Abstract
BackgroundTo report the predictive value of nail-fold capillaroscopy (NFC) patterns of vasculopathy for systemic sclerosis (Scleroderma; SSc) in an unselected cohort of patients with Raynaud’s phenomenon (RP).MethodsPatients referred to a tertiary SSc clinic with RP were evaluated by light/video-NFC. Clinical diagnosis, details and serology were recorded. Primary RP was defined as RP with no features of connective tissue disease (CTD)/antibody. NFC patterns were determined: normal, non-specific, ‘early’, ‘active’ or ‘late’ SSc patterns. Fulfilment of the VEDOSS or 2013 ACR/EULAR criteria for SSc was determined following NFC assessment.ResultsThree hundred forty-seven patients were referred: mean (SD) age 47 (15.2) years. On clinical review, 54 (16 %) did not have RP, 69 (20 %) had primary RP, 52 (15 %) had SSc and 172 (50 %) had secondary RP. NFC SSc pattern was detected in 80 (23 %) patients; 37/52 with SSc, 30/172 with secondary RP, 9/69 with primary RP and 4/54 with no RP. For identifying patients who met either the VEDOSS or 2013 ACR/EULAR criteria for SSc, detection of a SSc NFC pattern had a sensitivity of 71 %, specificity 95 %, positive predictive value 84 % and negative predictive value 90 %.ConclusionsThe absence of SSc NFC pattern in patients with RP or suspected CTD is very valuable in the exclusion of SSc.
Highlights
To report the predictive value of nail-fold capillaroscopy (NFC) patterns of vasculopathy for systemic sclerosis (Scleroderma; SSc) in an unselected cohort of patients with Raynaud’s phenomenon (RP)
Patient characteristics Three hundred forty-seven patients were referred to the NFC service between January 2009 and October 2013; mean (SD) age was 47 (15.2) years, 83 % were female
Considering the entire unselected cohort, the detection of a SSc NFC pattern had a sensitivity of 71 % (95 % confidence interval (CI) 61 to 80 %), specificity of 95 %, positive predictive value of 84 % and negative predictive value of 90 % for identifying patients who met the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) or 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) criteria
Summary
To report the predictive value of nail-fold capillaroscopy (NFC) patterns of vasculopathy for systemic sclerosis (Scleroderma; SSc) in an unselected cohort of patients with Raynaud’s phenomenon (RP). NFC has been shown to be helpful in the assessment of pRP and predicting progression to connective tissue disease (CTD) [6,7,8]; a meta-analysis reported any abnormal capillaroscopy pattern offered a positive predictive value of 47 % and negative predictive value of 93 % for developing CTD [9]. In patients with pRP, a SSc NFC pattern, in particular, was reported by Pavlov-Dolijanovic et al, to offer a negative predictive value of 99 % for the future development of SSc, with an odd ratio of 163 (95 % confidence interval: 97.9, 271.5) [10]
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