Role of digital artery adrenoceptors in Raynaud's disease.

Raynaud's disease is characterized by excessive cutaneous vasoconstriction in response to ambient cold. A functional disturbance in the local regulation of digital vasomotion has been proposed. The purpose of this study was to determine whether there is an alteration in the postjunctional adrenergic receptors in the digital circulation of patients with Raynaud's disease. Furthermore, we sought to determine whether this abnormality was responsible for the excessive cold-induced vasoconstriction in these patients. Finger blood flow was measured by strain-gauge venous occlusion plethysmography in 10 patients with Raynaud's disease and in 10 normal volunteers in a 22 degrees C room. Measurements of finger blood flow and mean systemic arterial pressure were made during intra-arterial infusions of the alpha 1-adrenergic antagonist, prazosin, or the alpha 2-adrenergic antagonist, yohimbine, at room temperature and during local cooling of the hand. Basal finger blood flow in normal subjects was significantly greater than that of patients (8.6 +/- 2.7 vs 1.7 +/- 0.5 ml/100 ml per min; normal vs Raynaud's subjects; p < 0.05). In normal subjects, either prazosin or yohimbine induced dose-dependent increases in finger blood flow. The maximal increase in finger blood flow induced by prazosin was significantly greater than that in response to yohimbine (29.2 +/- 10.1 vs 2.8 +/- 2.1 ml/100 ml per min; prazosin vs yohimbine; p < 0.05). By contrast, in the Raynaud's patients, prazosin or yohimbine induced maximal increases in finger blood flow that were not significant (7.1 +/- 1.8 vs 5.0 +/- 2.2 ml/100 ml per min; prazosin vs yohimbine; p = NS). The response to prazosin in Raynaud's patients was significantly less than that of the normal volunteers (p < 0.05). In normal subjects, during intra-arterial infusion of vehicle alone, cooling induced a 52.6 +/- 5.8% reduction in finger blood flow. This cold-induced vasoconstriction was blunted, but not qualitatively altered, by either adrenergic antagonist. In the Raynaud's patients, during the intra-arterial infusion of the vehicle, cooling induced a 68.2 +/- 7.8% reduction in finger blood flow. Infusion of either adrenergic antagonist blunted, but did not qualitatively alter, the response to cold. Finger blood flow is less in patients with Raynaud's disease than in normal subjects when studied in a 22 degrees C room. In normal subjects, postjunctional alpha 1-adrenergic receptors appear to predominate in the control of digital vasoconstriction. Postjunctional alpha 1- and alpha 2-adrenoceptors play an equal role in adrenergic regulation of finger blood flow in patients with Raynaud's disease. In both normal and Raynaud's subjects, selective antagonism of alpha 1- or alpha 2-adrenergic receptors does not abolish local cold-induced vasoconstriction. Therefore, it is likely that a nonadrenergic mechanism contributes to local cold-induced vasoconstriction.

Association between Raynaud's phenomenon and migraine in a random population of hospital employees.

OBJECTIVE: To determine the relationship between migraine and Raynaud's phenomenon. METHODS: A detailed headache questionnaire was completed by 41 subjects with Raynaud's phenomenon identified in a random survey of hospital employees and by a group matched for age and sex without Raynaud's phenomenon. RESULTS: The prevalence of migraine was increased significantly in the group with Raynaud's phenomenon compared with the control group (24/41, 58.5% vs 10/41, 24.4%; p = 0.002). This result agrees with findings in hospital-based studies of patients with Raynaud's phenomenon. CONCLUSION: The prevalence of migraine is significantly increased in subjects with Raynaud's phenomenon, suggesting that these conditions may share a common pathogenetic defect or mechanism.

Color chart assisted diagnosis of Raynaud's phenomenon in an unselected hospital employee population.

The prevalence of Raynaud's phenomenon (RP) in 750 subjects was assessed by questionnaire, clinical interview, and use of a chart of color photographs taken of hands during attacks of RP. Eighty-three of 600 women (13.8%) and 7 of 150 men (4.7%) reported cold induced digital color changes on the questionnaire, 76 women (12.7%) and 6 men (4%) were diagnosed as having RP at an independent clinical interview, and 3 women could not be assessed using the color chart because of defective color perception. Fifty-four of the remaining 597 women (9%) and 6 of 150 men (4%) were diagnosed as having RP when a positive response to both chart and questionnaire was required. The color chart should be useful in lessening the frequency of false-positive responses and in standardizing the diagnosis for epidemiological surveys.

Increased prevalence of migraine and chest pain in patients with primary Raynaud disease.

OBJECTIVE: To investigate the prevalence of headaches and recurrent chest pain in patients with primary Raynaud disease. DESIGN: Postal survey. SETTING: Raynaud disease clinic at the Lahey Clinic Medical Center, a tertiary care hospital. PARTICIPANTS: A consecutive series of 120 patients with primary Raynaud disease who had been observed for at least 2 years, 97 of whom received and 93 of whom completed the questionnaire, and a control group of 93 age- and sex-matched hospital employees without Raynaud phenomenon. RESULTS: Migraine was diagnosed in 57 (61%) patients with primary Raynaud disease and in 21 (23%) of the control group (odds ratio, 5.4; 95% CI, 2.8 to 10.3). Migraine with aura and migraine without aura were more common in patients with Raynaud disease than in the control subjects (27% compared with 5%; P less than 0.001 and 34% compared with 17%; P = 0.008, respectively). Nonmigrainous headaches were more common in the control group (51% compared with 25%; P = 0.003). Chest pains were reported by 44 (47%) of the patients with Raynaud disease and by 15 (16%) of the control group (odds ratio, 4.4; CI, 2.4 to 9.3). Chest pains were more frequently reported by patients with Raynaud disease who had migraine (34 of 57, 60%) than by patients without migraine (10 of 36, 28%; P = 0.003). CONCLUSIONS: An increased prevalence of migraine was found in patients with primary Raynaud disease. Chest pain, often diagnosed as musculoskeletal or nonspecific, was common in patients with primary Raynaud phenomenon, especially in patients who had coexisting migraine.

Eosinophilic fasciitis and the carpal tunnel syndrome.

Eosinophilic fasciitis is characterised by induration and inflammation of the fascia of the extremities. It is a nosologically distinct clinical entity typified by swelling, tenderness, and stiffness of the extremities associated with peripheral eosinophilia. Six of our eight patients with eosinophilic fasciitis seen in a 3 1/2 year period have had an associated carpal tunnel syndrome. The presenting complaints in three patients were those of carpal tunnel syndrome and in the other three patients were muscle aching, swelling, and tightness. Diagnosis was made by EMG and deep fascial biopsy. Although small doses of corticosteroid medications were helpful initially in five of the six patients, two patients later required surgical decompression.

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.

Comparison of the hunting reaction in normals and individuals with Raynaud's disease.

Cold-induced vasodilation (CIVD or hunting reaction) was studied in eight subjects with Raynaud's disease, an idiopathic vasospastic disorder of the peripheral vasculature, and in nine normal subjects using 5, 10, 15 +/- 1 degree C water bath immersions of the right middle finger. Differences between Raynaud's and normal subjects were only marginal at 5 degrees C; at 10 degrees C, Raynaud's subjects showed a longer time to the first rise of skin temperature, had lower mean digital skin temperature, and a lower amplitude of their digital skin temperature during CIVD; at 15 degrees C, Raynaud's subjects had a longer time to first rise, lower number of CIVD cycles, and a lower recovery temperature.

Induced vasodilation as treatment for Raynaud's disease.

We examined the efficacy of induced vasodilation as a treatment of idiopathic Raynaud's disease. Eight persons with Raynaud's disease and seven normal persons each received 27 simultaneous pairings of hand immersion in warm water (43 degrees C) for 10 minutes with exposure of the whole body to cold (0 degrees C). A second group of seven normal persons and nine persons with Raynaud's disease received no treatments. All subjects had cold test exposures (0 degrees C) at the start and end of the study. Subjects with Raynaud's disease who received treatments showed significant increases in digital temperatures (2.2 degrees C) during the cold test compared with the values of untreated subjects with Raynaud's disease (p less than 0.05); normal subjects who had received treatments showed no difference from those who had not. Digital temperatures of subjects with Raynaud's disease after treatment increased to levels approaching those of normal subjects, although they showed lower digital temperatures during initial exposure to cold (p less than 0.01). This therapy offers a practical alternative to traditional treatments.

Diagnosis and management of fibrositis syndrome and psychogenic rheumatism.

Diffuse aching and stiffness may be caused by a fibrositis syndrome or by psychogenic rheumatism. If fibrositis is present, a careful search for underlying disease is made, and appropriate therapy started depending upon the results of all diagnostic studies.

Arthritis rounds. Pneumocystis carinii associated with polyarteritis and immunosuppressive therapy.

Pneumocystis carinii characteristically causes pneumonia in patients with immunodeficiency disorders. It occurs most often in patients with malignancy or renal transplants whose immune response has been suppressed by corticosteroids or cytotoxic agents. Individuals with connective tissue disease who receive immunosuppressive drugs become susceptible to Pneumocystis. The incidence of Pneumocystis infection in connective tissue disease is low but may increase if immunosuppressive drugs are used more often. Our patient acquired Pneumocystis pneumonia after immunosuppressive therapy for polyarteritis nodosa. Prompt recognition of this condition is essential now that specific therapy is available. Untreated Pneumocystis infection is usually fatal.