Use of nailfold capillaroscopy for the early diagnosis of systemic sclerosis in patients with primary biliary cholangitis.

Background: Primary biliary cholangitis (PBC) is a cholestatic autoimmune disease and is often associated with systemic sclerosis (SSc). The prevalence of SSc in PBC patients ranges from 1-22% and the diagnosis is often delayed. The aim of this study was to evaluate the role of nailfold capillaroscopy (NFC) for early SSc diagnosis in PBC patients.

Methods: In this monocentric, cross-sectional study, NFC was performed in 56 PBC patients. Raynaud's phenomenon (RP) was assessed in each patient. Patients with major NFC abnormalities and those with a scleroderma pattern were screened for SSc-specific antibodies. The SSc diagnosis was established using the 2013 American College of Rheumatology and European League Against Rheumatism (ACR/AULAR) and Very Early Diagnosis Of Systemic Sclerosis (VEDOSS) criteria.

Results: NFC abnormalities were found in 31 patients (55%): 11 (20%) presented minor abnormalities, 17 (30%) had major abnormalities and 3 (5%) presented a scleroderma pattern. RP was found in 12 patients. Two patients with a scleroderma pattern were newly diagnosed with SSc. All patients newly diagnosed with SSc presented RP. No patient without RP was diagnosed with SSc.

Conclusions: Performing NFC in PBC patients can help anticipate the SSc diagnosis. RP should always be checked in PBC patients and should be an indication to perform NFC for early SSc diagnosis. A diagnostic algorithm is proposed.