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Raynaud's disease and phenomenon [keywords]
- [Pharmacology of Raynaud's Phenomenon.] [JOURNAL ARTICLE]
- Therapie 2014 3-4; 69(2):115-128.
Raynaud's phenomenon (RP) is characterised by transient ischaemia in the extremities in response to cold or emotions. It can be primary (idiopathic) or secondary to an underlying disease. The pathophysiology of RP is multifactorial and complex. Microvascular impairment is a hallmark of the disease. The objective of this work is to review the different pharmacological treatments currently used in the management of RP, from their mechanism of action to the available evidence regarding their efficacy. We also propose to discuss potential pharmacological targets such as the potentiation of the nitric oxide pathway, or the inhibition of the RhoA-Rho kinase pathway. The last part of this review deals with drug-induced RP. Among various medications, beta-blockers, interferons, tyrosine-kinase inhibitors or cytotoxic agents such as bleomycin are involved.
- Conversion to Paradoxical Finding on Technetium-99m-labeled RBC Scintigraphy after Treatment for Secondary Raynaud's Phenomenon. [JOURNAL ARTICLE]
- Nucl Med Mol Imaging 2013 Dec; 47(4):278-280.
An 18-year-old woman reported that after exposure to cold temperatures her fingers appeared blue and her hands and feet felt cold. Secondary Raynaud's phenomenon (RP) associated with peripheral vascular disease was suspected. Technetium (Tc)-99m-labeled RBC hand scintigraphy after cold change showed decreased blood pool activity in her fingers. The patient's symptoms improved after she received sarpogrelate HCL (200 mg/day) and nifedifine (40 mg/day). Follow-up scintigraphy performed 7 months after the patient started treatment showed paradoxically increased blood pool activity in her fingers after cold challenge. To the best of our knowledge, this is the first case report of a patient with secondary RP showing paradoxical change on scintigraphy after she received medication that improved her symptoms.
- Pulmonary pathologic manifestations of anti-glycyl-tRNA synthetase (anti-EJ)-related inflammatory myopathy. [JOURNAL ARTICLE]
- J Clin Pathol 2014 Jun 2.
Antisynthetase syndromes are a subset of the idiopathic inflammatory myopathies characterised by the presence of autoantibodies to aminoacyl transfer-RNA synthetases (ARS) and monotypic clinical features including Raynaud phenomenon, fever, non-erosive inflammatory arthritis and hyperkeratotic skin changes ('mechanic's hands'). Interstitial lung disease (ILD) is particularly common in ARS syndromes, affecting up to 90% of patients.Four patients with ARS syndrome who possessed anti-glycyl-tRNA synthetase (anti-EJ) autoantibodies were retrieved from the University of Pittsburgh database. We report their clinical, radiographic and histopathologic findings.Patients presented with dyspnoea accompanied by Raynaud phenomenon and 'mechanic's hands'. Lung disease was the first manifestation in all four patients (100%) who were all amyopathic. High-resolution CT of the chest showed patchy opacities and consolidations in two patients (50%) whose surgical lung biopsies revealed organising diffuse alveolar damage (DAD), and lower lung zone predominant reticular infiltrates and traction bronchiectasis without honeycomb change in two patients (50%) whose surgical lung biopsies revealed usual interstitial pneumonia (UIP). Mild lymphoplasmacytic inflammation and few scattered lymphoid aggregates were present, but we found no pathognomonic histopathologic features of anti-EJ ARS syndrome. Serologic testing revealed no other autoantibodies. All patients responded to immunosuppressive therapy.Identifying ARS-associated autoantibodies in ILD patients with or without myopathy is desirable because patients may respond well to immunosuppressive therapy, and their prognosis is better than that of patients with idiopathic forms of DAD or UIP.
- Effects of partially ionised medical oxygen, especially with o2•-, in vibration white finger patients. [Journal Article]
- Int J Environ Res Public Health 2014; 11(6):5698-707.
A major symptom of hand-arm vibration syndrome is a secondary Raynaud's phenomenon-vibration white finger (VWF)-which results from a vasospasm of the digital arteries caused by work with vibration devices leading to occupational disease. Pharmacotherapy of VWF is often ineffective or has adverse effects. The aim of this work was to verify the influence of inhalation of partially ionized oxygen (O2•-) on peripheral blood vessels in the hands of patients with VWF. Ninety one (91)patients with VWF underwent four-finger adsorption plethysmography, and the pulse wave amplitude was recorded expressed in numeric parameters-called the native record. Next, a cold water test was conducted following with second plethysmography. The patients were divided in to the three groups. First and second inhaled 20-min of ionized oxygen O2•- or oxygen O2 respectively. Thirth group was control without treatment. All three groups a follow-up third plethysmography-the post-therapy record. Changes in the pulse wave amplitudes were evaluated. Inpatients group inhaling O2•- a modest increase of pulse wave amplitude was observed compared to the native record; patients inhaling medical oxygen O2 and the control showed a undesirable decline of pulse wave amplitude in VWF fingers. Strong vasodilatation were more frequent in the group inhaling O2•- compare to O2 (p < 0.05). Peripheral vasodilatation achieved by inhalation of O2•- could be used for VWF treatment without undesirable side effect in hospital as well as at home environment.
- Measuring Microangiopathy Abnormalities in Systemic Sclerosis Patients: The Role of Capillaroscopy-Based Scoring Models. [JOURNAL ARTICLE]
- Am J Med Sci 2014 May 21.
: Capillaroscopy is a noninvasive imaging technique for the in vivo study of microcirculation. The role of a qualitative evaluation of capillaroscopy in the assessment of Raynaud's phenomenon secondary to scleroderma spectrum disorder, particularly systemic sclerosis (SSc), is well defined. The usefulness of capillaroscopy in the follow-up of SSc patients and the possible prognostic role for the appearance of typical SSc vascular and visceral involvement, namely, digital ulcers, pulmonary arterial hypertension, and mortality, is suggested by many authors but still under debate. In this regard, and for a reliable and repeatable longitudinal evaluation of SSc microangiopathy, a quantitative analysis should be required. In this review, we describe the current classifications proposed to define the SSc microvascular involvement and the scoring methods suggested for a semiquantitative and quantitative analysis of microangiopathy and its correlation with clinical manifestations of disease.
- [Systemic lupus erythematosus associated pulmonary arterial hypertension: clinical analysis of 91 cases]. [English Abstract, Journal Article]
- Zhonghua Yi Xue Za Zhi 2014 Apr; 94(13):969-72.
To explore the clinical characteristics and therapeutic efficacy of systemic lupus erythematosus (SLE) associated pulmonary arterial hypertension (PAH).A total of 91 cases of SLE-PAH from 2007 to 2011 were reviewed and followed up. They were divided into 2 groups: group A: New York Heart Association (NYHA) functional class 1 and 2; group B: NYHA functional class 3 and 4.There were 2 males and 89 females with a mean age of 37 ± 11 years. The mean duration of SLE disease process was 7 ± 6 years. PAH was the primary symptom of SLE onset in 10 cases. Pulmonary arterial systolic pressure (PASP) as measured by ultrasonic cardiography (UCG) were between 40 to 128 mmHg. Eighteen cases (19.78%) underwent right heart catheterization (RHC). There were good parallels of PASP value between RHC and UCG. The main characteristics included Raynaud's phenomenon (53.8%), pericardial effusion (51.6%) and a high titer of anti-RNP antibody (57.1%). PASP was positively associated with SLE disease activity in mild and moderate cases. Among 27 mortality cases, there were 4 in group A (14.8%) and 23 in group B (85.2%) . And the causes of immortality were mostly non-cardiac in group A and right heart failure in group B. Cyclophosphamide was effective in mild and moderate cases. Forty-four cases received PAH target treatment and it could decrease the PASP in mild and moderate cases and significantly prolong the survival for severe cases.The major clinical characteristics of SLE-PAH patients include Raynaud's phenomenon, pericardial effusion and positivity of anti-RNP antibody. PASP is positively associated with SLE disease activity in mild and moderate cases for whom intravenous pulse cyclophosphamide therapy may be effective. For severe cases, concomitant PAH target therapy may significantly improve the prognosis.
- Novel photoplethysmography cardiovascular assessments in patients with Raynaud's phenomenon and systemic sclerosis: a pilot study. [JOURNAL ARTICLE]
- Rheumatology (Oxford) 2014 May 21.
Multisite photoplethysmography (PPG) cardiovascular assessments can evaluate endothelial, peripheral autonomic and arterial dysfunction. The aim of this pilot study was to investigate the potential clinical utility of the technology in assessing patients with SSc and primary RP (PRP).Multisite PPG pulse measurements, a reference ankle brachial pressure index (ABPI) and a full clinical assessment were undertaken for three subject groups: SSc, PRP and controls. Endothelial and autonomic function and arterial disease measures were obtained using pulse wave analysis.Nineteen SSc, 19 PRP and 23 control subjects were assessed and compared. Endothelial function was significantly impaired in SSc (P < 0.02), but with no difference between controls and PRP. Receiver operating characteristic-based classification accuracy was 81% (sensitivity 90%, specificity 74%) for separating SSc from controls and 82% (sensitivity 84%, specificity 79%) for separating SSc from PRP. SSc patients with digital ulcers had significantly lower endothelial function compared with those without ulcers (P < 0.05). Autonomic dysfunction was suggested in both SSc and PRP and was most exaggerated in patients with diffuse SSc. All groups had overall normal ABPI and arterial stiffness timing measures. Bilateral timing differences at the toes, which represents peripheral occlusive arterial disease, did show increased asymmetry in SSc (P < 0.02).Multisite PPG pulse technology showed potential diagnostic ability. By using measures of endothelial function, it differentiated SSc from control and PRP subjects with an accuracy of at least 81%. Objective pulse-derived measures of autonomic function and arterial disease in SSc have also been reported in this pilot study.
- Crohn's disease of the small bowel, complicated by primary biliary cirrhosis, Hashimoto thyroiditis, and Raynaud's phenomenon: favorable response of all disorders to adalimumab treatment. [Journal Article]
- Gastroenterol Hepatol Bed Bench 2013; 6(2):101-5.
We describe the case of a male patient suffering from long-lasting Crohn's disease of the small bowel who developed thyroiditis Hassimoto, Raynaud's phenomenon, and primary biliary cirrhosis, during the course of the underlying bowel disease. It is not clear whether these co-morbidities appeared coincidentally, or because they share some common immunopathogenetic mechanisms. In this patient, Crohn's disease favorably responded to the treatment with an anti-TNF-α agent (adalimumab). The serum titers of antimitochondrial antibodyies and cholestatic enzymes considerably reduced during the 3-year treatment with the biologic agent. Raynaud's phenomenon, also, completely disappeared. Bearing in mind the possible involvement of TNF-α in the pathogenesis of primary biliary cirrhosis, it could be argued that the clinical and laboratory improvement of liver disease, as well as the reduction in serum titers of antimitochondrial antibodies, might be due to the anti-TNF-α action of adalimumab. We suggest that it would be worth further investigating the role of biologic agents in the treatment of patients with primary biliary cirrhosis.
- Cluster Analysis of Autoantibodies in 852 Patients with Systemic Lupus Erythematosus from a Single Center. [JOURNAL ARTICLE]
- J Rheumatol 2014 May 15.
Associations between autoantibodies and clinical features have been described in systemic lupus erythematosus (SLE). Herein, we aimed to define autoantibody clusters and their clinical correlations in a large cohort of patients with SLE.We analyzed 852 patients with SLE who attended our clinic. Seven autoantibodies were selected for cluster analysis: anti-DNA, anti-Sm, anti-RNP, anticardiolipin (aCL) immunoglobulin (Ig)G or IgM, lupus anticoagulant (LAC), anti-Ro, and anti-La. Two-step clustering and Kaplan-Meier survival analyses were used.Five clusters were identified. A cluster consisted of patients with only anti-dsDNA antibodies, a cluster of anti-Sm and anti-RNP, a cluster of aCL IgG/M and LAC, and a cluster of anti-Ro and anti-La antibodies. Analysis revealed 1 more cluster that consisted of patients who did not belong to any of the clusters formed by antibodies chosen for cluster analysis. Sm/RNP cluster had significantly higher incidence of pulmonary hypertension and Raynaud phenomenon. DsDNA cluster had the highest incidence of renal involvement. In the aCL/LAC cluster, there were significantly more patients with neuropsychiatric involvement, antiphospholipid syndrome, autoimmune hemolytic anemia, and thrombocytopenia. According to the Systemic Lupus International Collaborating Clinics damage index, the highest frequency of damage was in the aCL/LAC cluster. Comparison of 10 and 20 years survival showed reduced survival in the aCL/LAC cluster.This study supports the existence of autoantibody clusters with distinct clinical features in SLE and shows that forming clinical subsets according to autoantibody clusters may be useful in predicting the outcome of the disease. Autoantibody clusters in SLE may exhibit differences according to the clinical setting or population.
- Paraneoplastic sclerodermiform syndrome - case report. [JOURNAL ARTICLE]
- Acta Reumatol Port 2014 Jan-Mar; 39(1):87-90.
Occasionally, auto-immune diseases may emerge as paraneoplastic syndromes. This is especially recognized in the case of polymyositis/dermatomyostis, but it is an extremely rare event in systemic sclerosis (SSc). The authors report the case of a sixty-year-old woman who presented with Raynaud's phenomenon and rapidly progressing skin thickness of the forearms, hands and lower limbs. Patient evaluation revealed a colorectal carcinoma. The patient was referred to the oncology department. This concomitance of cancer and SSc with rapid progression of the latter, suggests that the scleroderma might have a paraneoplastic origin. Such an hypothesis deserves consideration in every case as early diagnosis may be decisive to control the progression of either disease.