Background Raynaud’s phenomenon (RP) is classified as primary (PRP) or secondary (SRP) depending on its association with an underlying disease. PRP can evolve to SRP when associated to connective tissue diseases, in particular systemic sclerosis, therefore a constant monitoring is necessary in these patients (1-2).
Objectives This is a capillaroscopy-based prospective study to investigate the transition from PRP to SRP in a large cohort of patients, as well as to assess the appearance of the nailfold scleroderma-pattern of microangiopathy in patients with isolated RP, negative capillaroscopy and positive antinuclear antibodies (ANA) at baseline.
Methods 2853 patients with RP were investigated by clinical interview, laboratory examinations, and nailfold videocapillaroscopy (NVC). Patients achieving a diagnosis of PRP at first visit (normal nailfold capillaroscopy, normal or negative laboratory findings, symmetric disease, absence of tissue ulceration, exclusion of an underlying disease) were yearly followed to monitor the appearance of specific morphological alterations at NVC, autoantibodies positivity, or clinical manifestations of an underlying disease. Nailfold microangiopathy was detected by NVC, and capillary abnormalities were scored and classified in the proper patterns of microangiopathy (“early”, “active” and “late“) (3-5).
Results At first visit, 797 (28%) patients out of 2853 were showing a scleroderma-pattern by NVC and a diagnosis of SRP was obtained. Among the other 2056 patients showing a normal capillaroscopy at baseline, 1718 patients were lost during follow-up or not prospectively evaluable, and 338 patients were followed for a mean time of 50±39SD months. Among these last, 160 patients (47%) were positive for ANA, and 178 (53%) were ANA negative (PRP patients). During the follow-up, the scleroderma-pattern was diagnosed by NVC in 64 (19%) patients out of 338; in particular, it was found in 49 (31%) of the ANA-positive patients (28 “early”, 5 “active”, 9 “late”, 7 like-pattern at the end of the follow-up), and in 15 (8%) of the ANA-negative patients (14 “early”, 1 “active” NVC pattern at the end of the follow-up). The time of transition from normal/not specific capillary alterations to the “early” scleroderma-pattern was 3.5±3SD years. Enlarged capillaries (mean diameter 33 micrometres, range 21-50) and mild reduction of capillary density (observed number ≥8 capillaries/mm) were found the most frequent markers at first NVC visit in those RP patients who progressed to a scleroderma pattern.
Conclusions This study demonstrates in a large cohort, that 8% of patients initially diagnosed as affected by PRP may transit to SRP. The transition from normal NVC pattern to scleroderma-pattern was observed in 19% of patients with RP, being more frequent in those with ANA positivity (31%). PRP patients showing major not-specific alterations of nailfold capillaries at first NVC, should be monitored every 6-12 months since at risk of transition to SRP.
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Disclosure of Interest: None Declared
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