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1.
BMJ Case Rep ; 17(3)2024 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-38442964

RESUMO

We report a case of Raynaud's phenomenon in a patient with psoriatic arthritis (PsA). A middle-aged right-handed housewife presented with complaints of severely painful hand discolouration for 1 week, which usually worsened with cold exposure. She was diagnosed with PsA 6 months earlier. Her PsA was well controlled with weekly methotrexate. Physical examination showed no features of scleroderma or skin necrosis of her right hand. Both radial pulses were strong and symmetrical. Her nailfolds were visibly normal. The extractable nuclear antigen panel and other blood investigations were negative for scleroderma and other possible causes of secondary Raynaud's phenomenon. Occupational or environmental factors were also excluded. Dermatoscope examination of the nailfolds revealed some areas of dilated capillary loops, areas of vascular sparing and proximal nail fold telangiectasia. The diagnosis of secondary Raynaud's phenomenon was made, and an oral calcium channel blocker was started. The patient had significant improvement in symptoms shortly afterwards.


Assuntos
Artrite Psoriásica , Doença de Raynaud , Esclerodermia Localizada , Feminino , Pessoa de Meia-Idade , Humanos , Artrite Psoriásica/complicações , Artrite Psoriásica/diagnóstico , Artrite Psoriásica/tratamento farmacológico , Doença de Raynaud/complicações , Doença de Raynaud/diagnóstico , Bloqueadores dos Canais de Cálcio , Mãos , Metotrexato
2.
Sci Rep ; 14(1): 2719, 2024 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-38302542

RESUMO

Hand-arm vibration injury is a well-known occupational disorder that affects many workers globally. The diagnosis is based mainly on quantitative psychophysical tests and medical history. Typical manifestations of hand-arm vibration injury entail episodes of finger blanching, Raynaud's phenomenon (RP) and sensorineural symptoms from affected nerve fibres and mechanoreceptors in the skin. Differences in serum levels of 17 different biomarkers between 92 patients with hand-arm vibration injury and 51 controls were analysed. Patients with hand-arm vibration injury entailing RP and sensorineural manifestations showed elevated levels of biomarkers associated with endothelial injury or dysfunction, inflammation, vaso- or neuroprotective compensatory, or apoptotic mechanisms: intercellular adhesion molecule-1 (ICAM-1), monocyte chemoattractant protein-1 (MCP-1); thrombomodulin (TM), heat shock protein 27 (HSP27); von Willebrand factor, calcitonin gene-related peptide (CGRP) and caspase-3. This study adds important knowledge on pathophysiological mechanisms that can contribute to the implementation of a more objective method for diagnosis of hand-arm vibration injury.


Assuntos
Traumatismos do Braço , Traumatismos da Mão , Doenças Profissionais , Doença de Raynaud , Humanos , Vibração , Mãos , Dedos/inervação , Biomarcadores
3.
Ann Plast Surg ; 92(3): 287-293, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38394270

RESUMO

BACKGROUND: Autologous fat grafting (AFG) has emerged as a promising treatment option for Raynaud phenomenon. However, existing studies are limited by short follow-up, and there is little evidence regarding predictive factors for successful outcomes. METHODS: A retrospective chart review and standardized phone interviews were performed for all patients (n = 17, 65% response rate) treated with AFG to the hands or feet at our institution for primary or secondary Raynaud from 2010 to 2021. Each occurrence of AFG was defined as a separate surgery (n = 23), with an average follow-up of 3.7 years. RESULTS: At follow-up, patients reported a 31% reduction in cold attack frequency, a 45% reduction in the intensity of individual attacks, a 29% reduction in the duration of attacks, and a 40% improvement in overall Raynaud Condition Score (P < 0.01). Although initial AFG to an extremity significantly improved symptoms, subsequent attempts were not shown to statistically improve outcomes. Digital ulcers were present in 65% of cases, and AFG resulted in ulcer healing in 87% of those cases. Median duration of maximum symptom relief was 1 year postoperatively, with 74% of patients reporting diminishing symptom relief by 4 years postoperatively. Those with a BMI ≥25, with primary Raynaud phenomenon or without preoperative ulcers experienced significantly longer symptom relief (P < 0.05). Average patient satisfaction was 7.7 of 10, and 91% would recommend the procedure to others. CONCLUSIONS: Autologous fat grafting is an effective, albeit sometimes temporary, treatment for Raynaud and digital ulcers. Certain patients may be more likely to experience lasting symptom relief beyond 1 year.


Assuntos
Tecido Adiposo , Doença de Raynaud , Úlcera Cutânea , Humanos , Tecido Adiposo/transplante , Estudos Retrospectivos , Mãos/cirurgia , Transplante Autólogo/métodos , Doença de Raynaud/cirurgia
4.
Semin Arthritis Rheum ; 65: 152372, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38325052

RESUMO

OBJECTIVE: To explore, from patients' perspectives, the symptoms and impact of Raynaud's phenomenon (RP) on the feet of patients with systemic sclerosis (SSc-RP), and to identify which foot-related domains are important to patients. METHODS: Forty participants (34 women) with SSc-RP took part in one of six focus groups held in the United Kingdom or United States. Participants were purposively sampled to ensure diversity in disease type, duration, and ethnicity. The topic guide included questions on RP impact, self-management, and treatment expectations. Qualitative content analysis was employed to identify key concepts in the data relating to foot-specific symptoms and their impact. Themes were organized by corresponding domains of potential importance. RESULTS: Twenty-eight participants (70 %) reported experiencing RP in their feet. Five themes were identified corresponding to domains of potential importance: temperature changes, pain, cramping and stiffness, numbness, and color changes. These issues negatively affected participants' lives, impairing walking, driving, and socializing, and causing issues with footwear and hosiery. CONCLUSIONS: This large qualitative study exploring the experiences of patients with SSc-RP in the feet identified several key domains of high importance to patients. SSc-RP is common in the feet, presents in several patterns, and impacts multiple aspects of patients' lives. These findings indicate where future foot-specific interventions for RP could be targeted. Findings from this study improve understanding of what domains are important to patients with SSc-RP affecting the feet and will contribute to the development of a core outcome set for foot and ankle disorders in rheumatic and musculoskeletal diseases.


Assuntos
Doença de Raynaud , Escleroderma Sistêmico , Humanos , Feminino , Tornozelo , Escleroderma Sistêmico/complicações , Pesquisa Qualitativa , Dor/complicações , Doença de Raynaud/etiologia
6.
Clin Rheumatol ; 43(2): 733-741, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38190091

RESUMO

INTRODUCTION: Juvenile Sjögren's disease (jSjD) is a rare autoimmune disease characterized by exocrine gland involvement and systemic manifestations, including small vessel vasculitis and Raynaud's phenomenon (RP). We aimed to investigate the microvascular status in jSjD patients by nailfold videocapillaroscopy (NVC) and the potential correlations with clinical and serological features. METHODS: Clinical data from thirteen consecutive jSjD patients (11 females and 2 males), with a mean age of 16 ± 4 years, diagnosed before 16 years of age (mean age at diagnosis 12 ± 3) according to the 2016 American College of Rheumatology/EULAR criteria for adult SjD, were collected including age- and sex-matched healthy controls (HCs). Clinical, laboratory, and instrumental data were collected, together with NVC examination. Non-specific and specific NVC parameters were investigated, such as capillary density, capillary dilations, giant capillaries, microhaemorrhages and abnormal shapes. Associations between NVC findings and clinical/serological features were explored and analysed using parametrical and non-parametrical tests. RESULTS: Capillary density reduction correlated significantly with articular involvement (arthralgias) (p = 0.024). Microhaemorrhages correlated with lower C3 levels (p = 0.034). No specific NVC pattern for jSjD was identified, whereas abnormal capillary shapes were significantly higher in jSjD patients than HCs (p = 0.005). NVC abnormalities were not associated with SjD-specific instrumental tests (biopsy, imaging, Schirmer's test). RP was present in 8% of jSjD patients. CONCLUSIONS: The reduction of capillary density, as well as microhaemorrhages at NVC analysis, are significantly associated with some clinical aspects like articular involvement and serum biomarkers (C3 reduction). The NVC is suggested as safe and further analysis in jSjD patients.


Assuntos
Doenças Autoimunes , Doença de Raynaud , Escleroderma Sistêmico , Síndrome de Sjogren , Masculino , Adulto , Feminino , Humanos , Criança , Adolescente , Adulto Jovem , Angioscopia Microscópica/métodos , Unhas/irrigação sanguínea , Capilares/diagnóstico por imagem , Capilares/patologia , Doenças Autoimunes/patologia , Síndrome de Sjogren/diagnóstico por imagem , Síndrome de Sjogren/patologia , Doença de Raynaud/patologia , Escleroderma Sistêmico/patologia
7.
BMJ Case Rep ; 17(1)2024 Jan 16.
Artigo em Inglês | MEDLINE | ID: mdl-38233001

RESUMO

Vibration white finger is a form of secondary Raynaud's phenomenon (RP) caused by the use of handheld vibrating tools. RP usually appears on the extremities of the fingers, and its borders are well recognised. No reports have been published on 'mottled' RP in continuous observation from the onset to the disappearance of RP. A man in his 60s who had been using vibrating tools such as jackhammers and tampers for 30 years presented with sensations of coldness, burning and numbness. Whole-body cold exposure was performed outdoors in winter, and RP was photographed continuously. 'Mottled' RP can be defined as triphasic colour changes: white, blue and red. The patient was taken off work, kept warm and medicated. His symptoms improved slightly after 10 years of follow-up, but the RP did not disappear. 'Mottled' RP is rare and refractory and should be recognised as a form of RP.


Assuntos
Síndrome da Vibração do Segmento Mão-Braço , Doenças Profissionais , Doença de Raynaud , Masculino , Humanos , Síndrome da Vibração do Segmento Mão-Braço/complicações , Síndrome da Vibração do Segmento Mão-Braço/diagnóstico , Vibração/efeitos adversos , Doença de Raynaud/diagnóstico , Doença de Raynaud/etiologia , Dedos , Hipestesia , Doenças Profissionais/etiologia , Doenças Profissionais/complicações
9.
Rev Med Interne ; 45(2): 109-113, 2024 Feb.
Artigo em Francês | MEDLINE | ID: mdl-37980267

RESUMO

Systemic sclerosis (SSc) is a rare connective tissue disease characterized by inflammation, fibrosis, and autoimmunity. Despite few clinical trials when compared to other autoimmune diseases, SSc has benefited from renewed interest over the past ten years and a large number of clinical trials have been performed or are underway. We present here the results of the trials published in the last 5 years in ScS according to the chosen endpoint criteria and describe the trials in progress or expected in the years to come.


Assuntos
Produtos Biológicos , Doenças do Tecido Conjuntivo , Doença de Raynaud , Escleroderma Sistêmico , Humanos , Produtos Biológicos/uso terapêutico , Escleroderma Sistêmico/tratamento farmacológico
11.
Occup Med (Lond) ; 74(1): 128-130, 2024 02 19.
Artigo em Inglês | MEDLINE | ID: mdl-37796654

RESUMO

This is the first known case report of COVID-19-related Raynaud's phenomenon (RP) identified at routine health surveillance of a male exposed to hand-transmitted vibration. The temporal relationship with COVID infection followed the course previously reported, being a late feature associated with mild COVID, and followed by gradual spontaneous recovery. It is not known whether the RP, in this case, is solely due to COVID or represents a summative effect of COVID and vibration exposure. A prudent approach is recommended in such circumstances with limitation of vibration exposure and continuing frequent surveillance pending improvement of the RP, with further prompt investigation if the expected recovery does not occur.


Assuntos
COVID-19 , Doença de Raynaud , Humanos , Masculino , Vibração/efeitos adversos , COVID-19/complicações , Mãos , Doença de Raynaud/complicações
12.
Int J Circumpolar Health ; 83(1): 2295576, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38109321

RESUMO

The literature on Raynaud's phenomenon (RP) in the feet is scarce, especially in the occupational setting. The primary aim of our study was to investigate the occurrence of RP in the feet of miners. As part of the MineHealth project, written surveys and clinical examinations were completed by 260 Arctic open-pit miners working in northern Sweden and Norway (participation rate 53.6%). Data on RP were collected using standardised colour charts and questionnaire items. Clinical examination included assessing the perception of vibration and pain in both feet. There were eight women and three men who reported RP in the feet. Four also had RP in their hands but none acknowledged any first-degree relatives with the condition. Nine reported exposure to foot-transmitted vibration and one to hand-arm vibration. Seven showed signs of neurosensory injury in the feet. To conclude, the occurrence of RP in the feet of miners was 4.4%. Most cases with RP in the feet did not report the condition in the hands and were exposed to vibration transmitted directly to the feet. There were no reports of a hereditary component. Most cases with RP in the feet also had clinical findings suggestive of peripheral neuropathy in the feet.


Assuntos
Doenças Profissionais , Doença de Raynaud , Masculino , Humanos , Feminino , Doenças Profissionais/epidemiologia , Doença de Raynaud/epidemiologia , Mãos , Vibração/efeitos adversos , Dor
14.
Medicine (Baltimore) ; 102(51): e36654, 2023 Dec 22.
Artigo em Inglês | MEDLINE | ID: mdl-38134088

RESUMO

BACKGROUND: To investigate the risk factors for the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE). METHODS: The literature related to risk factors for the development of PAH in SLE patients was searched by the computer on China national knowledge infrastructure (CNKI), PubMed, and Embase, and the literature search was limited to the period of library construction to October 2022. Two researchers independently performed literature screening and literature information extracting, including first author, publication time, case collection time, sample size, and study factors, and used the Newcastle-Ottawa Scale (NOS) to evaluate the quality of the literature. The relationship between each clinical manifestation and laboratory index and the occurrence of PAH in SLE patients was evaluated based on the ratio (OR value) and its 95% CI. RESULTS: A total of 24 publications were included, including 23 case-control studies and 1 cohort study with NOS ≥ 6, and the overall quality of the literature was high. The risk of PAH was higher in SLE patients who developed Raynaud phenomenon than in those who did not [OR = 2.39, 95% CI (1.91, 2.99), P < .05]; the risk of PAH was higher in SLE patients who were positive for anti-RNP antibodies than in those who were negative for anti-RNP antibodies [OR = 1.77, 95% CI (1.17, 3.2.65), P < .05]; the risk of PAH was higher in SLE patients with interstitial lung lesions than in those without combined interstitial lung lesions [OR = 3.28, 95% CI (2.37, 4.53), P < .05]; the risk of PAH was higher in SLE patients with combined serositis than in those without serositis [OR = 2.28, 95% CI (1.83, 2.84), P < .05]. The risk of PAH was higher in SLE patients with combined pericardial effusion than in those without pericardial effusion [OR = 2.97, 95% CI (2.37, 3.72), P < .05]; the risk of PAH was higher in SLE patients with combined vasculitis than in those without vasculitis [OR = 1.50, 95% CI (1.08, 2.07), P < .05]; rheumatoid factor-positive SLE patients had a higher risk of PAH than those with rheumatoid factor-negative [OR = 1.66, 95% CI (1.24, 2.24), P < .05]. CONCLUSION: Raynaud phenomenon, vasculitis, anti-RNP antibodies, serositis, interstitial lung lesions, rheumatoid factor, and pericardial effusion are risk factors for the development of PAH in patients with SLE.


Assuntos
Hipertensão Pulmonar , Lúpus Eritematoso Sistêmico , Derrame Pericárdico , Hipertensão Arterial Pulmonar , Doença de Raynaud , Serosite , Vasculite , Humanos , Hipertensão Arterial Pulmonar/etiologia , Hipertensão Arterial Pulmonar/complicações , Estudos de Coortes , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/diagnóstico , Serosite/complicações , Fator Reumatoide , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/diagnóstico , Hipertensão Pulmonar Primária Familiar/complicações , Fatores de Risco , Doença de Raynaud/complicações , Doença de Raynaud/epidemiologia , Vasculite/complicações
16.
Sci Rep ; 13(1): 19751, 2023 11 13.
Artigo em Inglês | MEDLINE | ID: mdl-37957209

RESUMO

We evaluated the predictive value of dynamic blood flow scintigraphy with 99mTc-HDP (hydroxymethylene diphosphonate) for therapeutic response in patients with Raynaud's phenomenon (RP). Eighty patients who underwent dynamic blood flow scintigraphy using the one-hand chilling method were enrolled. We analyzed the quantitative variables as the ratio of chilled fingers to ambient fingers (CAfinger), that of the chilled hand to ambient hand (CAhand), and that of chilled fingers to ambient palm (FPR) (CAFPR) at 15 and 30 s after 99mTc-HDP bolus injection. Total cumulative radioactivity counts for 180 s were obtained. We evaluated the clinical utility of these quantitative parameters with other clinical variables, including RP severity, therapeutic compliance, types of RP, and scintigraphic interpretation of findings in patients with RP. Fifty-two patients showed poor therapeutic response. There were significant differences between good- and poor-therapeutic responder groups in RP intensity (p = 0.003), CAfinger15s (p = 0.008), CAfinger30s (p = 0.002), CAfinger180s (p = 0.011), CAhand15s (p = 0.008), CAhand30s (p = 0.007), CAhand180s (p = 0.017), CAFPR30s (p = 0.004), and CAFPR180s (p = 0.002). After multivariate logistic regression analysis, only CAfinger30s (p = 0.002) had an independent predictive value of the therapeutic response. 99mTc-HDP dynamic blood flow scintigraphy could be helpful in predicting the therapeutic response in patients with RP.


Assuntos
Mãos , Doença de Raynaud , Humanos , Dedos , Cintilografia , Medronato de Tecnécio Tc 99m , Difosfonatos , Doença de Raynaud/diagnóstico por imagem , Doença de Raynaud/tratamento farmacológico , Calafrios
18.
BMC Pediatr ; 23(1): 574, 2023 11 17.
Artigo em Inglês | MEDLINE | ID: mdl-37978357

RESUMO

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm affecting infants or young children. KHE includes a spectrum of lesions, ranging from small and superficial tumors to large and invasive lesions with Kasabach-Merritt phenomenon (KMP). Currently, no published studies have reported a KHE presenting as thrombocytopenia and Raynaud phenomenon. CASE PRESENTATION: A 2-year-old boy with right hand swelling and thrombocytopenia was admitted to our hospital. His right hand turned swelling and red, even occasionally cyanotic. This condition became worse in response to cool environments and improved with warming, and platelet counts were between 50 ~ 80 × 10^9/L. Physical examination on admission revealed the swelling and frostbite-like rash of the right-hand fingers, and the skin temperature of the right hand was lower than the left. On day 3 of admission, chest CT results showed an irregular mass on the right side of the spine. The puncture biopsy demonstrated positive CD31, D2-40, and FLI1 immunohistochemical staining, but negative GLUT1 staining, confirming the diagnosis of KHE. Furthermore, endothelin-1 (ET1) expression levels significantly increased, and eNOS and A20 expression levels significantly decreased comparing with control patients. The patient received methylprednisolone and sirolimus treatments, and his condition gradually improved during the follow-up. CONCLUSIONS: We reported the first case of KHE presenting with thrombocytopenia and Raynaud phenomenon. The development of Raynaud phenomenon could be associated with increased ET-1 and reduced eNOS and A20 expressions. Careful differential diagnosis of hidden KHE should be considered in children with thrombocytopenia and Raynaud phenomenon.


Assuntos
Hemangioendotelioma , Síndrome de Kasabach-Merritt , Doença de Raynaud , Sarcoma de Kaposi , Lactente , Criança , Masculino , Humanos , Pré-Escolar , Síndrome de Kasabach-Merritt/complicações , Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/patologia , Hemangioendotelioma/complicações , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/patologia , Sarcoma de Kaposi/complicações , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/patologia , Doença de Raynaud/complicações , Doença de Raynaud/diagnóstico
19.
Nat Commun ; 14(1): 6156, 2023 10 12.
Artigo em Inglês | MEDLINE | ID: mdl-37828025

RESUMO

Raynaud's phenomenon (RP) is a common vasospastic disorder that causes severe pain and ulcers, but despite its high reported heritability, no causal genes have been robustly identified. We conducted a genome-wide association study including 5,147 RP cases and 439,294 controls, based on diagnoses from electronic health records, and identified three unreported genomic regions associated with the risk of RP (p < 5 × 10-8). We prioritized ADRA2A (rs7090046, odds ratio (OR) per allele: 1.26; 95%-CI: 1.20-1.31; p < 9.6 × 10-27) and IRX1 (rs12653958, OR: 1.17; 95%-CI: 1.12-1.22, p < 4.8 × 10-13) as candidate causal genes through integration of gene expression in disease relevant tissues. We further identified a likely causal detrimental effect of low fasting glucose levels on RP risk (rG = -0.21; p-value = 2.3 × 10-3), and systematically highlighted drug repurposing opportunities, like the antidepressant mirtazapine. Our results provide the first robust evidence for a strong genetic contribution to RP and highlight a so far underrated role of α2A-adrenoreceptor signalling, encoded at ADRA2A, as a possible mechanism for hypersensitivity to catecholamine-induced vasospasms.


Assuntos
Estudo de Associação Genômica Ampla , Doença de Raynaud , Humanos , Úlcera , Doença de Raynaud/genética , Doença de Raynaud/complicações , Dor/complicações , Fatores de Transcrição/genética , Proteínas de Homeodomínio , Receptores Adrenérgicos alfa 2/genética
20.
Toxins (Basel) ; 15(10)2023 10 04.
Artigo em Inglês | MEDLINE | ID: mdl-37888629

RESUMO

A field biologist was bitten by a female Nikolsky's viper (Vipera berus nikolskii) in Kharkiv Oblast, Ukraine. Two months later, the patient began to experience cold-induced vasospasm of the affected digit diagnosed as acquired Raynaud phenomenon. The patient had more than 30 occurrences during the single winter following the bite, but the signs and symptoms of Raynaud phenomenon disappeared with the end of winter. This report describes the case and puts it into context with the literature on the topic of toxin-induced peripheral vasospastic disorders and their potential importance in snakebite envenoming.


Assuntos
Doença de Raynaud , Mordeduras de Serpentes , Viperidae , Animais , Humanos , Feminino , Mordeduras de Serpentes/complicações , Venenos de Víboras/toxicidade , Extremidade Superior , Doença de Raynaud/diagnóstico , Doença de Raynaud/etiologia , Antivenenos
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