Background/Purpose:   Raynaud’s phenomenon is common but can progress to definite connective tissue disease. We have observed long term follow up of an unselected consecutive cohort of isolated RP to define frequency of progression and negative predictive value of nailfold capillaroscopy (NC) and ANA testing. Cases attending a dedicated Raynaud’s clinic were stratified according to baseline assessment and observed serially. Frequency of SSc or connective tissue diagnosis at presentation and progression of nailfold capillary abnormality or development of positive ANA was determined during routine follow up.

Methods: Cases of suspected RP were referred from primary care to a dedicated Raynaud’s clinic. In addition to clinical management of Raynaud’s symptoms all cases had baseline ANA testing and nailfold capillaroscopy (NC) for stratification and diagnosis. There were also clinically evaluated for presence of connective tissue disease (CTD). Those with a SSc specific ANA were referred for evaluation comprehensive assessment in a specialized CTD clinic. In cases of isolated RP without evidence of CTD but considered at risk of progression annual review was performed. The number of cases with significant evolution was determined.

Results: Patients with definite symptoms of RP were assessed by clinical examination, autoantibody testing and capillaroscopy (n=457). Of these, 119 cases were assessed as potential secondary RP and were reviewed at least once. Mean (SD) follow up of RP cases was 2.0 (1.8) years with a range of 1 to 12 years. A total of 350/457 (76.7%) had negative ANA, an additional 3/119 (2.5%) became ANA positive during follow-up. NC confirmed 335/457 (73.3%) had normal capillaries and 7/119 (5.9%) developed abnormal NVC during follow up, none with ANA evolution. At first assessment 23/457 (5%) had SSc specific ANA positivity (centromere, Scl-70, RNApol3, U3-RNP reactivity) and these were referred to the CTD clinic for evaluation and review. Of these, 12/23 (52.2%) had abnormal NVC and 8/23 (34.8%) had clinical evidence of connective tissue disease. 4 fulfilled classification criteria for SSc (2 limited and 2 diffuse SSc). No SSc pattern ANA was observed in other patients during follow up. No cases developed defined CTD within the follow up period (ANA+/NC+). 28 ANA positive cases were reviewed and none developed NC progression to abnormal. Thus, overall the rate of significant change in these tests was low and no patients with negative ANA and normal NC progressed.

Conclusion: Feasibility and utility of systematic baseline assessment of cases presenting with isolated RP is confirmed. Routine assessment of ANA and NC is known to be useful in identifying cases of RP with SSc specific ANA, including new SSc diagnosis, and those at risk of long term progression. However, these tests are often normal and then the rate of progression is very low. In this cohort cases with negative tests did not show any clinical progression confirming that these tests have a robust negative predictive value.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/low-rate-of-progression-in-cases-of-isolated-raynauds-phenomenon-screened-by-nailfold-videocapillaroscopy-and-antinuclear-antibody-status-supports-negative-predictive-value-of-these-tests/