Paroxysmal finger haematoma (PFH) is an under-recognised vascular acrosyndrome with no epidemiological description to date. The aim of this work was to evaluate the prevalence, risk factors and ...clinical correlates of PFH in a population-based sample of subjects and to describe their semiological characteristics.
This cross-sectional study of random samples of the general population in three geographic areas of France involved 802 subjects, 548 women and 254 men, aged 18 to 84 years. The diagnosis of PFH was made from a report by the subject of a history of recurrent haematoma in the fingers with a sudden, painful and unexpected occurrence. Diagnosis of associated conditions and evaluation of lifestyle variables were obtained through standardised medical interview and examination.
A history of PFH was detected in 71 subjects, with a prevalence of 1.2% in men and 12.4% in women; there was no significant regional variation. Onset before 40 years of age was rare. Besides female sex and age, no socio-economical nor lifestyle risk factors were detected. PFH was associated with Raynaud phenomenon and a history of chilblains, but no link with any health threatening disease was found. In addition to the sudden onset of pain and hematoma, the main clinical features were a frequent digital swelling during the painful attack, and their predominant location on the volar side of the first and second phalanges of the third or second fingers of the dominant hand.
PFH is a benign phenomenon, frequently found in middle-aged women, to be classified among the vascular acrosyndromes. Patients seeking medical evaluation for this disorder should be reassured.
Background Occlusion of the ulnar artery is found in a substantial proportion of elderly patients. The aim of this study was to estimate the prevalence of ulnar artery occlusion in a sample of the ...general population of France, look for its risk factors, and evaluate its clinical correlates. Methods This study was an offshoot of a cross-sectional epidemiologic study in the general population of four locations in France (Tarentaise, Grenoble, Nyons, and Toulon). In phase I, random samples of 2000 individuals per location aged ≥18 years old were interviewed by phone for screening of Raynaud phenomenon. In phase II, subsamples of individuals were invited to a medical interview and physical examination where the presence of Raynaud phenomenon and occupational risk factors were recorded and a bilateral clinical Allen test was performed for the detection of ulnar artery occlusion. Phase II comprised 688 women and 335 men. Results In 36 men and seven women, at least one occluded ulnar artery was found. The estimated prevalence was 9.6% in men and 1.0% in women ( P < .001). The occluded artery was more often in the dominant hand of both men (8.1% vs 2.4%; P < .001) and women (0.9% vs 0.4%; P = .34). Ulnar artery occlusion was found more often in men aged >50 years (16.4%) than in younger men (1.4%; P < .001). Besides age, male sex, and dominant side, the only independent risk factor was an occupational exposure in men to repeated palmar trauma, with a significant quantitative relationship in the frequency of the impacts ( P < .001) and the duration of the exposure ( P < .001). Exposures to hand-held vibrating tools and cigarette smoking did not show a significant relationship in the multivariate analysis. Most individuals with ulnar artery occlusion did not have associated complaints; however, the diagnostic criteria for Raynaud phenomenon was validated in 13 of the 36 affected men. The association remained significant after adjusting for occupational exposure to vibrating tools. One individual reported a previous episode consistent with an attack of permanent digital ischemia. Conclusion This study confirms a substantial prevalence of ulnar artery occlusions in the general population, mostly in middle-aged and elderly men, which appears to be principally related to an occupational exposure to repeated occupational palmar trauma. Although there is a significant association with Raynaud phenomenon, most often the consequences of this occlusion remain subclinical.
We report on the preliminary evaluation of a well-designed program, Living with Lymphedema. This longitudinal cohort study assessed patients' quality of life using questionnaires. Our main objective ...was to evaluate the satisfaction of the patients and their adherence to the program. This was done using a specific questionnaire of satisfaction as well as by noting patients' adherence to the program (number of patients attending all three consultations). The secondary objective was to assess the effect of the program on the patient's quality of life. The assessment criteria were the evolution of the Medical Outcomes Study 36-Item Short Form Health Survey and EuroQol questionnaire scores between the first (C1) and third (C3) consultations.
The Living with Lymphedema program targeted all patients with lymphedema in the Grenoble (France) conurbation and within the GRANTED health care network that includes vascular medicine specialists, primary care physicians, physical therapists, and dietitians in the Alpine region of France. All studied patients were ambulatory patients. The GRANTED network took care only of the educational aspect of the disease. All patients with primary or secondary lymphedema were offered the Living with Lymphedema program, whatever their age and the location of the lymphedema (upper or lower limbs). The collection of patient data conformed to the ethical and administrative regulations of the regional health authority. Grenoble Institutional Review Board (CPP Sud-Est V; No. 5891) approval for the study was specifically obtained for this evaluation on December 24, 2012. The program was built around one-to-one consultations, group workshops, and more specialized appointments. It was complementary to the routine medical care received by the patient (not evaluated in this study). It proposed three individual “educational” consultations, seven group workshops, and two specialized consultations with a dietitian. All the consultations or workshops were led by certified professionals trained in therapeutic education.
The cohort was the 34 patients included in the program. We found a significant improvement in the physical dimension of the Medical Outcomes Study 36-Item Short Form Health Survey score (P = .01) between C1 and C3 but not for the psychic dimension. Visual analog scale scores of the ability to cope with the lymphedema showed a statistically significant improvement between C1 and C3 (P = .05). No difference was observed in adherence to compression therapy.
This therapeutic educational program showed a significant improvement in several criteria of quality of life and in the autonomy of patients with lymphedema.
Identification of an association between IRF5 rs2004640 and systemic sclerosis (SSc) has highlighted a key role for type 1 interferon (IFN). Additional functional IRF5 variants have been identified ...as autoimmune susceptibility factors. Our aim was to investigate whether IRF5 haplotypes confer susceptibility to SSc, and to perform genotype haplotype-phenotype correlation analyses.
We genotyped IRF5 rs377385, rs2004640, and rs10954213 in 1623 individuals of French European Caucasian origin. SSc patient subphenotypes were analyzed according to cutaneous subsets and for SSc-related pulmonary fibrosis.
Case-control studies of single markers revealed an association between IRF5 rs3757385, rs2004640, and rs10954213 variants and SSc. We identified an IRF5 risk haplotype "R" (p(adj) = 0.024, OR 1.23, 95% CI 1.07-1.40) and a mirrored protective haplotype "P" (p(adj) = 8.8 x 10(-3), OR 0.78, 95% CI 0.68-0.90) for SSc susceptibility. Genotype-phenotype correlation analyses failed to detect any association with a single marker. By contrast, phenotype-haplotype correlation analysis was able to detect intra-cohort association and to discriminate SSc patients with from those without the following clinical traits: "R" and/or "P" haplotypes identified diffuse cutaneous SSc (p = 0.0081) and fibrosing alveolitis (p = 0.018).
IRF5 haplotypes are more informative than single markers, suggesting that they could be helpful for risk stratification of SSc patients. Our study provides further evidence of a key role of IRF5 in SSc severity.
Introduction
Severe Raynaud's syndrome and DUs are the most prevalent manifestations of SSc peripheral microvascular disease. We tested whether treprostinil iontophoresis on the finger pad of ...patients with SSc would improve digital blood flow during hand cooling.
Methods
Eleven patients with limited cutaneous SSc underwent a double‐blinded iontophoresis of treprostinil (2.56 × 10−4 M during two hours) and placebo (NaCl 0.9%) on two finger pads. Then, the hand was inserted for 30 minutes in a fenestrated cooling box at 8°C, and skin blood flow was recorded continuously using LSCI.
Results
During the local cooling, CVC was significantly higher at the treprostinil site than at the placebo site and remained higher 30 minutes after the test.
Conclusions
In patients with SSc, digital treprostinil iontophoresis shifts skin blood flow upward during local cooling of the hand and during the initial rewarming phase. Digital treprostinil iontophoresis should now be tested in larger scale studies.
Because the natural history of primary Raynaud phenomenon (RP) is unclear, we undertook this long-term (14 years) follow-up of an epidemiologic study on RP to investigate the incidence, remittance ...rate, and transition rate toward systemic sclerosis and other scleroderma spectrum disorders in a population-based sample of subjects.
In 1988 and 1989, 296 subjects obtained from a random sample of the general population of the Alpine valley of Tarentaise (southeast France) completed a cross-sectional study on RP. Of these, 78 met the diagnostic criteria for RP (RP+). From April 2002 to March 2003, we were able to get follow-up information on 292 people (dropout rate, 1.4%). Eighteen subjects (6.1%) had died, and the remaining 274 were successfully contacted. They were first evaluated by a standardized phone interview regarding their cold sensitivity, digital color changes, and RP. If any significant medical changes related to RP and/or suggesting scleroderma were reported, these subjects were invited for a medical evaluation.
Mortality was similar in RP+ and RP− subjects, and no death was due to an RP-related condition. Seven cases of new RP were diagnosed in the RP− group, which corresponds to an annual incidence rate of 0.25%. Among the 72 RP+ subjects and the 7 subjects with a new RP available for follow-up, none developed clinical features of scleroderma. A disappearance of RP attacks for 2 winters or more was reported by 24 RP+ subjects (33%).
These results show that, in the general population, RP is most often a benign condition and may disappear in a substantial proportion of subjects.
Objectives The goal was to create a simple diagnostic scoring system able to differentiate leg symptoms related to chronic venous disorders (CVD) from those produced by other causes. Methods A ...prospective observational study was done of ambulatory or hospitalized patients from 13 private and five institutional vascular centers. Two groups of patients with leg symptoms were investigated. The CVD-positive (CVD+) patients had clinically documented CVD and significant venous reflux at duplex ultrasound examination, but no arterial, rheumatologic, or neurologic disorders. The CVD-negative (CVD−) patients had disorders of an arterial, rheumatic, or neurologic origin but no clinical or duplex ultrasound signs of CVD. Patients completed a 54-item questionnaire characterizing their symptoms. Items of the diagnostic score were selected as those associated with the highest likelihood ratio (LR) for CVD in a first series of patients. They were combined into a few criteria, the number of which, when validated in a patient, would produce the value of the score for this patient. Selection of the most relevant score was performed through the comparison of the surface area under receiver operating characteristic curves. The construct validity of this score was evaluated through a factor analysis. The diagnostic performance of the scoring system was evaluated in a second series of patients similar to the first series. Results In the construction phase, 123 CVD+ patients and 94 CVD− patients were enrolled. The validation series consisted of 92 patients (67 venous and 25 nonvenous). The most efficient scoring system was composed of four combined criteria: sensation of heavy or swollen legs (sensitivity, 0.82; specificity, 0.55; LR, 1.84); associated with itching, impatient legs, or phlebalgia (sensitivity, 0.55; specificity, 0.92; LR, 6.41); worsened by a hot environment or improved by a cold environment (sensitivity, 0.78; specificity, 0.71; LR, 2.72); not worsened by walking (sensitivity, 0.89; specificity, 0.62; LR, 2.33). The score was calculated as the number (0 to 4) of the above criteria validated by the patient, with a threshold level of >3, it was shown to have a high specificity (0.95) and a fair sensitivity (0.75) for CVD. Factor analysis showed the construct validity of the score, and consistent results were found in the validation series. Conclusion The clinical relevance of this scoring system remains to be evaluated prospectively in standard clinical conditions. However, these results already document that venous symptoms can be differentiated from leg symptoms of other origins.
Systemic sclerosis (SSc) is a multisystem disease characterized by microvascular dysfunction and excessive fibrosis. However, the relationship between these 2 features remains unclear. Endothelial ...dysfunction can be assessed by quantifying plasma asymmetric dimethylarginine (ADMA), an endogenous inhibitor of endothelial nitric oxide synthase. Matrix remodeling can be assessed by quantifying serum tissue inhibitor of matrix metalloproteinases-1 (TIMP-1). Both biomarkers are elevated in patients with SSc. Our objective was to test whether plasma ADMA is correlated with serum TIMP-1.
We enrolled 91 subjects, 39 patients with SSc, 28 patients with primary Raynaud's phenomenon (RP), and 24 healthy volunteers. Plasma ADMA concentrations were measured by liquid chromatography-tandem mass spectrometry. Serum TIMP-1 concentrations were determined by ELISA.
Mean ADMA concentrations were higher in patients with SSc (0.68 microM +/- 0.12) than in patients with primary RP or healthy volunteers (respectively, 0.56 microM +/- 0.14 and 0.62 microM +/- 0.12; p = 0.002). Median serum TIMP-1 concentrations were increased in patients with SSc compared to primary RP and healthy volunteers 12 (9-15), 11 (8-13), and 10 (7-13) nM, respectively; p = 0.05. In the SSc group, we observed a statistically significant correlation between plasma ADMA and serum TIMP-1 (r = 0.34, p = 0.035).
These data are consistent with our hypothesis of an association of endothelial dysfunction and matrix remodeling in scleroderma spectrum disorders.
Objective: Clinical classifications attempt to summarize a large amount of information in a few indices. CEAP is the most comprehensive and widely used classification of chronic venous disorders. The ...objective was to evaluate, in a routine clinical setting, the information associated with each CEAP clinical class and their ascending severity and additivity. Methods: This work was a multicenter evaluation of newly designed software dedicated to the management of venous diseases. Forty-nine angiologists from nine European countries entered a total of 872 full records of unselected patients. The data were analyzed to evaluate the informational value of each of the clinical classes and to test their ascending severity and additivity, with monovariate and multivariate statistical techniques with SPSS/PC software on the database of the 872 right lower limbs. Results: The series consisted of 700 women (80.3%) and 172 men, aged 18 to 100 years (median, 53 years). The ascending severity of the classes was shown with the statistical association of higher severity C classes with the age of the patient, a history of previous deep vein thrombosis, the diameter class of the most dilated varicose vein, venous symptoms, and the presence of a corona phlebectatica. The additivity, as measured with the Cronbach α coefficient analysis, was satisfactory in highest classes but poorer within the first three classes, and factor analysis of correspondences showed the heterogeneity of the variables that make the classification. Conclusion: The information summarized with the CEAP clinical classes shows a good ascending severity but a poorer additivity. These limitations seem to be related to the heterogeneity of the information content, which suggests some refinements of this basic tool for clinical research in the field of chronic venous disorders. (J Vasc Surg 2003;37:827-33.)
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