Hildegard R. Maricq

Skin capillary abnormalities as indicators of organ involvement in scleroderma (systemic sclerosis), Raynaud's syndrome and dermatomyositis

Forty-four study patients with scleroderma (systemic sclerosis) (28 patients), Raynauds syndrome (13 patients) or dermatomyositis (three patients) were observed for skin capillary abnormalities by widefield microscopy and compared with three control groups of 20 subjects each: (1) patients with other rheumatic disease, (2) hospitalized patients with nonrheumatic conditions, and (3) healthy volunteers. The distinctive microvascular pattern (dilated and distorted capillary loops alternating with avascular areas) previously reported in scleroderma and dermatomyositis was observed almost exclusively in the study patients. The severity of capillary abnormalities varied among the diagnostic subgroups, and a positive correlation was found between the degree and extent of abnormal microvascular patterns and multisystem involvement. On this basis, widefield nailfold capillary observations are proposed as a simple, inexpensive, reproducible technic for making an improved early diagnosis and predicting multisystem involvement in scleroderma, Raynauds syndrome and dermatomyositis, presently a group of loosely associated and overlapping connective tissue disorders which often defy early and precise diagnosis.

Prevalence of Raynaud phenomenon in the general population: A preliminary study by questionnaire

A short questionnaire inquiring about cold sensitivity of the fingers was administered to 1752 randomly selected subjects in a probability sample drawn from the adult population of the state of South Carolina. The overall prevalence of reported cold sensitivity was approximately 10% and showed no sex or race difference. A female preponderance was revealed only after subjects exposed to vibrating tools were excluded, and then only in the white group. Estimates of the prevalence of Raynaud phenomenon were obtained using the following criteria: cold sensitive subjects reporting white and/or blue color changes: 4.6%; cold sensitivity leading to medical consultation: 3.0%; combination of the two criteria above: 1.9%.

Prevalence of raynaud phenomenon in the adult population of South Carolina

A prevalence estimate for Raynaud phenomenon among adult residents of South Carolina was based on data obtained from respondents in a statewide health survey, followed by face-to-face interviews and clinical screening for Raynaud phenomenon, using a screening procedure developed by the authors. The survey obtained 5246 personal interviews from a probability sample of over 3000 households, and 494 survey subjects participated in the clinical screening. The prevalence estimates and their standard errors were computed using survey case weights, design-based estimation, and logistic modelling techniques. The prevalence of Raynaud phenomenon among adult residents of South Carolina was determined to be 3.5%, with a standard error of 0.6%. Prevalence was higher for females (4.3%, SE = 0.7%) than for males (2.7%, SE = 0.6%). These figures are much lower than most estimates in the existing literature on Raynaud phenomenon.

Association between Cigarette and Alcohol Consumption and Raynaud’s Phenomenon

The association between alcohol and cigarette consumption and Raynauds Phenomenon (RP) was examined by using data from an American-French collaborative, cross-sectional, epidemiological study in five geographically varied regions (Charleston, South Carolina, USA; and Grenoble, Tarentaise, Nyons, and Toulon, France). Using logistic regression models that take into account the sampling weights, the association was examined stratified by gender and adjusted for age, body mass index, self-perceived health, and education. Overall, neither cigarette nor alcohol consumption showed a significant association with RP. In men, however, a V-shaped relationship between drinking and RP was observed, with mild consumption (1 to 7 drinks per week) exhibiting a protective effect over abstinence, whereas occasional (less than 1 drink per week), moderate (8 to 18 drinks per week) and heavy consumption (more than 18 drinks per week) did not. Among the participants with RP, no significant association was observed between RP attack frequencies and the amount of either alcohol or cigarette consumption. These negative findings suggest that having RP is not strongly affected by alcohol or cigarette consumption.

Different factors influencing the expression of Raynaud's phenomenon in men and women

OBJECTIVE To determine whether the risk profile for Raynauds phenomenon (RP) is different between men and women. METHODS In this cross-sectional study of 800 women and 725 men participating in the Framingham Offspring Study, the association of age, marital status, smoking, alcohol use, diabetes, hypertension, and hypercholesterolemia with prevalent RP was examined in men and women separately, after adjusting for relevant confounders. RESULTS The prevalence of RP was 9.6% (n = 77) in women and 5.8% (n = 42) in men. In women, marital status and alcohol use were each associated with prevalent RP (for marital status adjusted odds ratio [OR] 2.3, 95% confidence interval [95% CI] 1.4-3.9; for alcohol use OR 2.2, 95% CI 1.0-5.2), whereas these factors were not associated with RP in men (marital status OR 1.4, 95% CI 0.6-3.5; alcohol use OR 1.0, 95% CI 0.2-4.4). In men, older age (OR 2.3, 95% CI 1.0-5.2) and smoking (OR 2.6, 95% CI 1.1-6.3) were associated with prevalent RP; these factors were not associated with RP in women (older age OR 0.8, 95% CI 0.4-1.6; smoking OR 0.7, 95% CI 0.4-1.1). Diabetes, hypertension, and hypercholesterolemia were not associated with RP in either sex. CONCLUSION The results indicate that risk factors for RP differ between men and women. Age and smoking were associated with RP in men only, while the associations of marital status and alcohol use with RP were observed in women only. These findings suggest that different mechanisms influence the expression of RP in men and women.

Familial clustering of scleroderma spectrum disease

This is the second case report of familial scleroderma (systemic sclerosis) in South Carolina. The family includes two cases of scleroderma meeting American Rheumatism Association criteria, one of systemic sclerosis sine scleroderma, and two other cases of undifferentiated connective tissue disease with features of scleroderma spectrum disorders; there are also two cases of Raynauds phenomenon (one associated with rheumatoid arthritis), for a total of seven affected relatives. Evidence of scleroderma spectrum disorders was sought in six siblings of the two co-index cases and in 23 of the 35 offspring. Laboratory studies included antinuclear antibody determinations and typing for the following genetic markers: HLA (A, B, C, DR), complotypes, Gm and Km allotypes, and alpha-1 antitrypsin phenotypes. No common genetic markers restricted to affected members of this family were found, and no environmental exposures were detected that could explain this familial clustering of cases. This report should, however, add to the slowly accumulating information on the genetic characteristics of families at unusually high risk for scleroderma spectrum disorders. Positive antinuclear antibody tests at a titer of 1/40 or higher were present in 57 percent of the first-degree relatives of the affected cases.

Direct and indirect immunofluorescent findings in dermatomyositis

Three skin biopsies (proximal nailfold, extensor forearm and buttock) and serum samples were studied by direct and indirect immunofluorescence, immunodiffusion and radioimmunoassay techniques in 6 patients with dermatomyositis. A variety of seroligic and immunopathologic abnormalities was observed in the patients. Three of ft patients had antinuclear antibodies (ANA) of a speckled pattern mixed with a peculiar dot pattern. Epidermal intercellular deposits of immunoglobulins were seen in the nailfold biopsies of 2 patients; one of them also had IgG deposits in the cytoplasm of epidermal cells. Forearm and buttock biopsies were negative.

Prevalence, risk factors, and clinical correlates of ulnar artery occlusion in the general population

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 >or=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.

Evaluation of treatment efficacy of Raynaud phenomenon by digital blood pressure response to cooling

Our previous studies have suggested that digital blood pressure response to cooling could provide a measure of the efficacy of treatments that are administered to patients with Raynaud phenomenon (RP). This method was used on 158 primary RP patients participating in a multicenter, randomized clinical trial that compared the efficacy of sustained-release nifedi-pine with temperature biofeedback in the treatment of RP. A pill placebo and electromyography served as controls. The response to local finger cooling was measured at 30°,20°,15° and 10°C in a temperature-controlled room under standardized conditions. The results showed that, at the 15°C and 10°C local cooling temperatures, the patients in the nifedipine group had a higher mean digital systolic blood pressure, a higher relative digital systolic blood pressure (RDSP), a smaller proportion of subjects with RDSP, 70% and a smaller proportion of subjects with a zero reopening pressure than the patients in the three other treatment groups. These results were statistically significant at 10°C, the nifedipine group being significantly different from all others (p, 0.05); no significant difference was found between the three other treatment groups.

A thermal vascular test for distinguishing between patients with Raynaud's phenomenon and healthy controls

This study tested the reliability and validity of a diagnostic thermal vascular test (TVT) for patients with Raynauds Phenomenon (RP). The TVT examined digital blood pressure responses to combined cooling and occlusion and was developed as part of the Raynauds Treatment Study, a multicenter clinical trial comparing the efficacy of biofeedback and pharmacological treatment. A computerized system permitted efficient, accurate, and uniform testing at different geographical sites. A comparison of 199 patients with RP and 52 healthy controls is reported. The TVT showed a sensitivity of 79% and a specificity of 88%. Test-retest reliability was acceptable (r = .80). Addition of a psychological challenge failed to improve the discrimination between patients with RP and controls. The TVT separated patients with RP and controls as well as or better than existing tests and did so with enhanced ease of operation.