clinical presentation and overall context. The patients signed an informed consent for participation of the study. Results: 12 of the examined patients were diagnosed with primary RP and their capillaroscopic examination revealed absence of microangiopathy i.e., normal pattern or non-specific changes (mainly dilated capillaries). In 7 patients the final diagnosis was UCTD and 4 of them exhibited microvascular pathology i.e. “scleroderma-like” pattern, while in 3 cases the capillaroscopic findings were non-specific. Among other patients 1 case was diagnosed with prescleroderma with “early” phase “scleroderma” pattern (according to definition of Cutolo et al., 2000 (1)), 1 case was with onset of SLE (“scleroderma-like” pattern, active phase) and in one case the microvascular pathology that included single giant capillary loop no other signs of connective tissue disease were found and the final conclusion was “suspected secondary” RP with necessity for a regular follow-up. Conclusion: In conclusion, definite features of microvascular pathology, known as “scleroderma-like” capillaroscopic pattern, could be observed as an initial pathological feature in CTD different from SSc such as UCTD and SLE and the overall diagnosis should be made in the overall context. Capillaroscopy inherits high significance in patients with UCTD, in whom clinical presentation could be obscure in the beginning and identification of microvascular capillaroscopic pathology is among the crucial signs to support the diagnosis. Future studies are necessary to delineate the role of microvascular pathology for prediction of future evolution of UCTD. REFERENCES: [1]Cutolo M, Sulli A, Pizzorni C AS. Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol. 2000;27(1):155–60. Disclosure of Interests: None declared. Citation: Ann Rheum Dis, volume 80, supplement 1, year 2021, page 1257Session: Scleroderma, myositis and related syndromes (Publication Only)