Raynaud's phenomenon in forestry workers in Quebec.

A survey of 1540 forestry workers in Quebec found a prevalence of Raynaud''s phenomenon of 30.5% among chain-saw users and 8.7% among nonusers. Prevalence was related directly to duration of use of the chain-saw. The mean interval (+/- the standard deviation) between the time the chain-saw was first used and the onset of Raynaud''s phenomenon was 7.8 +/- 5.6 years. After 20 years of chain-saw use over 50% of the population had Raynaud''s phenomenon. Relative risks among those using a chain-saw for more than 10 years were 3.60 for nonsmokers and 6.55 for smokers. Other factors associated with the phenomenon included a family history of nonoccupational Raynaud''s phenomenon, previous injuries to the arms, climate and type of residence during the wood-cutting season. Decreased work capacity, interference with leisure activities and changes in professional orientation often resulted from this disease. These problems should be taken into account when disability compensation is being considered.     

Cutaneous microcirculation in systemic sclerosis and response to intra-arterial reserpine.

The cutaneous microcirculation in the hand was measured in 23 patients with systemic sclerosis, 19 with Raynaud''s phenomenon and four without Raynaud''s phenomenon, and 20 controls. The patients with Raynaud''s phenomenon had a reduced basal blood flow and an exaggerated further reduction on local cold stimulation, though both were normal in patients without Raynaud''s phenomenon. Reflex-induced vascular changes by cold stimulation of the contralateral hand showed no differences between the three groups. The blood flows were similar in the affected skin of the anterior chest wall in four patients with systemic sclerosis and peripheral Raynaud''s phenomenon and matched controls. In the seven most severely affected patients 1 mg of intra-arterial reserpine produced a prompt improvement in the cutaneous microcirculation which usually lasted one to three weeks but occasionally much longer. By judicious use of repeated injection guided by measurements of the microcirculation it was possible to heal indolent ulcers of the fingers. The results indicate that vascular changes are common in systemic sclerosis but are not fundamental in the pathogenesis of the disease. More probably there is a general soft tissue abnormality that usually but not necessarily affects the vessels.     

Para-Clinical and Immunological Evaluation in Buerger's Disease as a Suspected Autoimmune Disease: Case Series

Background:Autoimmunity causes the loss of normal immune homeostasis and involves the presence of autoantibodies and inflammation. Thromboangiitis obliterans or Buerger''s disease (BD) refers to a type of vascular obstructive syndrome, with tobacco exposure accounting for disease formation and progression. However, the current understanding of autoimmunity is unclear in the context of BD, and the scientific findings are not enough to support autoimmune mechanisms. This study was aimed at investigating autoimmunity factors in patients with BD.Methods:Clinical and experimental examinations were performed on 80 patients with BD. The diagnostic work-up for autoimmunity was composed of IgM rheumatoid factor (RF), anti-nuclear antibodies (ANA), The erythrocyte sedimentation rate (ESR), anti-cyclic citrullinated peptide (CCP) antibodies, Antiphospholipid antibodies (APA), Anti-cardiolipin antibodies (ACLA), anti-double-stranded DNA (ds-DNA), and extractable nuclear antigen (ENA) profile. Immunomarkers were detected using the quantitative enzyme-linked immunosorbent assay (ELISA).Results:Raynaud''s phenomenon (84.93%), cold sensitivity (76.25%), and claudication (73.75%) were the most common symptoms in the BD patients. Also, 64.29% represented with high ANA levels and positive RF, while 42.11% were found with increased ANA and ESR levels. The ANA/RF positive BD patients had ESR> 15 mm/hr and a high prevalence of cold sensitivity, claudication, and Raynaud''s phenomenon (p> 0.05).Conclusion:There is a possibility of a non-specific autoimmune disposition among BD patients. RF and ANA could be considered for predicting disease progression.Key Words: Antibodies, Autoimmunity, Buerger''s Disease, Immune System     

Anticentromere antibody positive Sj?gren's Syndrome: a retrospective descriptive analysis

Introduction

A subgroup of patients with primary Sjögren''s Syndrome (SS) and positive anticentromere antibodies (ACA) were recognized as having features intermediate between SS and systemic sclerosis (SSc). Our goal was to describe this group clinically and serologically and define its tendency to evolve to full blown SSc.

Methods

Among 535 patients with primary SS we identified 20 ACA positive (ACA+/SS). We compared them to 61 randomly selected ACA negative SS patients (ACA-/SS), 31 ACA positive SSc patients with sicca manifestations [SSc/(+) sicca] and 20 ACA positive SSc patients without sicca manifestations [SSc/(-) sicca].

Results

Prevalence of ACA among SS patients was 3.7%. Cases and controls did not differ in sex ratio and age at disease onset. ACA+/SS patients had a lower prevalence of dry eyes, hypergammaglobulinaemia, anti-Ro and anti-La antibodies and a higher prevalence of Raynaud''s phenomenon and dysphagia compared to ACA-/SS patients. They also had lower prevalence of telangiectasias, puffy fingers, sclerodactyly, Raynaud''s phenomenon, digital ulcers and gastroesophageal reflux in comparison to both of the SSc subgroups and a lower prevalence of dyspnoea and lung fibrosis compared to the SSc/(+) sicca subgroup. Two patients originally having ACA+/SS evolved to full blown SSc. Four deaths occurred, all among SSc patients. Kaplan Meier analysis showed a significant difference between cases and controls in time from disease onset to development of gastroesophageal reflux, telangiectasias, digital ulcers, arthritis, puffy fingers, xerostomia, hypergammaglobulinaemia and dysphagia.

Conclusions

ACA+/SS has a clinical phenotype intermediate between ACA-/SS and SSc and shows little tendency to evolve to SSc.     

Attitudes among NHS doctors to requests for euthanasia.

OBJECTIVES--To explore NHS doctors'' attitudes to competent patients'' requests for euthanasia and to estimate the proportion of doctors who have taken active steps to hasten a patient''s death. DESIGN--Anonymous postal questionnaire, with no possibility of follow up. The survey was conducted from December 1992 to March 1993. SUBJECTS--All (221) general practitioners and 203 hospital consultants in one area of England. RESULTS--273 doctors responded to a question on whether a patient had ever asked them to hasten death. Of these, 163 had been asked to; 124 of these had been asked to take active steps to hasten death; 38 of 119 (32%) of these had complied with such a request (95% confidence interval 23% to 40%). This proportion represented 12% of all those who returned a completed questionnaire and 9% of all those who had been sent a questionnaire (95% confidence interval 6.3% to 11.7%). A larger proportion of the respondents (142/307 (46%)), however, would consider taking active steps to bring about the death of a patient if it was legal to do so. CONCLUSIONS--Many doctors face difficult decisions about euthanasia. For the benefit of both patients and doctors euthanasia should be discussed more openly.     

Raynaud's phenomenon and thermal entrainment: an objective test.

A new objective test for diagnosing Raynaud''s phenomenon was assessed in practice. The test is based on entrainment of the thermal vasomotor control system and entails non-invasive measurement of blood-flow responses in one hand while alternating thermal stimuli are applied to the contralateral hand. A significant (p less than 0.001) abnormality of vasomotor control was found in patients with Raynaud''s phenomenon compared with normal subjects. When applied clinically this test is diagnostic and indicates the severity of the disease and the effect of treatment.     

Treatment of Raynaud's phenomenon by fibrinolytic enhancement.

Twenty patients with advanced Raynaud''s phenomenon, in 14 of whom it was secondary to scleroderma, were treated with stanozolol, an anabolic steroid that enhances natural fibrinolysis. All showed an increase in hand blood flow and a reduction in symptoms during treatment. This response may have been caused by the lysis of fibrin deposited in the digital arteries and the reduction of plasma viscosity. Stanozolol is a useful addition to the treatment of patients with advanced Raynaud''s phenomenon who have trophic changes.     

Traumatic vasospastic disease in chain-saw operators.

Raynaud''s phenomenon is commonly induced in chain-saw operators by vibration; the hand guiding the tool is the more severely affected. The condition tends to persist after use of the chain-saw is stopped but compensation is rarely sought. Among 17 cases of Raynaud''s phenomenon in lumberjacks the condition was found to be related to use of the chain-saw in 14, 10 of whom had to give up their work in colder weather because the disease was so disabling. Two criteria essential to establish the condition as vibration-induced Raynaud''s phenomenon are the presence of symptoms for at least 2 years and a history of at least 1 year''s constant use of the chain-saw. Careful physical examination and simple tests of vascular function will provide objective evidence of permanent damage by which the patients may be classified and compensated.     

Comparison of intravenous infusions of iloprost and oral nifedipine in treatment of Raynaud's phenomenon in patients with systemic sclerosis: a double blind randomised study.

OBJECTIVE--To compare the long term effects of short term intravenous infusions of iloprost with those of oral nifedipine in patients with Raynaud''s phenomenon associated with systemic sclerosis. DESIGN--Double blind, placebo controlled, randomised group comparison. SETTING--Dermatology outpatient clinic. PATIENTS--Twenty three patients with Raynaud''s phenomenon associated with well documented systemic sclerosis (American Rheumatism Association criteria) and with typical abnormalities in fingernail folds on capillaroscopy. INTERVENTIONS--Twelve patients were randomised to receive intravenous infusions of iloprost starting at 0.5 ng/kg/min and increased by 0.5 ng/kg/min every 15 minutes to a maximum of 2.0 ng/kg/min for eight hours on three consecutive days with a further single infusion at week 8. Placebo capsules were given concurrently. Eleven patients were randomised to receive nifedipine, starting at 30 mg daily and increased to 60 mg daily after four weeks for another 12 weeks. Infusions of placebo were given in the same manner as the infusions of iloprost. One patient from each group withdrew because of social reasons and three patients receiving nifedipine withdrew because of side effects. END POINT--Reduction in number, duration, and severity of attacks of Raynaud''s phenomenon, reduction in number of digital lesions, increase in digital blood flow. MEASUREMENTS AND MAIN RESULTS--Measurements were taken at 0, 4, 8, 12, and 16 weeks. Both regimens produced a reduction in the number, duration, and severity of attacks of Raynaud''s phenomenon. The mean (SE) number of digital lesions was reduced with iloprost (from 3.5 (1.6) to 0.6 (0.3] and with nifedipine (from 4.3 (0.8) to 1.4 (0.5] after 16 weeks. Hand temperature and digital and microcirculatory blood flow were increased with iloprost but not with nifedipine. CONCLUSION--Both iloprost and nifedipine are beneficial in the treatment of Raynaud''s phenomenon. With nifedipine, however, side effects are common. Short term infusions of iloprost provide longlasting relief of symptoms, and side effects occur only during the infusions and are dose dependent.     

Treatment of Raynaud's phenomenon with the 5-HT2-receptor antagonist ketanserin.

The selective 5-hydroxytryptamine2-(5-HT2)-receptor-blocking agent ketanserin was given in a dose of 10 mg intravenously to nine patients with Raynaud''s phenomenon. The effect on blood flow was assessed by photopletysmography and measurments of skin temperature. Digital blood flow and skin temperature increased significantly after ketanserin injection, whereas the placebo (saline 9 g/l) had no such effect. This study suggests that ketanserin may be useful in the treatment of Raynaud''s phenomenon.     

The Vancouver Lymphadenopathy-AIDS Study: 1. Persistent generalized lymphadenopathy

The Vancouver Lymphadenopathy-AIDS (acquired immune deficiency syndrome) Study is an ongoing prospective study of over 700 homosexual men attending six primary care practices in central Vancouver. A case-control study of risk factors for persistent generalized lymphadenopathy in homosexual men was conducted in five of the practices. The participants completed a questionnaire and underwent a complete physical examination at the time of enrolment and at a subsequent visit not less than 3 months later, and laboratory tests were performed after both visits. Persistent generalized lymphadenopathy was defined as the presence of lymph nodes greater than 1 cm in diameter at two or more extrainguinal sites for more than 3 months. Of the 519 patients who had completed both visits by February 1984, 126 (24%) were found to have the disease, and two controls without lymphadenopathy were frequencymatched on the basis of age and practice to each subject. More than 100 male sexual partners during one''s lifetime, frequent receptive anal intercourse, a history of gonorrhea, use of illicit drugs and sexual contact in Los Angeles were identified as independent risk factors for persistent generalized lymphadenopathy. The similarity of these risk factors to those established for AIDS supports the hypothesis of a common etiology for the two diseases, and the high prevalence rate of persistent generalized lymphadenopathy further supports the hypothesis that AIDS is an uncommon response to a relatively common agent.     

Anti-Jo-1 antibody: a marker for myositis with interstitial lung disease.

An autoantibody known as anti-Jo-1 antibody is found in 25% of patients with myositis. Its prevalence in patients with both myositis and cryptogenic fibrosing alveolitis was 68% (13 out of 19 patients), compared with 7.5% in patients with myositis alone (four of 53) and 3% in patients with cryptogenic fibrosing alveolitis alone (two of 62). Anti-Jo-1 antibody may be useful in indicating patients with myositis and cryptogenic fibrosing alveolitis. Raynaud''s phenomenon, the sicca syndrome, and mild arthritis are also often part of the syndrome.     

A study of patients attending without appointments in an urban general practice.

OBJECTIVE--To ascertain which social and psychological characteristics are associated with patients attending surgeries without appointments. DESIGN--Prospective study of patients attending an urban centre group practice. SETTING--Urban health centre group practice with five doctors and 12,000 patients in an area of high (greater than 20%) unemployment and social deprivation. PATIENTS--All attenders at the open access surgery and one in four consecutive attenders by appointment, selected sequentially from the first three appointments, during 10 days in January 1989. Patients participating in the pilot study, reattending during the study period, or attending antenatal clinics were excluded. MAIN OUTCOME MEASURES--Patients'' attitude to making appointments and reasons for attending, including perception of urgency, with respect to sociodemographic and psychosocial data obtained from a self completed questionnaire before the consultation. Doctors'' diagnosis and perception of urgency obtained from a separate questionnaire. RESULTS--86% (141/172) Of patients attending without appointments and 96% (139/145) with appointments responded to the questionnaire. The need for consultation was considered to be "very urgent" or "fairly urgent" in significantly more of the open access group than the appointments group (89%, 124/139 v 66%, 91/138; chi 2 = 27.04, df = 3; p less than 0.001), although the doctors did not share the same views. Significantly more patients had self limiting conditions of recent onset in the open access than in the appointments group (75%, 101/135 v 48%, 59/123: p less than 0.001). Overall, open access attendance was significantly linked with social support (39%, 48/124 v 26%, 32/123; p less than 0.05) and with marital separations or intentions to separate (10%, 9/87 v 0/92; 47%, 32/87 v 22%, 20/92 respectively; both p less than 0.001), but the doctors recorded significantly fewer psychological and social problems in these patients (p less than 0.05). Although almost half those in the appointments group considered that making appointments was inconvenient, more of those in the open access group agreed with this view (47%, 60/129 v 61%, 80/131). CONCLUSIONS--There was an important link between social support problems and a negative attitude to making appointments. In our previous experience encouraging patients to make appointments has been unsuccessful; practices serving areas with a high prevalence of social deprivation providing a mixed open access and appointments system may better serve patients'' needs.     

Raynaud's phenomenon as side effect of beta-blockers in hypertension.

A series of 102 hypertensive patients were assessed for the frequency of symptoms of Raynaud''s phenomenon and absent peripheral pulses. Out of 21 patients receiving methyldopa alone only one had cold hands and feet whereas among patients on beta-blockers the incidence was 50%. The frequency of both symptoms and absent pulses was highest in patients taking propranolol compared with those taking atenolol or oxprenolol. Patients without a foot pulse were much more likely to have cold hands. A change from propranolol to oxprenolol in some symptomatic patients resulted in improvement. In two patients the skin temperature fell after an 80-mg dose of propranolol. The mechanism by which beta-blockers induce Raynaud''s phenomenon is still not clear.     

Diabetes registers: a grassroots approach.

OBJECTIVES--To compile a register of diabetic patients within the catchment area of a district general hospital and evaluate the characteristics of the population using aggregated data from a general practice audit. DESIGN--Cross sectional study. Practices identified all known diabetics and completed a questionnaire from information in each patient''s medical record. SETTING--Practices affiliated to a district audit group in south east England. MAIN OUTCOME MEASURES--Number of participating practices; prevalence of diabetes and its complications; and sex distribution of patients, age at diagnosis and review, treatment, and metabolic control. Comparisons with similar data from other studies. RESULTS--41 out of 43 practices participated, and 2574 diabetic patients were identified (prevalence 1.18%). 52.4% of patients were male. The mean age was 61.6 years. 32% of patients were treated with insulin, 51.5% with oral hypoglycaemic agents, 16.5% with diet alone. The mean random blood glucose concentration was 10.4 mmol/l and glycosylated haemoglobin 10.1%. 8% had proteinuria, 7% a history of myocardial infarction, 5% a history of stroke, and 2% a diabetes related amputation. These proportions were not significantly different from those found in studies performed by different methods in Poole, Islington, Powys, Trowbridge, and Southall. CONCLUSION--It is feasible to compile a register of diabetic patients in a district and evaluate their characteristics by using only general practice sources.     

Early detection and treatment of hyperlipidemia: physician practices in Canada.

We surveyed primary care physicians in Canada to determine their current practices regarding the detection and treatment of hyperlipidemia in asymptomatic adults 20 years of age or more and to determine the role of selected patient characteristics (age, sex and the presence of coronary heart disease [CHD] risk factors) in their management decisions. The self-administered questionnaire was completed by 428 of 804 family physicians and general practitioners. The proportion of physicians who reported having tested at least 50% of their adult patients varied from 29% to 85% and was related to the number of CHD risk factors present and the patient''s age. The proportion of respondents who reported starting dietary or drug therapy among patients with a cholesterol level of 6.2 mmol/L or less increased as the number of CHD risk factors increased and was not related to patient age or sex. According to the factors examined our results suggest that primary care physicians in Canada select patients for screening and treatment mainly on the basis of CHD risk factors present and that their approach is more conservative than that recommended by the Canadian and US consensus conferences.     

Exaggerated postural vasoconstrictor reflex in Raynaud's phenomenon.

The central and local regulation of capillary blood flow in the finger was studied by the local xenon-133 washout technique in women with primary Raynaud''s phenomenon, men with vibration induced white finger, and their respective sex matched controls. The vasoconstrictor response to venous stasis of 40 mm Hg elicited by local reflex was normal in both types of Raynaud''s phenomenon. Change in posture from lying to sitting induced vasoconstriction in all groups, which was abolished by proximal nervous blockade. The vasoconstrictor response to sitting was augmented in both groups of subjects with Raynaud''s phenomenon compared with their sex matched controls. These results show the existence of central and local postural vasoconstrictor reflexes in normal fingers. In both types of Raynaud''s phenomenon there was hyperreactivity of the central sympathetic nervous system to orthostatic stress and normal function of digital arterioles and postganglionic sympathetic nerve fibres.     

Can primary prevention or selective screening for melanoma be more precisely targeted through general practice? A prospective study to validate a self administered risk score.

OBJECTIVES: To establish whether a questionnaire incorporating MacKie''s risk factor flow chart can identify patients at high risk for melanoma so that they can be targeted for primary and secondary prevention. To validate the risk score derived from the questionnaire and test the feasibility of self completion by comparing patients'' self reported skin characteristics with a skin examination performed by an experienced general practitioner. DESIGN: Prospective questionnaire survey followed by a comparative study. SETTING: 16 randomly selected group practices in a health district in Cheshire, United Kingdom. SUBJECTS: Questionnaire survey--3105 consecutive patients aged 16 years and over attending for a primary care consultation; comparative study--a self selected subsample of 388 of the 3,105 patients. MAIN OUTCOME MEASURES: MacKie risk group for melanoma. Comparison of high risk skin characteristics reported by patients and those noted during a skin examination by a doctor (kappa statistic). RESULTS: 4.3% of patients (87% women) were in the highest risk group and 4.4% (79% men) were in the second highest risk group, as defined by the MacKie score. Agreement between patients'' self appraisal of skin characteristics and clinical skin examinations was reflected in kappa values of 0.67 for freckles, 0.60 for moles, and 0.43 for atypical naevi. CONCLUSION: This questionnaire helped to identify a group at high risk for melanoma. Furthermore, good agreement was found when the patient''s risk scores were compared with results of the clinical skin examination. This risk score is potentially useful in targeting primary and secondary prevention of melanoma through general practice.     

Self screening for risk of melanoma: validity of self mole counting by patients in a single general practice.

OBJECTIVE--To validate self screening by patients of high mole counts, assess the within family association of sun protection behaviour and mole counts, and estimate prevalence of risk factors for melanoma. SETTING AND SUBJECTS--Systematic sample of families from a single affluent general practice population in Wessex. DESIGN--Subjects completed a questionnaire about risk factors for melanoma and counted their moles. Subsequently a mole count was done by a general practitioner trained at dermatology clinics. MAIN OUTCOME MEASURES--Validation of self counts by observer''s count. Within family association of sun protection behaviour and mole counts; self reported risk factors. RESULTS--199/237 subjects (84%) returned the questionnaire; 212/237 (89%) were examined. High counts by patients on the front of the trunk (> 7 moles of > or = 2 mm) were reasonably sensitive (79%), predictive (75%), and specific (97%) of the observer''s mole counts (kappa = 0.74), unlike arm or total body counts. Sun protection behaviour correlated between individuals and other family members (Spearman''s coefficient r = 0.50, P < 0.01). In the past three months 15/114 adults (13.2%, 95% confidence interval 7.0% to 19.4%) reported any change in a mole and 6/114 (5.3%, 2.0% to 11.1%) "major" changes; 6/109 adults (5.5%, 2.1% to 11.6%) had both high mole counts and freckling. CONCLUSIONS--Asking patients to count trunk moles could be a feasible way of identifying patients at high risk of melanoma. Concentrating on reported major changes in moles should avoid considerable workload in general practice. The generalisability of these findings and the adverse effects, net benefit in earlier diagnosis and prevention, and workload implications of such self screening need further research.