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Raynaud's disease and phenomenon [keywords]
- Digital Thermography of the Fingers and Toes in Raynaud's Phenomenon. [Journal Article]
- J Korean Med Sci 2014 Apr; 29(4):502-6.
The aim of this study was to determine whether skin temperature measurement by digital thermography on hands and feet is useful for diagnosis of Raynaud's phenomenon (RP). Fifty-seven patients with RP (primary RP, n = 33; secondary RP, n = 24) and 146 healthy volunteers were recruited. After acclimation to room temperature for 30 min, thermal imaging of palmar aspect of hands and dorsal aspect of feet were taken. Temperature differences between palm (center) and the coolest finger and temperature differences between foot dorsum (center) and first toe significantly differed between patients and controls. The area under curve analysis showed that temperature difference of the coolest finger (cutoff value: 2.2℃) differentiated RP patients from controls (sensitivity/specificity: 67/60%, respectively). Temperature differences of first toe (cutoff value: 3.11℃) also discriminated RP patients (sensitivity/specificity: about 73/66%, respectively). A combination of thermographic assessment of the coolest finger and first toe was highly effective in men (sensitivity/specificity : about 88/60%, respectively) while thermographic assessment of first toe was solely sufficient for women (sensitivity/specificity: about 74/68%, respectively). Thermographic assessment of the coolest finger and first toe is useful for diagnosing RP. In women, thermography of first toe is highly recommended.
- The Concept of Early Systemic Sclerosis Following 2013 ACR\EULAR Criteria for the Classification of Systemic Sclerosis. [JOURNAL ARTICLE]
- Curr Rheumatol Rev 2014 Apr 3.
For many years clinicians as well as clinical investigators have made the diagnosis of systemic sclerosis (SSc) in patients satisfying the 1980 American College of Rheumatology-ACR (at that time named American Rheumatism Association-ARA) preliminary criteria for the classification of this condition, leading to missing the diagnosis of SSc in some patients affected by the disease, particularly those with the limited cutaneous- sine scleroderma subset, but unsatisfying the criteria e.g. patients with Raynaud's phenomenon (RP), digital pitting scars or ulcers or sclerodactyly and telangiectasias and/or typical esophagopathy and/or interstitial fibrosis, undetected by conventional Thorax X-Ray and evident at High Resolution Computed Tomography of the Lung. </p><p> In such a setting, LeRoy and Medsger proposed to label as affected by limited SSc (lSSc) or early SSc, cases presenting with RP and either an SSc-type nailfold capillary pattern or SSc-selective autoantibodies. Koenig et al., in 2008, validated these criteria and proposed to name early SSc patients with RP and either a scleroderma marker autoantibody or typical capillaroscopic abnormalities or both, who had been clearly shown to have high probability, but not the certainty to develop definite SSc during a 20-year follow-up. </p><p> This definition has been recently challenged by the development of the new ACR/EULAR criteria for the classification of SSc. At present, to be labeled as affected by early SSc, a patient should suffer from RP associated with either scleroderma marker autoantibody or typical capillaroscopic findings and should not satisfy either 2013 ACR/EULAR criteria for the classification of SSc or criteria for SSc sine scleroderma. </p><p>
- Use of bosentan for digital ulcers related to systemic sclerosis: a real-life retrospective French study of 89 patients treated since specific approval. [JOURNAL ARTICLE]
- Scand J Rheumatol 2014 Apr 11.
Objectives: Ischaemic digital ulcers (DUs) are a common complication of systemic sclerosis (SSc). This study aimed to characterize patients with SSc and ongoing DUs treated with the endothelin receptor antagonist bosentan in clinical practice in France. Method: An observational, retrospective, longitudinal study was conducted in 10 French expert centres. Medical records from randomly selected adult SSc patients who received treatment with bosentan for DU prevention from March 2007 to December 2010 were analysed. The primary objective was to determine the profile of patients at treatment initiation. Secondary objectives were to monitor bosentan dosing, treatment schedule, and reasons for treatment termination. Results: The study included 89 patients (mean age 52 years, 69% female, 44% diffuse cutaneous SSc). At bosentan treatment initiation, the mean duration of Raynaud's phenomenon was 15 ± 12 years, and the mean time since first episode with DU was 6.5 ± 7 years. Most patients had a history of at least two episodes with DUs, separated by < 12 months (61%), and had received intravenous iloprost (63%). Previous DU complications included auto-amputation (8%), surgical amputation (6%), osteitis (6%), and gangrene (4.5%). Active smokers (25%) had a history of significantly more surgical amputation (p = 0.004) and osteitis (p = 0.004) than non-smokers. At least one active DU at bosentan initiation was detected in 82% of patients. Bosentan was used according to prescription guidelines and was well tolerated; six patients (7%) withdrew from treatment because of raised liver enzymes. Conclusions: Patients treated with bosentan for DU prevention in France have severe, refractory, ongoing ulcerative disease. Active smoking was correlated to a history of DU complications. Tolerance of bosentan was comparable to previous studies.
- [Capillaroscopy in rheumatological practice--one center experience]. [English Abstract, Journal Article]
- Lijec Vjesn 2014 Jan-Feb; 136(1-2):28-32.
Capillaroscopy is a method for evaluating morphological characteristics of nailfold capillaries. The simplicity, noninvasiveness and easiness-to-perform make the method accessible in everyday rheumatological practice. Raynaud's phenomenon is the main indication for performing capillaroscopy (differentiating between primary and secondary Raynaud's phenomenon) and diagnosing early stages of systemic sclerosis. According to some authors capillaroscopy should be included in the work-up algorithm for patients with puffy fingers and Raynaud's phenomenon. Other autoimmune conditions (systemic lupus erythematosus, polymyositis/dermatomyositis, mixed connective tissue disease, antiphospholipid syndrome and other diseases which affect microvasculature - diabetes mellitus, thromboangiitis obliterans) can have some abnormalities of the capillaroscopic pattern. We present the results of the capillaroscopies performed in our center during the period of one year.
- The clinical phenotype associated with myositis-specific and associated autoantibodies: A meta-analysis revisiting the so-called antisynthetase syndrome. [REVIEW]
- Autoimmun Rev 2014 Apr 3.
To describe the clinical spectrum associated with aminoacyl-transfer RNA synthetase (ARS) autoantibodies in patients with idiopathic inflammatory myositis defined according to Peter and Bohan's criteria.Cohort studies were selected from MEDLINE and Embase up to August 2013. Two investigators independently extracted data on study design, patient characteristics, and clinical features (interstitial lung disease [ILD], fever, mechanic's hands [MH], Raynaud's phenomenon [RPh], arthralgia, sclerodactyly, cancer and dermatomyositis-specific rash) according to the presence of myositis-specific (anti-aminoacyl-transfer RNA synthetase [ARS], anti-signal recognition particle [anti-SRP] and anti-Mi2) and myositis-associated (anti-PM/Scl, anti-U1-RNP and anti-Ku) autoantibodies.27 studies (3487 patients) were included in the meta-analysis. Arthralgia (75%, CI 67-81) and ILD (69%, CI 63-74) were the most prevalent clinical signs associated with anti-ARS autoantibodies. Anti-Mi2 and anti-SRP autoantibodies were associated with few extramuscular signs. ARS autoantibodies were identified in 13% of patients with cancer-associated myositis (5-25). Patients with non-anti-Jo1 ARS had greater odds of presenting fever (RR 0.63, CI 0.52-0.90) and ILD (RR 0.87, CI 0.81-0.93) compared to those with anti-Jo1 autoantibodies. The frequencies of myositis (RR 1.60, CI 1.38-1.85), arthralgia (RR 1.52, CI 1.32-1.76) and MH (RR 1.47, CI 1.11-1.94) were almost 50% higher in patients with anti-Jo1 compared to non-anti-Jo1 ARS autoantibodies. Patients with anti-PM/Scl differed from those with anti-ARS autoantibodies by a greater prevalence of RPh (RR 0.70, CI 0.53-0.94) and sclerodactyly (RR 0.47, CI 0.25-0.89). ILD was less frequent in patients with anti-U1-RNP autoantibodies (RR 3.35, CI 1.07-10.43). No difference was observed between anti-ARS and myositis-associated autoantibodies for other outcomes.The presence of anti-ARS autoantibodies delimits a heterogeneous subset of patients with a high prevalence of myositis, MH, arthralgia in anti-Jo1 patients, and RPh and fever in non-anti-Jo1 patients. The clinical signs of populations positive for anti-PM/Scl and anti-ARS autoantibodies largely overlap, especially with regard to ILD, challenging the clinical delimitation of the antisynthetase syndrome.
- Cryofibrinogenemia After a Liver Transplant: First Reported Case Posttransplant and a Case-Based Review of the Nontransplant Literature. [JOURNAL ARTICLE]
- Exp Clin Transplant 2014 Mar 28.
Cryofibrinogenemia is a rare disorder in which plasma, not serum, forms a cryoprecipitate. Patients with cryofibrinogenemia may be asymptomatic, or they may have painful ulcers, purpura, livedo reticularis, Raynaud phenomenon, perniosis of the extremities, thrombosis, and arthralgia. Cryofibrinogenemia may be primary or secondary to an underlying disorder such as connective tissue disease, malignancy, infection, drugs, or thromboembolic disease. Here, we present a 41-year-old woman with a pancreatic neuroendocrine tumor who underwent a Whipple procedure in 2003 followed by 2 liver transplants for hepatic metastases. Three years posttransplant, we discovered a biopsy-proven metastatic lesion in her femur. Five years posttransplant, she developed acute, severe pain in both feet, and was found to have cryofibrinogenemia despite immunosuppression posttransplant. Testing for connective tissue diseases and hematologic malignancy were negative. She was treated with high-dose prednisone, which completely resolved her symptoms. We also conducted a review of the literature via a PubMed search to summarize the association of cryofibrinogenemia with malignancy and treating cryofibrinogenemia with corticosteroids. Our study is the first reported case of cryofibrinogenemia that developed secondary to a neuroendocrine tumor posttransplant. Our report suggests that cryofibrinogenemia may occur despite immunosuppression adequate to prevent graft rejection, and that high-dose corticosteroids are an effective treatment for posttransplant cryofibrinogenemia.
- An Iranian Scoring System for Diagnosing Buerger's Disease. [Journal Article]
- Acta Med Iran 2014; 52(1):60-5.
Buerger's disease or thromboangiitis obliterans (TAO) seems to be common in IR Iran, The present study aimed to evaluate an Iranian population with Buerger's disease in order to suggest a diagnostic criterion for Buerger's disease based on the most frequent findings and to compare it with Papa diagnostic criteria. In a cross-sectional study, all patients with resting limb pain, limb ischemic ulcers, intermittent claudication and limb ischemia who referred to the Vascular Clinic of Sina Hospital during 2009-2011 were evaluated. The patients were allocated to Buerger's and non-Buerger's groups; Evaluating 122 patients (61 in each group), according to the model each clinical manifestations and risk factors in the patients with Buerger's disease obtained a score. Absent pulsation, abnormal distal Doppler sonography and ischemic ulcer were respectively present in 58 (95.1%), 58 (95.1%) and 49 (80.3%) individuals with Buerger's disease. Multivariate linear regression analysis and multivariate logistic regression analysis were used for modeling. Considering the model finding findings, diagnostic criteria including age, sex, smoking, Raynaud's phenomenon, abnormal proximal Doppler, diabetes mellitus and hyperlipidemia were suggested (R2=0.582); the sensitivity and specificity of the criteria was respectively 95.1% and 78.7%. Compared with Papa criteria, Kappa coefficient was measured at 0.66 with a P-value<0.001. It seems that the recommended criteria have an acceptable accuracy in diagnosing Buerger's disease, especially in the Iranian population; however, it is necessary to conduct more studies with larger sample sizes to evaluate the criteria, especially in other populations.
- A case of sine scleroderma with parenchymal lung disease. [Journal Article]
- Adv Biomed Res 2014.:39.
Systemic sclerosis sine scleroderma is a subtype of scleroderma, which is characterized by involvement of visceral organs, but no characteristic skin alteration. The involved organs could be kidneys, heart, gastrointestinal system, and lungs. Interstitial lung disease (ILD) is one of the pulmonary manifestations of sine scleroderma. We report a 38-year-old woman presenting with chill, fever, generalized malaise, dyspnea on exertion, and dry cough with a history of Raynaud's phenomenon, who was evaluated by physical examination, spirometry, and computed tomography scan, that all lead to the diagnosis of ILD. Combination of high-titer positive anti-nuclear antibody, high erythrocyte sedimentation rate, positive C-reactive protein, and ILD could be explained by sine scleroderma.
- Differential diagnosis of Raynaud's phenomenon based on modeling of finger thermoregulation. [Journal Article]
- Physiol Meas 2014 Apr; 35(4):703-16.
Raynaud's phenomenon (RP) is a vasospastic disorder of small arteries, pre-capillary arteries, and cutaneous arteriovenous shunts of the extremities, typically induced by cold exposure and emotional stress. RP is either primary (PRP) or secondary to connective tissue diseases such as systemic sclerosis (SSc). Early differential diagnosis is crucial in order to set the proper therapeutic strategy. To this goal, thermal infrared imaging data from 18 healthy controls (HCs) and 48 RP patients (20 PRP, 28 SSc) were processed through a model for a second-order time-invariant system with exponential critically damped dynamic response. Subject classification on the basis of the model parameters provides 100% true-positive discrimination for RP patients (PRP and SSc) and healthy, and 90% of correct classification within the group of patients. The proposed method may provide useful hints for early differential diagnosis in the assessment of RP disease.
- Clinical and autoantibody profile in systemic sclerosis: baseline characteristics from a West Malaysian cohort. [JOURNAL ARTICLE]
- Int J Rheum Dis 2014 Feb 23.
To evaluate the clinical and antibody profile of systemic sclerosis (SSc) in a Malaysian cohort.Consecutive patients with SSc in University Malaya Medical Centre from March to November 2012 were included in this study. In addition to clinical characterization, all subjects underwent autoantibody testing using Euroline immunoblot assay. The association between clinical features and autoantibody profile was evaluated.There were 31, predominantly Chinese (45.2%), subjects. Limited cutaneous disease was the most common subtype (71%). Raynaud's phenomenon was the most commonly observed feature (83.9%). Nine (29%) had esophageal dysmotility symptoms and 23 (74.2%), including all patients with diffuse SSc, had symptoms of gastro-esophageal reflux disease (GERD). Restrictive pattern on pulmonary function test and evidence of lung fibrosis were seen in more than 70% of patients. Echocardiographic evidence of pulmonary arterial hypertension was seen in 58.1%. Telangiectasia, calcinosis, digital ulcers, digital pulp loss or pitting were seen more commonly in the diffuse subtype. The two most prevalent autoantibodies were anti-Scl-70 and anti-Ro-52. The presence of anti-Scl-70 was significantly associated with restrictive lung disease (P = 0.05). Anti-Ro-52 was associated with control subjects with other autoimmune diseases (P = 0.043). The presence of anti-PM-Scl-75 was associated with overlap syndrome (P = 0.032). Patients with anticentromere antibodies were more likely to have vasculitic rash (P = 0.012).In Malaysia, SSc most commonly affects the Chinese. Limited cutaneous is more common than diffuse subtype. Features of CREST (calcinosis, Reynaud disease, esophageal dysmotility, sclerodactyly, telangiectasia) are more commonly observed in the diffuse cutaneous subgroup. Anti-Scl-70 and anti-Ro-52 antibodies are promising biomarkers for pulmonary involvement in SSc.