AB0621 From Vedoss To Established Systemic Sclerosis Diagnosis According To The New ACR/EULAR 2013 Classification Criteria: A French-Italian Capillaroscopic Survey

Abstract

BackgroundNailfoldCapillaroscopy (NC) is a diagnostic and sensitive tool in order to investigate Raynaud's phenomenon (RF) that is the hallmark of both Systemic Sclerosis (SSc) and Very Early Diagnosis Of ScS (VEDOSS) patients. Thus NC is largely used in patients with these conditions and NC specific abnormalities have become a minor criteria in ACR/EULAR SSc Criteria in 2013.ObjectivesAim of our study was to monitor NC changes in a group of 66 VEDOSS patients, enrolled in two EUSTAR center.MethodsWe selected those patients who developed SSc at the follow-up. Moreover, we compared the NC features of these patients with NC of those VEDOSS patients who did not move to SSc, in order to find out NC features, if any, suitable as predictive risk factor for SSc at the time of VEDOSS diagnosis.ResultsIn our 66 VEDOSS patients, 21 of them (19 females, mean age 55 yrs) shifted to SSc in a mean follow-up time of 31 months (range 6–60 months). Table 1 shows the main features of matched VEDOSS patients: Group A with unchanging diagnosis and Group B progressing into SSc. Table 2 shows the progression of NC abnormalities from VEDOSS to established SSc diagnosis in Group B patients.Table 1FeaturesGroup A (n=21)Group B (n=21)Age, yrs (range)50 (28–70)55 (27–77)Sex (M/F)1/202/19Disease duration, month (range)51 (14–70)50 (21–70)Follow up, months (range)–31 (6–60)Anti-centromere Ab (n)1114Anti-Scl70 Ab (n)35ANA positivity (n)72NC PatternNormal = 11 (52.3%)Normal = 8 (38.1%)Early = 7 (33.3%)Early = 8 (38.1%)Active = 2 (9.6%)Active = 4 (19%)Late = 1 (4.8%)Late = 1 (4.8%)Table 2NC ParameterNC at baselineNC at follow-up(VEDOSS diagnosis)(SSc diagnosis)Loop diameter86 μm98 μmApex width176 μm187 μmCapillary distributionSlighty irregular = 11 (52.4%)Slightly irregular = 14 (66.6%)Disorganized = 4 (19%)Disorganized = 6 (28.6%)Capillary density (n/mm2)Normal = 12 ( 57.1%)Normal = 9 ( 42.8%)Reduced = 9 (42.8%)Reduced = 12 (57.1%)Capillary enlargementEnlarged loops = 10 (47.6%)Enlarged loops = 6 (28.6%) (enlarged = 20–50 μm; giant >50 μmGiant loops = 10 (47.6%)Giant loops = 11 (52.4%)Avascular areas (n pts/%)2/9.5%6/28.6%NC Pattern (n)Early = 8 (38%)Early = 5 (23.8%)Active = 4 (19%)Active = 7 (33.3%)Late = 1 (4.7%)Late = 2 (9.5%)Score >1 (n pts)1315When we compared basal NC of Group A and Group B patients, in former VEDOSS developing SScwe found a significantly higher NC score (>1), 13 versus 5 cases (p<0.03) and a larger mean apex width, 177 μm (range 50–640) versus 104 μm (range 10–280) (p<0.005).ConclusionsOur study describes a progression of NC changes during the evolution of early SSc. Moreover we identified some NC features, such as higher NC score and larger apex width, whose presence at the very early diagnosis of SSc may suggests its development in an established disease, thus underlying the relevance of specific NC abnormalities as possible predictive risk factors.Disclosure of InterestNone declared

Nailfold Capillaroscopy in Systemic Sclerosis Patients with and without Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

“…Nailfold capillaroscopy is a non-invasive diagnostic tool in SSc, allowing clinicians to assess microvascular damage in early stages of the disease [ 10 ]. It is included in the latest classification criteria for SSc [ 11 ] as well as in the criteria for the very early diagnosis of SSc (VEDOSS) [ 12 ]. Recently, nailfold capillaroscopy has also emerged as a potential surrogate marker of SSc progression [ 13 ] as several studies have demonstrated that microvascular alterations might correlate with visceral organ involvement, particularly vascular complications such as PAH [ 13 , 14 , 15 ].…”

Evaluation of hypertension in systemic sclerosis and systemic lupus erythematosus overlap

“…In fact, RP with a positive ANA, puffy fingers and abnormal nailfold capillaroscopy that represents microcirculatory abnormalities, has a high predictive value for developing SSc. 8 However, while an SSc-pattern of nailfold videocapillaroscopy (NVC) is described, this pattern is also found in SLE. 9 In addition, in ANA positive individuals, autoantibodies to self-antigens that are not traditionally captured by current screening techniques reveal that SLE patients specifically display reactivity to many autoantigens.…”

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

“…Secondary Raynaud’s phenomenon (SRP) occurs in response to cold temperature or emotional stress, in the setting of underlying vascular disturbance, and is often associated with digital pain and ischemic ulcers [ 89 , 90 , 91 , 92 ]. However, it may occasionally lead to gangrene with tissue loss or the need for digital amputation [ 89 , 90 ].…”