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Raynaud's disease: pathophysiology, diagnosis, and treatment.

Raynaud's disease is a common vasospastic disorder affecting the digits of both hands. Women are most commonly affected. This disorder occurs in two forms: Raynaud's disease and Raynaud's phenomenon. Raynaud's phenomenon is associated with a secondary etiology, most commonly scleroderma. Symptoms may precede the onset of connective tissue disease by a number of years. The pathophysiology, differential diagnosis, recommendations for referral, and treatment of Raynaud's disease are presented. A protocol for use by the nurse practitioner in the primary care setting is provided.

Diagnosis, Differential↗

Long-term monitoring of the effects of thymoxamine hydrochloride tablets in the management of patients with Raynaud's disease.

Seventeen patients with Raynaud's disease were followed whilst receiving treatment with 40 mg thymoxamine hydrochloride 4-times daily for period ranging between 11 and 19 months. Digital artery patency and blood flow changes, assessed by Doppler ultrasound techniques after different thermal stresses, were monitored regularly as were platelet aggregation to ADP and collagen, platelet adhesion to glass beads, measures of blood coagulation and fibrinolysis, and plasma viscosity. Significant clinical improvement noted at 1 month appeared to improve further at 3 months and was maintained thereafter. Vessel patency rates at 10 degrees C and 21 degrees C improved significantly during treatment and both collagen- induced aggregation and platelet retention were significantly inhibited. These unexpected effects on platelet function are not readily explained by the drug's documented activity as a selective alpha-adrenergic antagonist and they may represent other hitherto unrecognized pharmacological effects which merit further exploration. The study also confirmed the usefulness of Doppler techniques for continuous atraumatic evaluation of digital vessel patency and investigation of therapeutic regimens on intermittent digital artery obstruction in Raynaud's disease.

Adult↗

Role of cold and emotional stress in Raynaud's disease and scleroderma.

Research on the aetiology of Raynaud's disease and phenomenon has been hindered by the difficulty of provoking attacks in the laboratory. A study was therefore conducted in which digital and ambient temperatures, electrocardiograms, and stress ratings were obtained during ambulatory monitoring in patients with idiopathic Raynaud's disease, Raynaud's phenomenon secondary to scleroderma, and in normal subjects. In Raynaud's disease about one third of the vasospastic attacks were associated with tachycardia and increased stress ratings without declines in ambient temperature. In contrast, cold alone was enough to provoke most attacks of Raynaud's phenomenon in scleroderma. Chronically increased stress ratings in patients with scleroderma and increased muscle tension in anticipation of a cold stimulus suggest that these patients have different patterns of stress responses from those with Raynaud's disease.

Cold Temperature↗

Induced vasodilation as a home treatment for Raynaud's disease.

Ten patients with Raynaud's disease treated themselves at home using induced vasodilation 3 times/day, every other day, for 18 treatment days. The patients, dressed in indoor clothing, immersed both hands in warm tap water (43-45 degrees C) for 8-10 min while exposed to naturally occurring ambient cold. Results of pre- and posttreatment cold exposures showed a significant mean increase in digital temperature of 3.4 degrees C (p less than .001). Conditioning therapy appears to be an effective, feasible alternative to drug or surgical therapy.

Adult↗

[Heredity and genetic aspects of Raynaud's disease].

The pathophysiology of primary Raynaud's phenomenon (Raynaud's disease) remains uncertain but the transmission of this primary microcirculatory dysregulation seems strongly influenced by genetic factors. For a long time, physicians have found that the hereditary factor plays an important role in the genesis of Raynaud's disease. Familial analysis and twin studies have confirmed the role of an hereditary factor. It seems heterogeneous but pedigree analysis indicates the possibility of an autosomal dominant transmission influenced by sex, in some families, allowing an approach called "reverse genetic" based on linkage analysis. Such an approach has focused on few loci but sequencing of candidate genes for genetic mutations remains negative. Given the supposed heterogeneity of the genetic transmission of Raynaud's disease, diversification of strategies in molecular genetics is suitable with reference to techniques applied to multifactorial heredity.

Female↗

Lack of habituation of the pattern of cardiovascular response evoked by sound in subjects with primary Raynaud's disease.

1. The vasospasm of primary Raynaud's disease can be triggered by acute emotional stress. We have studied the pattern of cardiovascular response evoked by acute emotional stress, a sound stimulus of 90 dB, 2 kHz for 30 s, in eight subjects with primary Raynaud's disease and in eight age- and sex-matched controls, the sound being repeated five times on each of days 1, 3 and 5. 2. In controls, the first sound evoked the pattern of the alerting response that is characteristic of acute emotional stress: a rise in arterial pressure and heart rate, a decrease in vascular conductance in the cutaneous circulation of the digit, assessed by laser Doppler recording of erythrocyte (red cell) flux in the digit divided by arterial pressure, and an increase in forearm muscle vascular conductance, assessed from forearm blood flow recorded by venous occlusion plethysmography divided by arterial pressure. 3. In the subjects with primary Raynaud's disease, baselines of arterial pressure, digital cutaneous vascular conductance and forearm vascular conductance were not significantly different from those of the controls and they too showed the alerting response to the first sound, the magnitudes of the changes being comparable to those of the controls. 4. In both the controls and subjects with primary Raynaud's disease, the evoked responses were consistent on repetition of the sound on day 1. In contrast, judging from the means of the changes evoked on each day, the controls showed habituation of the individual components of the alerting response over days 1, 3 and 5, whereas the subjects with primary Raynaud's disease showed no habituation of either the forearm muscle vasodilatation or the digital vasoconstriction. Conversely, the decrease in digital cutaneous vascular conductance evoked by a single deep breath was fully reproducible in both controls and subjects with primary Raynaud's disease when tested at the beginning and end of each experimental day. 5. These results allow the novel conclusion that subjects with primary Raynaud's disease have an abnormality of the central neural modulation of the brain stem areas that integrate the cardiovascular components of the alerting response to acute emotional stress, such that habituation of the vasodilator and vasoconstrictor components of the response on repetition of the stimulus is impaired. We propose that such persistence of vasoconstrictor responses to stressful stimuli predisposes to vasospasm, particularly if neurally mediated vasoconstriction is reinforced by locally released vasoconstrictor factors.

Adult↗

Raynaud's phenomenon as an early sign of connective tissue diseases.

Raynaud's phenomenon (RP) is seen in 5-10% of the population and generally follows an indolent course. In some cases it may be the first sign of a connective tissue disease (CTD). In patients referred to the clinician because of their RP, obviously the more severe cases, CTD, in particular scleroderma, will develop, however, in a considerable percentage. Risk factors for the evolution of CTD are (a) the severity of RP at onset, (b) a positive test for antinuclear antibodies, (c) nailfold capillary abnormalities, and (d) older age at onset. Patients with RP who are at risk for the development of CTD should be followed in order to diagnose (and treat) a developing CTD as early as possible.

Antibodies, Antinuclear↗

Biofeedback as therapy in Raynaud's disease.

The following dimensions of Raynaud's disease are reviewed: (a) etiological factors, particularly those of a psychological nature, (b) proposed biological mechanisms of vasospastic episodes, (c) efficacy of pharmaceutical and surgical interventions, and (d) use of biofeedback as therapy. Emotional stress appears to be wholly sufficient to induce vasopastic episodes in victims. Some authors further hypothesize that suppressed anger may be involved in the phenomenon. Vehicles proposed to account for the symptoms include local vascular pathology, sympathetic discharge, and biochemical phenomena. Sympathectomy does not produce permanent remission and vasodilator medications tend to have undesirable side effects even when successful. Documented success with feedback techniques is sparse but encouraging. Published outcome studies are often confounded by the use of additional treatment modes such as relaxation training and hypnosis. The study of Raynaud's disease has enormous potential for the understanding of psychosomatic illness in a holistic fashion. Stresses induce immediate physiological responses, and corrective conditioning is easily implemented and monitored.

Adult↗

Reserpine treatment of Raynaud's disease.

Six patients with primary Raynaud's disease were investigated with magnification hand arteriography, measurement of finger systolic pressure and response to a newly devised local cooling test. They were treated with reserpine 0.5 mg injected into the brachial artery, and the clinical effects were followed by a scoring system and by the change in digital arterial tone during local cooling. The latter was registered as the change in systolic finger blood pressure measured indirectly by a finger-plethysmograph during stepwise decrease in finger temperature, until at a critical temperature complete closure of the digital arteries occurred. Shortly after injection the clinical condition improved, and closure of the digital arteries during local cooling was impeded. But within a week the cooling test showed a transient deterioration with closure of the digital arteries at the same or a somewhat higher temperature than before the reserpine injection. The clinical score followed this trend. According to animal experiments the shortlasting vascular effect of reserpine on primary Raynaud's disease might be due to depletion of the sympathetic nerve terminals for norepinephrine.

Adult↗

Raynaud's disease: patient education as a primary nursing intervention.

The terms Raynaud's disease and Raynaud's phenomenon are often used interchangeably to identify two distinct disease states that initially appear with similar symptoms but then have very different sequelae. Triggered primarily by cold weather, both conditions commonly result in a vasospastic response predominantly observed in the fingers and toes. A review of the relevant health care literature indicates that there is little, if any, information available on Raynaud's disease that would be of particular and specific interest to the nursing profession. So that they can provide appropriate patient teaching, nurses need to be familiar with the precipitating factors, signs and symptoms, evaluation methods, and treatments of Raynaud's disease. This article focuses specifically on Raynaud's disease, which affects many women but relatively few men. A historic perspective on this disease state is provided, and postulated pathophysiologic mechanisms for the onset of this condition are addressed. The patient who is experiencing symptoms of this disease may have complaints of pain and numbness particularly in the fingers. The nurse's primary role is patient education so that the onset of symptoms associated with this disease can be identified and minimized. A discussion of nursing implications with a focus on teaching is provided. A case study is presented of a patient with Raynaud's disease, which will serve to highlight and expound on key information provided throughout the text of this article.

Adolescent↗