Long-term follow-up of nailfold videocapillaroscopic changes in dermatomyositis versus systemic sclerosis patients.

To identify nailfold videocapillaroscopy (NVC) changes in patients with dermatomyositis (DM) during a 3-year follow-up and to compare the NVC findings between DM and systemic sclerosis (SSc) patients at their first visit. Retrospective study of 24 DM and 24 SSc patients, matched for age and disease duration at first NVC. Capillaroscopic patterns/scores and clinical parameters had been yearly assessed. Nineteen out of 24 DM patients (79%) showed a NVC "scleroderma-like pattern." No statistically significant variation of all the capillaroscopic scores was observed during the 3-year follow-up. By comparing DM patients with or without anti-Jo-1 positivity, no statistically significant difference of the scores of the main capillary parameters was observed at baseline between the groups. Comparing at baseline DM with SSc patients, the giant capillary and microhemorrhage scores were significantly higher in SSc than those in DM patients (p = 0.04 and p = 0.05, respectively), while capillary density, ramification (abnormally shaped capillaries, expression of angiogenesis), and disorganization scores were higher in DM patients (p = 0.05, p = 0.002, p = 0.004, respectively). The absolute number of ramified capillaries was significantly higher in DM patients (p = 0.002), while the absolute capillary number was significantly higher in SSc patients (p = 0.05) at baseline. This pilot study demonstrates, for the first time, over long-term, that the capillaroscopic manifestations of DM persist in contrast to the progressive changes described in SSc patients, and the anti-Jo-1 positivity does not seem to modify the NVC pattern.

Correlations between blood perfusion and dermal thickness in different skin areas of systemic sclerosis patients.

OBJECTIVE: To identify possible correlations between skin blood perfusion (BP) and dermal thickness (DT) in different skin areas of systemic sclerosis (SSc) patients. METHODS: Sixty-two SSc patients, according to 2013 EULAR/ACR criteria, and 62 healthy subjects (CNT) were enrolled. Skin BP was analysed by laser speckle contrast analysis (LASCA) at the level of dorsum of the middle phalanx of the third fingers, dorsal aspect of the hands and zygoma. DT was assessed by both skin high frequency ultrasound (US) and modified Rodnan skin score (mRSS) in the same above reported areas. All patients were studied also by nailfold videocapillaroscopy (NVC) to assess the proper pattern of microvascular damage ("Early", "Active", or "Late"). RESULTS: At the level of finger dorsum a statistically significant negative correlation was observed in SSc patients between skin BP and both ultrasound-DT (p=0.0005 r=0.43) and mRSS (p=0.0007 r=0.42), but not at the level of hand dorsum and zygoma. No statistically significant correlation was present between skin BP and ultrasound-DT at any level in CNT. In detail, SSc patients, compared to CNT, showed a statistically significant lower BP only at level of fingers (median PU 72.6 vs 136.1 respectively, p<0.0001) and a statistically significant higher ultrasound-DT at the level of dorsum of 3th finger bilaterally (median mm 0.9 vs 0.7, p<0.0001), dorsum of hands (median mm 0.9 vs 0.7, p<0.0001) and zygoma (median mm 0.8 vs 0.7, p<0.0001). A significant positive correlation between ultrasound-DT and mRSS was observed in SSc patients at level of the three areas (dorsum of fingers p<0.0001 r=0.51; dorsum of hands p=0.03 r=0.27; zygoma p=0.0001 r=0.45). A progressive decrease of skin BP and increase of ultrasound-DT was found correlated with the progression of the severity of NVC patterns. CONCLUSIONS: This study demonstrates for the first time in SSc patients a significant inverse relationship between skin BP, measured by LASCA, and DT, evaluated by both US and mRSS, at the level of dorsum of the middle phalanx of the third fingers.

Sexual distress, sexual dysfunction and relationship quality in women with systemic sclerosis: correlation with clinical variables.

To assess the rate of sexual distress, sexual dysfunction and relationship quality and their association with clinical variables in women with systemic sclerosis (SSc), 102 sexually active women with SSc were recruited. Sexual distress, sexual dysfunction and dissatisfaction with relationship quality were investigated by Female Sexual Distress Scale Revised (FSDS-R), Female Sexual Function Index (FSFI) and Dyadic Adjustment Scale (DAS), respectively. The patients underwent medical examinations and nailfold videocapillaroscopy (NVC). Of the 102 patients, 37 (36%) reported sexual distress with FSDS-R score >11, 45 (44%) had sexual dysfunction with FSFI score <19 and 49 (48%) were not satisfied with relationship quality with DAS score <100. There was a negative correlation (p<0.001, R= -0.30) between FSDS-R and FSFI. No correlation was found between FSDS-R and DAS. FSFI showed a positive correlation with DAS (p<0.0001, R= 0.36). Age correlated negatively (p<0.05, R= -0.26) with FSFI, while FSDS-R and DAS did not correlate (p>0.05) with age. SSc women with digital ulcers (DU) had a reduction of FSFI and DAS compared with women without DU. In patients with late capillaroscopic pattern, mean value of FSFI was significantly lower than the other two capillaroscopic patterns. DAS decreased with progression of capillaroscopic damage. In a high percentage of women with SSc FSDS-R was increased, while FSFI and DAS were reduced. Age correlated negatively with FSFI, while skin score showed a negative correlation with DAS. Digital vascular damage negatively influenced FSFI and DAS.