Anti-centromere antibody and CREST syndrome in patients with primary biliary cirrhosis.
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Anti-centromere antibodies (ACA) in 41 sera from patients with primary biliary cirrhosis (PBC) were analyzed by an immunoblotting method and the correlation between the presence of ACA and the clinical features in these PBC patients was studied. In 10 of 16 ACA-positive PBC patients, one or more clinical features of CREST syndrome (PBC-CREST) were found. Statistical differences were observed in age at disease onset, serum levels of IgM and total bilirubin and titer of anti-M2 antibody, between PBC-CREST patients and the PBC patients without CREST symptoms (PBC-non CREST). By immunoblotting analysis, three major epitopes of ACA were identified at 18 kD, 80 kD and 140 kD polypeptides. The 18 kD polypeptides were detected in all 16 ACA-positive PBC patients. From these results, it is suggested that ACA-positive PBC-CREST patients can be separated from ACA-negative PBC-CREST and PBC-non CREST patients. (+info)
Increased asymmetric dimethylarginine and endothelin 1 levels in secondary Raynaud's phenomenon: implications for vascular dysfunction and progression of disease.
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OBJECTIVE: To compare microvascular and macrovascular functions in a cohort of patients with primary and secondary Raynaud's phenomenon (RP) who were matched for demographic, risk factor, and severity profiles. METHODS: Forty patients with primary or secondary RP matched for vascular risk factors and severity scores underwent testing of endothelial function and cold pressor responsiveness of the brachial artery. Microvascular perfusion of the digital vasculature was assessed using laser Doppler fluxmetry in response to reactive hyperemia. Plasma was assayed for endothelin 1 (ET-1), asymmetric dimethylarginine (ADMA), intercellular adhesion molecule 1, vascular cell adhesion molecule 1 (VCAM-1), and monocyte chemoattractant protein 1 (MCP-1). RESULTS: Patients with RP had abnormal vasoconstrictor responses to cold pressor tests (CPT) that were similar in primary and secondary RP. There were no differences in median flow-mediated and nitroglycerin-mediated dilation or CPT of the brachial artery in the 2 populations. Patients with secondary RP were characterized by abnormalities in microvascular responses to reactive hyperemia, with a reduction in area under the curve adjusted for baseline perfusion, but not in time to peak response or peak perfusion ratio. Plasma ET-1, ADMA, VCAM-1, and MCP-1 levels were significantly elevated in secondary RP compared with primary RP. There was a significant negative correlation between ET-1 and ADMA values and measures of microvascular perfusion but not macrovascular endothelial function. CONCLUSION: Secondary RP is characterized by elevations in plasma ET-1 and ADMA levels that may contribute to alterations in cutaneous microvascular function. (+info)
Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.
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BACKGROUND: Hypothenar hammer syndrome (HHS) is a cause of vascular insufficiency to the hand and may be manifest as Raynaud's phenomenon. The cause is trauma to the vulnerable portion of the ulnar artery as it passes over the hamate bone, which may result in thrombosis, irregularity or aneurysm formation. AIM: This review was undertaken in order to clarify the features of HHS that may differentiate it from hand-arm vibration syndrome. METHODS: A tiered review of world literature was undertaken using Medline and EMBase as the primary search engines. Fifty-two relevant articles were critically reviewed. CONCLUSION: Colour and temperature changes occur more diffusely in HHS than in classical Raynaud's phenomenon and the absence of the triphasic colour change may alert clinicians to the diagnosis, which may be confirmed by Allen's test. Doppler or arteriographic studies are required for confirmation. It is important to recognize the possibility of HHS in the occupational setting as a potentially curable cause of Raynaud's phenomenon, distinct from hand-arm vibration syndrome. The possibility exists of HHS occurring as a result of repeated hypothenar trauma from vibrating tools, in which case the nature and magnitude of the individual episode of trauma may be more important than the weighted acceleration level of vibration exposure. (+info)
A critique of a UK standardized test of finger rewarming after cold provocation in the diagnosis and staging of hand-arm vibration syndrome.
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BACKGROUND: Accurate diagnosis and staging of hand-arm vibration syndrome (HAVS) is important in health surveillance of vibration-exposed workers and the substantial number of related medico-legal cases. The measurement of the rewarming rate of fingers after cold provocation to the hands (CPT) has been suggested as a useful test in diagnosing HAVS. AIM: To investigate the diagnostic value of a standardized version of the CPT test using a 15 degrees C cold challenge for 5 min applied in the recent compensation assessment of UK miners. METHODS: Analysis of a subset of UK miners assessed at our unit, together with data from a small repeatability study of the standardized CPT in normal subjects. RESULTS: Rewarming time in the CPT was significantly lower in those subjects classified as vascular Stockholm stage 0 compared with Stockholm stages 1-3 combined, but did not discriminate between the stages of abnormality. Using the suggested cut-off in the CPT test, the sensitivity and specificity were calculated as 43 and 78%, respectively. Receiver operator characteristic analysis suggested that the rewarming time of highest accuracy gave a sensitivity of 66% and specificity of 59%. In 10 miners who reported unilateral hand blanching, there was no significant difference in CPT measurements between blanching and non-blanching hands. Repeat CPT measurements in normal subjects suggested mean differences of 52 and 107 s for each hand, and the Bland-Altman coefficient of repeatability was approximately 600 s for all fingers. CONCLUSION: Single application of this standardized CPT test may have limited value in diagnosing the vascular component of HAVS in an individual. (+info)
Raynaud's phenomenon and antiphospholipid antibodies in systemic lupus erythematosus: is there an association?
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OBJECTIVE: To evaluate the association of IgG and IgM antibodies directed against different negatively charged phospholipids (that is, anticardiolipin (aCL), antiphosphatidylinositol, antiphosphatidylserine, and antiphosphatidic acid) and anti-beta(2)-glycoprotein I (abeta(2)GPI), with Raynaud's phenomenon in patients with systemic lupus erythematosus (SLE). METHODS: Ninety three patients with SLE (81 female), 40 with and 53 without Raynaud's phenomenon, were included in the study. IgG and IgM antiphospholipid antibodies and abeta(2)GPI were determined by enzyme linked immunosorbent assay (ELISA). RESULTS: Fifty patients (54%) were positive for IgG and/or IgM antibodies to one or more phospholipid antigens or to beta(2)GPI. The prevalence of all autoantibodies evaluated, either IgG or IgM, was higher in patients without than in those with Raynaud's phenomenon. A negative association was found between IgG aCL and Raynaud's phenomenon (p=0.038), whereas autoantibodies other than aCL were not significantly associated with Raynaud's phenomenon. CONCLUSION: Our results demonstrate no positive association between antiphospholipid antibodies and Raynaud's phenomenon in SLE and indicate that measurement of anti-negatively charged phospholipid antibodies other than aCL is not useful as a serological marker predictive for Raynaud's phenomenon. (+info)
White blood cell activation in Raynaud's phenomenon of systemic sclerosis and vibration induced white finger syndrome.
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Systemic sclerosis (SSc) and vibration induced white finger syndrome (VWF) are common causes of secondary Raynaud's phenomenon. Previous studies have suggested an increase in inflammation in patients with SSc. Vibration induced white finger syndrome occurs in workers exposed to vibration and is now a prescribed disease. In VWF, although it is recognised that vibration can cause direct damage to blood vessels, it does not explain why only some workers are affected. It is possible that an inflammatory process develops in these patients in the same way as is seen in SSc. Leukotriene B4, produced mainly by polymorphonuclear cells, is a potent mediator of inflammation. The plasma thiol concentration shows the degree of oxidation of plasma and a decreased concentration indicates the increased production of free radicals which are capable of oxidative damage. In this work, the white blood cell (WBC) production of leukotriene B4 and plasma thiol concentrations were measured in patients with SSc and VWF. Fifty nine patients were studied: 34 had SSc and 25 had VWF. The results were compared with 25 matched normal controls and are expressed as the median and range. After stimulation of the polymorphonuclear cells with calcium ionophore A23187 (1 microgram/ml), patients with SSc and VWF were found to have significantly increased leukotriene B4 production (23.5 (14.4-44.0) and 26 (14.4-39.4) ng/10(6) cells, respectively) compared with normal controls (17.2 (6.0-38.0) ng/10(6) cells). The plasma thiol concentration was shown to be significantly lower in patients with SSc and VWF (445 (375-475) and 450 (417-510) mumol/l, respectively) compared with normal controls (480 (418-555) mumol/l). Our results show increased leukotriene B4 production and increased free radical activity in patients with SSc and VWF. Although previous indirect evidence has suggested increased WBC activity in patients with SSc, this is reported directly here for the first time. In addition, the possibility of an inflammatory process occurring in patients with VWF, as shown here, has not previously been studied. This may be a further mechanism to explain the poor circulation in the fingers of these patients. (+info)
Immunological mechanisms in the pathogenesis of vinyl chloride disease.
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Vinyl chloride (VC) disease is a multisystem disorder incorporating Raynaud's phenomenon, acro-osteolysis, thrombocytopenia, portal fibrosis, and hepatic and pulmonary dysfunction. Immunological and immunochemical investigations showed the presence of circulating immune complexes in 19 out of 28 patients with the disease and in a further two out of 30 workers exposed to VC. The immunological data were reviewed in relation to the clinical picture of the disease and to the available evidence on the metabolism of VC. The results suggest that VC disease is an immune complex disorder and that the immune response is initiated by the adsorption of VC or a metabolite on to tissue or plasma protein. (+info)
Treatment of Raynaud's phenomenon with triiodothyronine corrects co-existent autonomic dysfunction: preliminary findings.
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Cardiovascular autonomic function was assessed in 9 subjects with Raynaud's phenomenon. The underlying diseases were systemic lupus erythematosus (n = 5), systemic sclerosis (n = 3) and rheumatoid arthritis (n = 1). Five standard non-invasive tests, 3 of heart rate and 2 of blood pressure, were employed. Compared with age and sex matched controls (n = 25), the number of values abnormal was 24 of 45 (53%) overall and between one and 4 (median, 2) individually. Significant differences were present for 3 tests, two of heart rate and one of blood pressure. The subjects were given triiodothyronine, 60 to 80 micrograms per day, for vasospastic attacks. Autonomic function was reassessed between weeks 4 and 9 (9 subjects) and between weeks 12 and 18 (8 subjects) after introduction of triiodothyronine. Test results showed a considerable improvement. At the second reassessment, the number of values abnormal was now 5 of 40 (12.5%) overall and nil (n = 4) or one (n = 4) individually. Significant differences remained for one heart rate test only. Adverse side effects to triiodothyronine occurred in a single subject and were readily controlled. Evidence of somatic neuropathy was present electrophysiologically in all 9 subjects and clinically in 8. Triiodothyronine may have corrected autonomic dysfunction by increasing blood flow to ischaemic peripheral nerves or by acting on the autonomic system more directly. Further study of triiodothyronine in autonomic insufficiency appears merited. (+info)