Ischemic digital ulcers (DU) are frequent and severe complications of systemic sclerosis (SSc). The purpose of our study was to assess the effect of DU on hand disability and pain in patients with ...SSc.
The Evaluation of the Impact of Recurrent Ischemic DU on Hand Disability in Patients with SSc (ECLIPSE) is a prospective, multicenter, noninterventional study with a 2-year followup. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan therapy were included between October 2009 and March 2011. This cohort is described at the time of inclusion.
There were 190 patients (132 females) from 53 centers. Mean age ± SD was 43 ± 15 years at SSc diagnosis and 53 ± 15 years at inclusion. In 105 patients (56.2%), DU were the first non-Raynaud symptoms of SSc. The mean time interval between the occurrence of Raynaud phenomenon and the first DU episode was 6.6 ± 9.1 years. The mean numbers of active DU and fingers affected per patient for both hands were 2.3 ± 1.8 and 2.2 ± 1.6, respectively. Presence of active DU at inclusion was significantly associated with pain and impaired hand function: Visual Analog Scale for pain (0 to 10) was 6.2 ± 2.6 versus 2.5 ± 2.4 (p < 0.0001) and Cochin Hand Function Scale for hand disability (0 to 90) was 38 ± 20 versus 25 ± 19 (p < 0.0001), respectively.
DU represent a major sign of SSc, often affecting multiple fingers and both hands. They are significantly associated with pain and hand disability.
Background Apart from compression therapy, physical therapy has scarcely been evaluated in the treatment of chronic venous disorders (CVDs). Spa treatment is a popular way to administer physical ...therapy for CVDs in France, but its efficacy has not yet been assessed in a large trial. The objective was to assess the efficacy of spa therapy for patients with advanced CVD (CEAP clinical classes C4-C5). Methods This was a single-blind (treatment concealed to the investigators) randomized, multicenter, controlled trial (French spa resorts). Inclusion criteria were primary or post-thrombotic CVD with skin changes but no active ulcer (C4a, C4b, or C5). The treated group had the usual 3-week spa treatment course soon after randomization; the control group had spa treatment after the 1-year comparison period. All patients continued their usual medical care including wearing compression stockings. Treatment consisted of four balneotherapy sessions per day for 6 days a week. Follow-up was performed at 6, 12 and 18 months by independent blinded investigators. The main outcome criterion was the incidence of leg ulcers at 12 months. Secondary criteria were a modified version of the Venous Clinical Severity Score, a visual analog scale for leg symptoms, and the Chronic Venous Insufficiency Questionnaire 2 and EuroQol 5D quality-of-life autoquestionnaires. Results Four hundred twenty-five subjects were enrolled: 214 in the treatment group (Spa) and 211 in the control group (Ctr); they were similar at baseline regarding their demographic characteristics, the severity of the CVD, and the outcome variables. At 1 year, the incidence of leg ulcers was not statistically different (Spa: +9.3%; 95% confidence interval CI, +5.6 - +14.3; Ctr: +6.1%; 95% CI, +3.2 - +10.4), whereas the Venous Clinical Severity Score improved significantly in the treatment group (Spa: −1.2; 95% CI, −1.6 - −0.8; Ctr: −0.6; 95% CI, −1.0 - −0.2; P = .04). A significant difference favoring spa treatment was found regarding symptoms after 1 year (Spa: −0.03; 95% CI, −0.57 - +0.51; Ctr: +0.87; 95% CI,+0.46 - +1.26; P = .009). EuroQol 5D improved in the treatment group (Spa: +0.01; 95% CI, −0.02 - +0.04) while it worsened (Ctr: −0.07; 95% CI, −0.10 - −0.04) in the control group ( P < .001). A similar pattern was found for the Chronic Venous Insufficiency Questionnaire 2 scale (Spa: −2.0; 95% CI, −4.4 - +0.4; Ctr: +2.4; 95% CI, +0.2 - +4.7; P = .008). The control patients showed similar improvements in clinical severity, symptoms, and quality of life after their own spa treatment (day 547). Conclusions In this study, the incidence of leg ulcers was not reduced after a 3-week spa therapy course. Nevertheless, our study demonstrates that spa therapy provides a significant and substantial improvement in clinical status, symptoms, and quality of life of patients with advanced venous insufficiency for at least 1 year.
Clinical analysis of the corona phlebectatica Uhl, Jean-François, MD; Cornu-Thenard, André, MD; Satger, Bernadette, MD ...
Journal of vascular surgery,
2012, 2012-Jan, 2012-01-00, 20120101, Volume:
55, Issue:
1
Journal Article
Peer reviewed
Open access
Background The corona phlebectatica (CP) is classically described as the presence of abnormally visible cutaneous blood vessels at the ankle with four components: “venous cups,” blue and red ...telangiectases, and capillary “stasis spots.” Previous studies showed that the presence of CP is strongly related to the clinical severity of chronic venous disorders (CVD) and the presence of incompetent leg perforators. The aim of this study was to select the most informative components of the CP in the assessment of the clinical severity of CVD patients. Methods A multicentric series of 262 unselected patients (524 limbs) consulted for CVD were clinically evaluated using a standardized form to record the CEAP “C” items and the presence of the four CP components. Standard categorical and ordinal statistics were used to describe the external validity of the CP components as severity indexes, taking the “C” classes as reference. Results “Stasis spots” ( P < .001; r = .44) and blue telangiectases ( P < .01; r = .32) were linearly associated with the ascending order of “C” classes, whereas the relationship is less clear for the red telangiectases and the “venous cups.” The association pattern of the four components showed that only the blue telangiectases and the “stasis spots” were consistent with each other. Blue telangiectases were found more sensitive (0.91 vs 0.75) but less specific (0.52 vs 0.80) than “stasis spots” for advanced venous insufficiency (CEAP “C4-6”). Conclusion This study shows that only blue telangiectases and “stasis spots” provide valuable information in patients with CVD and deserve to be taken into account in the evaluation of such patients. Further studies are needed to show the reproducibility of this data, which we regard as essential for clinical use.
Vascular impairment, a main feature of the pathogenesis of systemic sclerosis (SSc), involves both the macro- and the microvasculature. We compared and correlated simultaneously measured skin ...microvascular and brachial artery macrovascular post-occlusive hyperemia in 3 groups: patients with SSc, patients with primary Raynaud's phenomenon (RP), and healthy volunteers.
Thirty-three healthy volunteers, 36 patients with primary RP, and 42 patients with SSc were enrolled. For each subject, brachial artery flow-mediated dilation (FMD) and cutaneous post-occlusive reactive hyperemia (PORH) were simultaneously recorded after 5-minute occlusion of the brachial artery. Local thermal hyperemia, nitroglycerin-mediated dilation (NMD), intima-media thickness (IMT), and pulse wave velocity (PWV) were also assessed.
Digital cutaneous peak PORH was altered in patients with primary RP and SSc compared to healthy controls, whereas FMD was not significantly different among all groups. We observed a correlation between digital peak cutaneous vascular conductance and brachial FMD in healthy controls (r = 0.49; p = 0.004), but not in patients with primary RP or SSc. Thermal hyperemia was altered only in patients with SSc. Brachial NMD, IMT, and PWV were not different among all groups.
We observed a loss of the correlation between brachial FMD and digital cutaneous PORH in patients with SSc and primary RP. Microvascular function is impaired in SSc, whereas brachial artery endothelial function is normal.
Digital ulcers are the most frequent vascular manifestations of systemic sclerosis (SSc). Clinical features of patients with prior or current digital ulcers have not been extensively described. This ...cross-sectional analysis of a large multicenter cohort compared the characteristics of SSc patients with prior or current digital ulcers with those never affected.
Patients with prior/current digital ulcers or never affected were identified in the cohort of SSc patients enrolled in the French ItinérAIR-Sclérodermie registry. Rodnan skin scores, pulmonary function test results, and clinical and immunological data were analyzed to identify digital ulcerassociated clinical features.
Of 599 SSc patients, 317 had prior or current digital ulcers. These patients were more frequently male, with impaired diffusing capacity for carbon monoxide (DLCO), and higher Rodnan skin scores than patients never affected by digital ulcers. In a multivariate analysis, male gender, early onset of SSc, increased duration of SSc, high Rodnan skin score, and presence of anti-topoisomerase I antibodies (anti-topo I) were associated with prior or current digital ulcers. Comparison of patients with current digital ulcers versus patients never affected indicated that affected patients had increased duration of SSc, impaired DLCO, increased Rodnan score, and younger age at onset of SSc.
Male patients with early onset SSc, more severe skin fibrosis, impaired DLCO, and anti-topo I were most likely to exhibit prior or current digital ulcers. Confirmation of these results in a prospective longitudinal study may enable identification of patients at greatest risk of developing digital ulcers, facilitating management of this disabling complication.
The cutaneous microangiopathy plays a key role in the development of the skin clinical lesions of venous insufficiency. Capillaroscopy allows a non-invasive observation of the superficial skin ...capillaries of the lower leg, which have previously been shown to be altered in patients with advanced venous disease. As it is now available in a friendly, easy to handle way through modern video devices, we report our findings in a short series of patients with C3-C5 chronic venous disorders using this technique.
A total of 21 patients with venous insufficiency (C3-C5 on at least one leg) underwent a capillaroscopic examination of both legs and pictures recorded from the sites of the most severe venous skin lesions. This was performed with a CapXview handheld video-capillaroscope (×100 magnification), allowing easy manual measurement of maximum capillary bulk diameter and capillary density.
Dramatic changes in capillary density, size, and shape were easily observed at the site of the venous skin lesions. A significant negative linear relationship was found between capillary density and the "C" classes (
= -0.45;
< 0.001). A significant negative correlation was also found between capillary density and bulk diameter (
= -0.52;
< 0.001). The area under the ROC curve for the mathematical prediction of venous skin changes by capillary density was 0.842, which shows the strength of the link between the microvascular and the clinical status.
Video-capillaroscopy allows a direct observation of the cutaneous venous microangiopathy and provides the possibility to measure capillary density which allows its quantification. This simple to use technique shows the potential for a more precise follow-up and treatment evaluation of the cutaneous consequences of venous disease, which remains to be further investigated.
Objectives Except for compression therapy, physical therapy has scarcely been evaluated in the treatment of chronic venous disorders (CVD). Spa treatment is a popular way to administer physical ...therapy for CVD in France, but its efficacy has not been evaluated yet. This study aimed to assess the efficacy of balneotherapy associated with patient education, as performed in the spa resort of La Léchère, in patients with advanced chronic venous insufficiency (CEAP clinical classes C4/C5). Methods The study was a randomized controlled trial, spa therapy being administered on top of the usual medical care. Evaluation was by a blinded independent investigator. Subjects were patients with primary or post-thrombotic CVD with skin changes but no active ulcer (C4a, C4b, or C5), living in Grenoble area, and willing to undergo a spa treatment course in La Léchère. The treated group had the three week spa treatment course in La Léchère, soon after randomization; the control group also had a spa treatment, but starting at day 365. The treatment consisted of four balneology sessions per day, six days a week during three weeks, and three educational workshops. An independent follow-up was performed in Grenoble hospital every three months for 15 months. The main outcome criterion was the severity of the skin changes, as evaluated by means of malleolar chromametry. Quality of life, as measured by the Chronic Venous Insufficiency Questionnaire 2 scale, a visual analog scale (VAS) for leg symptoms, and the occurrence of leg ulcers were used as secondary criteria. The year after spa treatment in the treated group was compared with the year before spa treatment in the control group. Results Fifty-nine subjects were enrolled (29 in the treatment group and 30 in the control group). No statistically significant difference between groups was found at study onset regarding age, sex, etiology, CEAP “C” class, and the outcome variables. After treatment, chromametry showed significantly decreased pigmentation and erythema in the treatment group compared with the controls ( P < .01). Quality of life ( P < .01) and symptoms ( P < .001) also improved significantly. These differences remained significant after one year follow-up. The control patients improved similarly after their own spa treatment (day 450). Conclusion This study shows that spa therapy, associating balneotherapy and patient education, is able to improve significantly the skin trophic changes of the CVD patients and their CVD related quality of life and symptoms. This effect is of large magnitude and remains significant one year after the treatment course.
Subgroups of capillaroscopic scleroderma landscape have been correlated with stages of SSc: two groups for Maricq's classification (slow and active), and three for Cutolo's classification (early, ...active and late). We report inter- and intra-observer agreement for these classifications as a preliminary step in the multicentre prospective SCLEROCAP study, which aims to assess the classification and single capillaroscopic items as prognostic tools for SSc.
SCLEROCAP included 385 patients. Agreement was studied in the first 100 patients, who were independently rated twice by two observers, blind to patients' characteristics; 30 of the patients were rated once by six observers. After consensus meetings, these ratings were held again. Kappa and intraclass correlation coefficients were used to assess agreement.
Interobserver agreement on 100 patients was moderate for Maricq and Cutolo classifications κ 0.47 (0.28, 0.66) and 0.49 (0.33, 0.65), respectively, and became substantial after consensus meetings 0.64 (0.50, 0.77) and 0.69 (0.56, 0.81). Intra-observer agreement between two observers was moderate to substantial: κ 0.54 (0.33, 0.75) and 0.70 (0.57, 0.83) for Maricq's classification; 0.57 (0.38, 0.77) and 0.76 (0.65, 0.87) for Cutolo's. Thirty patients were rated once by each of six observers, and agreement was moderate to substantial: κ 0.57 ± 0.10 (Maricq) and 0.61 ± 0.12 (Cutolo). Agreement was substantial for bushy, giant capillaries and microhaemorrhages, moderate for capillary density and low for oedema, disorganization and avascular areas.
The moderate reproducibility of Maricq and Cutolo classifications might hamper their prognostic value in SSc patients. Consensus meetings improve reliability, a prerequisite for better prognostic performances. A focus on giant capillaries, haemorrhages and capillary density might be more reliable.
Abstract Objectives Ischemic digital ulcers (DU) represent a major complication of systemic sclerosis (SSc). We investigated the impact of controlling the ulcerative disease on disability, pain, and ...quality of life in SSc patients receiving bosentan. Methods ECLIPSE (Study AC-052-517) is a 2-year prospective, multicenter, and observational study. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan were included between October 2009 and March 2011. Disability scores Cochin Hand Function Scale (CHFS) and Health Assessment Questionnaire Disability Index (HAQ-DI), pain scores (visual analog scale), and quality-of-life scores (SF-36) were collected at inclusion and 1 year later (primary endpoint). A controlled ulcerative disease was defined by the absence of ongoing/new DU episode between inclusion and 1-year follow-up. Results Data were available at 1 year for 120 patients out of 190 included. During follow-up, 46 (38.3%) patients experienced a new DU episode. The number of DU per patient decreased from 1.4 ± 1.8 at inclusion to 0.6 ± 1.6 ( p < 0.0001) at 1 year. Disability scores decreased from 1.0 ± 0.7 to 0.9 ± 0.7 ( p = 0.04) for the HAQ-DI and from 29 ± 20 to 25 ± 20 ( p = 0.005) for the CHFS; the pain score decreased from 4.3 ± 3.1 to 2.9 ± 2.8 ( p < 0.0001). This improvement was attributed to patients with a controlled ulcerative disease (48.3%), who significantly improved HAQ-DI ( p = 0.04), CHFS ( p = 0.04), and pain score ( p = 0.046). Conclusions In patients with SSc, control of the ulcerative disease for 1 year was associated with significant attenuation of hand disability.
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