FRI0452 Nailfold capillaroscopy in antisynthetase syndrome (NASCAR): results of a multicenter, international study of the american and european network of antisynthetase syndrome (AENEAS)

Abstract

BackgroundAntisynthetase syndrome (ASSD) is an autoimmune disease characterised by the clinical triad arthritis, myositis, and interstitial lung disease (ILD). Despite Raynaud’s phenomenon (RP) is another typical feature of ASSD, nailfold videocapillaroscopy (NVC) assessment of these patients has been only sporadically described, without the elucidating data for clinicians.ObjectivesTo describe NVC features of ASSD patients and to investigate possible correlations with clinical and serological features of the disease.MethodsWe retrospectively analysed NVC images of 190 ASSD patients (females/males 3.76, mean age 49.7±12.8 years, mean disease duration 51.2±71.4 months, 133 anti-Jo-1 and 57 non-anti-Jo-1 positive patients). For each patient, we examined number of capillaries, giant capillaries, micro-haemorrhages, avascular areas, ramified capillaries, and the presence of scleroderma (SSc) patterns. Finally, we correlated NVC features with clinical and serological findings of ASSD patients.ResultsNVC abnormalities were observed in 62.1% of AASD patients compared with 29.3% of a group of 75 patients with primary Raynaud’s phenomenon (p<0.001). A SSc-like pattern was detected in 67 (35.3%) patients and it was associated with anti-Jo-1 antibodies (p=0.002) and also with a longer disease duration (p=0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP.ConclusionsNVC abnormalities are commonly observed in ASSD, independently to the occurrence of RP. The presence of a SSc-like pattern should let to identify a more defined ASSD subtype and prospective studies could confirm the association with clinical and serological features of ASSD.Disclosure of InterestNone declared

Nailfold capillaroscopy and autoimmune connective tissue diseases in patients from a Portuguese nailfold capillaroscopy clinic

“…A study by Manfredi A et al revealed that capillaroscopic findings (mainly capillary loss, giant, bushy and ramified capillaries) were clearly associated only to DM, while no significant difference was observed between PM and controls [35]. Recently, NFC abnormalities in ASSD were observed in up to 62% patients and scleroderma pattern was associated with positive anti-Jo1 autoantibody, but not to Raynaud's phenomenon [36]. In our IIM patients, results were in line with previous descriptions: all the DM and ASSD patients presented scleroderma pattern and all the PM's a non-scleroderma pattern.…”

The usefulness of nailfold capillaroscopy in scleroderma-spectrum disorders: a single-centre observational study

“…Furthermore, Sebastiani et al . have demonstrated capillaroscopic abnormalities in 62% of patients with antisynthetase syndrome; the pattern of scleroderma-like microangiopathy has been found to correlate with the presence of anti-Jo1 antibodies, yet not with the Raynaud’s phenomenon [ 13 ]. On the other hand, more than 40% of patients with idiopathic inflammatory myopathies have normal capillaroscopic images, and some have nonspecific changes [ 10 ].…”