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1.
Zhonghua Yi Xue Za Zhi ; 100(12): 938-941, 2020 Mar 31.
Artigo em Chinês | MEDLINE | ID: mdl-32234170

RESUMO

Objective: The aim of present study is to analyze clinical and laboratory features of mixed connective tissue disease (MCTD)-associated trigeminal neuropathy (TN). Methods: Clinical records of 12 cases of MCTD complicated with TN diagnosed in Peking University People's Hospital from January 2008 to October 2019 were analyzed retrospectively. Results: The present study included 12 cases, 1 males and 11 females, average age was(40±13)years. TN was developed before the diagosis of MCTD in 1 case. TN and MCTD were occurred simutaneously in 1 case. Raynaud phenomenon (12 cases), arthritis (10cases), edema of fingers (9cases), myositis (6 cases), and pulmonary involvement (11cases) were main cinical feature of MCTD-associate TN. Antinuclear antibody (ANA) and high titer anti-U1-RNP antibody could be detected in serum of all patients. Elevated erythrocyte sedimentation rate (ESR) and creatine kinase were found in serum of 7cases and 5cases, respectively. Blink reflex tests were positive in 6 cases. Neurological symptoms improve slowly without any progress by using glucocorticoid combined immunosuppressants or intravenous gamma globulin. Conclusions: TN is often associted with actived MCTD. Positive ANA and anti-U1-RNP antibody were common in MCTD-associated TN. Blink reflex test is essential to diagnose MCTD-associated TN. Intensive treatment of MCTD contributes to control the progress of TN.


Assuntos
Doença Mista do Tecido Conjuntivo , Doença de Raynaud , Doenças do Nervo Trigêmeo , Adulto , Anticorpos Antinucleares , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
2.
Autoimmun Rev ; 19(5): 102507, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32194200

RESUMO

A few scores predicting the short-term risk of mortality in Systemic sclerosis (SSc) have been reported to date. Our study aimed to create a predictive 15-year all-cause mortality score at the time of the diagnosis of SSc. The study was based on the Spanish Scleroderma Registry (RESCLE). The cohort was split up in derivation (DC) and validation cohort (VC). A multivariate analysis to detect variables related to all-cause mortality within the first 15 years from SSc diagnosis was performed, assigning points to the rounded beta values to create the score (RESCLESCORE). 1935 SSc patients were included. The variables in the final model were as follows: age at diagnosis (+2 points > 65 years-old), male gender (+1 point), lcSSc subset (-1 point), mode of onset other than Raynaud's (+1 point), cancer (+1 point) and visceral involvement, such as ILD (+1 point), PAH (+1 point), heart (+1 point) and renal involvement (+2 points). Autoantibodies did not achieve statistical significance in the multivariate analysis. The 3 categories of risk to predict 15-year all-cause mortality at the time of diagnosis were as follows: low risk (5% vs. 7%, p = .189), intermediate risk (26.5% vs. 25.5%, p = .911) and high risk (47.8% vs. 59%, p = .316). The AUC was 0.799 (DC) vs. 0.778 (VC) (p = .530). In conclusion, the RESCLESCORE demonstrated an excellent ability to categorize SSc patients at the time of diagnosis in separate 15-year all-cause mortality risk strata at the time of diagnosis.


Assuntos
Causas de Morte , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/mortalidade , Idoso , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Doença de Raynaud/diagnóstico , Doença de Raynaud/mortalidade , Sistema de Registros , Reprodutibilidade dos Testes , Espanha/epidemiologia
3.
Nat Rev Rheumatol ; 16(4): 208-221, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32099191

RESUMO

Raynaud phenomenon is a symptom complex caused by impaired digital perfusion and can occur as a primary phenomenon or secondary to a wide range of underlying causes. Raynaud phenomenon occurs in virtually all patients with systemic sclerosis (SSc) and is often the earliest clinical manifestation to occur. Careful assessment is required in patients with Raynaud phenomenon to avoid missing secondary causes such as SSc. Digital ulcers are a painful and disabling visible manifestation of digital vascular injury in patients with SSc. Progress has been made in the classification and assessment of digital ulcers and in understanding ulcer pathogenesis, and there are a wide range of treatments available to both prevent and heal digital ulcers, some of which are also used in Raynaud phenomenon management. In this Review, the assessment of patients with Raynaud phenomenon is discussed, including 'red flags' that are suggestive of SSc. The pathogenesis, classification and assessment of SSc-associated digital ulcers are also covered, alongside an overview of management approaches for SSc-associated Raynaud phenomenon and digital ulcers. Finally, unmet needs are discussed and the concept of a unified vascular phenotype in which therapies that affect the vasculature to support disease modification strategies is introduced.


Assuntos
Dedos/irrigação sanguínea , Doença de Raynaud/complicações , Escleroderma Sistêmico/complicações , Úlcera Cutânea/etiologia , Humanos , Úlcera Cutânea/diagnóstico
4.
Vasc Endovascular Surg ; 54(4): 362-366, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32077812

RESUMO

Selective periarterial sympathectomy in Raynaud phenomenon (RP) has not been adequately studied as there was no reliable method to evaluate outcomes. However, dynamic Doppler ultrasonography may have clinical value in the management and follow-up of patients with RP; but few reports describe using the device to assess surgical outcomes. Here, we report a case of successful digital sympathectomy in a single digit and the postoperative evaluation using ultrasonography. A 23-year-old patient with secondary RP underwent surgery targeting both common digital artery (ulnar side) and the proper digital artery (radial side). The procedure yielded immediate pain relief and the improvement of recurrent fingertip ulceration. The 1-year postoperative assessment with dynamic Doppler ultrasonography using a hockey-stick probe was performed with a cold provocation test and revealed peak systolic velocity improvement comparable to the nontreated ulnar side but prominent fibrosis on the radial aspect. We anticipate that Doppler ultrasonography may be an effective tool for the postoperative assessment of patients who underwent digital sympathectomy for treatment of RP.


Assuntos
Dedos/irrigação sanguínea , Artéria Radial/diagnóstico por imagem , Artéria Radial/inervação , Doença de Raynaud/cirurgia , Simpatectomia , Artéria Ulnar/diagnóstico por imagem , Artéria Ulnar/inervação , Ultrassonografia Doppler , Velocidade do Fluxo Sanguíneo , Feminino , Humanos , Valor Preditivo dos Testes , Doença de Raynaud/diagnóstico por imagem , Doença de Raynaud/fisiopatologia , Fluxo Sanguíneo Regional , Resultado do Tratamento , Grau de Desobstrução Vascular , Adulto Jovem
5.
Autoimmun Rev ; 19(3): 102458, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31927087

RESUMO

Capillaroscopy is a non-invasive and safe tool which allows the evaluation of the morphology of the microcirculation. Since its recent incorporation in the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis together with its assessed role to monitor disease progression, capillaroscopy became a 'mainstream' investigation for rheumatologists. Given its increasing use by a variety of physicians internationally both in daily practice to differentiate primary from secondary Raynaud's phenomenon, as well as in research context to predict disease progression and monitor treatment effects, standardisation in capillaroscopic image acquisition and analysis seems paramount. To step forward to this need, experts in the field of capillaroscopy/microcirculation provide in this very consensus paper their view on image acquisition and analysis, different capillaroscopic techniques, normal and abnormal capillaroscopic characteristics and their meaning, scoring systems and reliability of image acquisition and interpretation.


Assuntos
Angioscopia Microscópica , Unhas/irrigação sanguínea , Doença de Raynaud/diagnóstico , Escleroderma Sistêmico/diagnóstico , Humanos , Reprodutibilidade dos Testes
7.
Autoimmun Rev ; 19(1): 102439, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31734401

RESUMO

INTRODUCTION: Methylphenidate (Ritalin) is a CNS stimulant, and is a common treatment for children and adults with ADHD. It has been associated with Raynaud's phenomenon (RP) but not with Systemic Sclerosis (SSc). We report a case series of patients pointing out the connection between Methylphenidate and SSc. CASES: We identified three patients in a single Rheumatology clinic in Israel, who developed SSc following treatment with CNS stimulants for ADHD. All three cases had Raynaud's phenomenon, skin changes, pathological capillaroscopy and positive ANA. Symptoms appeared and worsened over months following the use of methylphenidate and subsided after its cessation. CONCLUSION: This is the first report in the literature of a causative relation between methylphenidate and the development of SSc, a serious, life-threatening condition. Patients treated with CNS stimulants should be followed closely for side-effects such as RP and skin changes.


Assuntos
Transtorno do Deficit de Atenção com Hiperatividade/tratamento farmacológico , Estimulantes do Sistema Nervoso Central/efeitos adversos , Escleroderma Sistêmico/induzido quimicamente , Adulto , Estimulantes do Sistema Nervoso Central/uso terapêutico , Feminino , Humanos , Israel , Doença de Raynaud/induzido quimicamente , Adulto Jovem
8.
Best Pract Res Clin Rheumatol ; 33(4): 101428, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31810547

RESUMO

The early diagnosis of systemic sclerosis (SSc) can be very difficult, when most of the typical signs and symptoms are absent. For this reason, the approach to SSc has changed during the last decades because the importance of an early diagnosis and treatment has been widely understood. "Very early SSc" is identified as a condition characterized by Raynaud's phenomenon, puffy fingers, disease-specific autoantibodies, and microvascular alterations at capillaroscopy. However, reliable biomarkers able to predict the disease evolution are missing, and decision whether to treat or not to treat in the earliest phase of the disease remains a dilemma. Presently, the only feasible clinical strategy in very early SSc remains a tight follow-up program to detect in "real time" the onset of internal organ involvement, which may thus allow an aggressive therapeutic agenda.


Assuntos
Escleroderma Sistêmico , Autoanticorpos , Diagnóstico Precoce , Humanos , Angioscopia Microscópica , Doença de Raynaud/etiologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico
9.
Dtsch Med Wochenschr ; 144(25): 1778-1783, 2019 12.
Artigo em Alemão | MEDLINE | ID: mdl-31847013

RESUMO

Maurice Raynaud first described color changes and symptoms of the fingers due to cold-induced vasospasm and restricted blood flow in his medical school thesis in 1862. Raynaud's phenomenon is common and exists as an uncomplicated primary Raynaud phenomenon and a Raynaud phenomenon secondary to underlying diseases and medication. Mechanisms contributing to altered vasoconstrictor activity are endothelial and not-endothelial. Cold-induced vasospasm is probably a thermoregulatory problem and effects are mediated by sympathetic activity and selective stimulation of alpha2c-adrenoreceptors.


Assuntos
Médicos/história , Doença de Raynaud , Dedos/fisiopatologia , França , História do Século XIX , Humanos , Masculino , Doença de Raynaud/história , Doença de Raynaud/fisiopatologia
10.
Rev Assoc Med Bras (1992) ; 65(10): 1314-1320, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31721965

RESUMO

OBJECTIVE: To review articles that evaluated the prevalence of Raynaud's phenomenon of occupational origin. METHODS: The search for articles was carried out in the Medline (via PubMed), Embase, Web of Science, Scientific Electronic Library Online (SciELO), and Latin America and Caribbean Health Sciences Literature (Lilacs) databases. RESULTS: 64 articles were obtained from the electronic search; 18 articles met the eligibility criteria. All studies discussed the exposure to vibrations in the upper limbs. In 6 of them, the thermal issue was directly or indirectly addressed. No studies have addressed exposure to vinyl chloride. CONCLUSIO: In general, a higher prevalence of Raynaud's phenomenon was found among vibratory tool operators compared to non-exposed workers, with an increase in the number of cases the higher the level of vibration and the time of exposure. Cold is a triggering and aggravating factor of the Raynaud phenomenon and seems to play an important role in the emergence of vascular manifestations of the hand-arm vibration syndrome.


Assuntos
Doenças Profissionais/etiologia , Exposição Ocupacional/efeitos adversos , Doença de Raynaud/etiologia , Temperatura Baixa/efeitos adversos , Síndrome da Vibração do Segmento Mão-Braço/complicações , Humanos , Doenças Profissionais/epidemiologia , Prevalência , Doença de Raynaud/epidemiologia , Fatores de Risco , Cloreto de Vinil/efeitos adversos
11.
Br J Hosp Med (Lond) ; 80(11): 658-664, 2019 Nov 02.
Artigo em Inglês | MEDLINE | ID: mdl-31707892

RESUMO

Raynaud's phenomenon is a common vasospastic condition which carries a significant burden of pain and hand-related disability (Hughes and Herrick, 2016). The prevalence of Raynaud's phenomenon in the general population has been reported to be approximately 5% (Garner et al, 2015). Raynaud's phenomenon can occur either as a primary ('idiopathic') phenomenon or secondary to a wide range of underlying medical conditions and drug causes. Therefore, hospital-based specialists are frequently involved in the care of patients with Raynaud's phenomenon and need to be aware of associated conditions and prescribed medications for Raynaud's phenomenon. In particular, Raynaud's phenomenon is often the earliest manifestation of an underlying autoimmune connective tissue disease (e.g. systemic sclerosis). A comprehensive clinical assessment is required including performing targeted investigations (e.g. nailfold capillaroscopy and systemic sclerosis-associated autoantibodies). Patient education and lifestyle adaptations is first-line treatment for Raynaud's phenomenon. There is a wide range of pharmacological options including oral and intravenous drug therapies available to treat Raynaud's phenomenon. Surgical intervention is sometimes required for refractory Raynaud's phenomenon and tissue ischaemia. This review describes the clinical manifestations of Raynaud's phenomenon including potential secondary causes and presents an approach to assessment and management.


Assuntos
Doença de Raynaud/etiologia , Testes Diagnósticos de Rotina , Dedos/irrigação sanguínea , Humanos , Isquemia/etiologia , Anamnese/métodos , Exame Físico/métodos , Doença de Raynaud/diagnóstico , Doença de Raynaud/terapia , Escleroderma Sistêmico/complicações , Úlcera Cutânea/complicações , Termografia/métodos
12.
Z Rheumatol ; 78(10): 967-978, 2019 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-31712897

RESUMO

Raynaud's phenomenon (RP) is a frequent and painful vasospasm of small arteries localized in acral body regions (most frequently the fingers). The more frequent so-called primary RP is caused merely by a functional dysregulation of the tonus of vascular walls. In contrast, the rarer secondary RP is additionally associated with structural abnormalities of blood vessels. Knowledge of RP is important for rheumatologists because secondary RP can be associated with the presence or development of severe underlying diseases, especially with systemic sclerosis. Thus, the rheumatologist has to be aware of this condition. In this article the diagnostic procedures and the most important treatment approaches are summarized.


Assuntos
Doença de Raynaud , Escleroderma Sistêmico , Humanos , Doença de Raynaud/complicações , Doença de Raynaud/terapia , Reumatologistas , Escleroderma Sistêmico/complicações
13.
Biomed Res Int ; 2019: 7908793, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31687398

RESUMO

The earliest clinical manifestation of SSc is usually Raynaud's phenomenon, a small-arteries vasospasm driven by vascular tone dysregulation and microcirculatory abnormalities, resulting in digital ulcers (DU) in up to 50% of patients. Many cytokines as well as growth factors have been shown to play a role in promoting vascular smooth muscle cell proliferation and fibroblast activation, leading to ischemic damage as well as skin fibrosis. We aim to investigate a possible difference in venous and arterial blood levels of many cytokines (Th1- and Th17-related), GM-CSF, and endothelin-1 (ET1) in patients with and without DU. In the same patients, the correlations between capillary damage, evaluated by nailfold videocapillaroscopy (NVC), extension of skin fibrosis, calculated by modified Rodnan skin score (mRSS), and cytokines, ET-1, and GM-CSF levels were also measured. Patients with DU showed venous levels of IL-1ß (p=0.024), IL-6 (p=0.012), IL-22(p=0.006), and TGF-ß (p=0.046) significantly higher compared to arterial levels and arterial levels of GM-CSF and TNF-alpha significantly higher compared to venous levels (p < 0.001). NVC abnormalities were correlated with arterial TNFa and venous IL22, IL23, and IL17 levels and negatively correlated with venous ET-1 levels, whereas mRSS showed a negative correlation with IL-21(ρ = -0.427, p=0.050). The increased Th17-cytokine levels in venous compared to arterial blood of patients with DU suggest local cytokine production on ulcer site. The higher TNFa and GM-CSF levels in arterial blood of DU patients support the attempt to mitigate the hypoxic damage, and the correlation between Th17-cytokines, mRSS, NVC, and ET1 agrees with the potent profibrotic stimulus at the onset of the disease, which decreases as the SSc progresses.


Assuntos
Artérias/metabolismo , Citocinas/sangue , Doença de Raynaud/sangue , Úlcera Cutânea/sangue , Células Th1/metabolismo , Células Th17/metabolismo , Veias/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Capilares/metabolismo , Citocinas/metabolismo , Endotelina-1/metabolismo , Feminino , Fibrose/metabolismo , Humanos , Masculino , Microcirculação/fisiologia , Angioscopia Microscópica/métodos , Pessoa de Meia-Idade , Doença de Raynaud/metabolismo , Pele/metabolismo , Úlcera Cutânea/metabolismo , Fator de Necrose Tumoral alfa/metabolismo
14.
Bratisl Lek Listy ; 120(11): 827-831, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31747762

RESUMO

BACKGROUND: This study was aimed to reveal whether there is a relationship between Raynaud Phenomenon (RP) and Helicobacter Pylori (HP). MATERIAL AND METHODS: Seventy-nine patients, who had been referred to outpatient clinic with Raynaud in the last 9 years were retrospectively screened. Of these, 29 patients with access to their data and who had HP screening tests were included in the study. RESULTS: HP direct antigen was found in feces in only one of 29 patients. When we compared the results of this study to the results of previous literature, it was observed that the patients admitted with RP symptoms by a gastroenterology outpatient clinic had a higher incidence for HP (+) scanning than the patients admitted with RP symptoms by a cardiovascular surgery policlinic (CVSOC). CONCLUSION: Although previous literature reports that HP leads to RP in the group of patients referred to other outpatient clinics, the same relation is very low in the Raynaud patient group in CVSOC. Patients admitted with RP in the CVSOC shouldn't be prescribed empirical antibiotic therapy for the eradication of HP. However, as the appropriate antibiotic regimen can resolve Raynaud symptoms in the presence of a HP(+) test, it makes this scanning rational for all symptomatic patients (Tab. 1, Fig. 1, Ref. 23).


Assuntos
Infecções por Helicobacter/complicações , Doença de Raynaud/complicações , Helicobacter pylori , Humanos , Doença de Raynaud/microbiologia , Estudos Retrospectivos
16.
Pediatr Rheumatol Online J ; 17(1): 68, 2019 Oct 16.
Artigo em Inglês | MEDLINE | ID: mdl-31619252

RESUMO

BACKGROUND: Infrared Thermography (IRT) has been used for over 30 years in the assessment of Raynaud Phenomenon (RP) and other peripheral microvascular dysfunctions in adults but, to date, very little experience is available on its use in children for this purpose. The first aim of the study was to assess reproducibility of thermographic examination after cold exposure by comparing inter-observer agreement in thermal imaging interpretation. The secondary aim was to evaluate whether IRT is reliable to diagnose and differentiate peripheral circulation disturbances in children. METHODS: Children with clinical diagnosis of primary Raynaud's phenomenon (PRP), secondary RP (SRP), acrocyanosis (AC) and age-matched controls underwent sequential measurements of skin temperature at distal interphalangeal (DIP) and metacarpophalangeal (MCP) joints with IRT at baseline and for 10 min after cold challenge test. Intraclass correlation coefficient (ICC) was calculated for inter-rater reliability in IRT interpretation, then temperature variations at MCP and DIP joints and the distal-dorsal difference (DDD) were analysed. RESULTS: Fourteen PRP, 16 SRP, 14 AC and 15 controls entered the study. ICC showed excellent agreement (> 0.93) for DIPs and MCPs in 192 measures for each subject. Patients with PRP, SRP and acrocyanosis showed significantly slower recovery at MCPs (p < 0.05) and at DIPs (p < 0.001) than controls. At baseline, higher temperature at DIPs and lower at MCPs was observed in PRP compared with SRP with significantly lower DDD (p < 0.001). Differently from AC, both PRP and SRP showed gain of temperature at DIPs and less at MCPs after cold challenge. PRP but not SRP patients returned to DIPs basal temperature by the end of re-warming time. Analysis of DDD confirmed that controls and PRP, SRP and AC patients significantly differed in fingers recovery pattern (p < 0.05). CONCLUSION: IRT appears reliable and reproducible in identifying children with peripheral microvascular disturbances. Our results show that IRT examination pointed out that PRP, SRP and AC patients present significant differences in basal extremities temperature and in re-warming pattern after cold challenge therefore IRT can be suggested as an objective tool for diagnosis and monitoring of disease.


Assuntos
Doenças Vasculares Periféricas/diagnóstico , Doença de Raynaud/diagnóstico , Termografia/métodos , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Raios Infravermelhos , Masculino , Microvasos/patologia , Doenças Vasculares Periféricas/patologia , Doença de Raynaud/patologia , Reprodutibilidade dos Testes , Temperatura Cutânea
17.
Minerva Cardioangiol ; 67(5): 392-398, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31637898

RESUMO

BACKGROUND: Raynaud syndrome (RS) is associated with vasospasm of the hand and fingers as a response to cold or stress. RS may cause discomfort and color changes (pallor, cyanosis, erythema, as single symptoms, but usually in combination, localized to one or more fingers). The aim of this 4-week registry study was the evaluation of subjects with mild, primary RS and their treatment with a standard management (SM) plan in comparison with SM associated with supplementation with Pycnogenol®. METHODS: A group of 67 females with mild, primary RS was included. All subjects were working in shops with refrigerators. No skin lesions were present. The age range was between 30 and 40; the vasospastic changes were symmetrical; no other physical findings were present. RESULTS: The two groups, receiving standard management (N.=33) or SM+Pycnogenol®, 100 mg/day, (N.=34) were comparable at inclusion. Considering the main symptoms, the decrease in coldness, burning pain, paresthesias and irregular color changes was more significant with Pycnogenol® (P<0.05) at 4 weeks. At thermography, low temperature areas and discrepancies in temperature and color were decreased with Pycnogenol® more than in controls (P<0.05). In the Pycnogenol® group, one subject (2.94%) decided to use drug treatment (PGE1) in 4 weeks in comparison with 5 controls (15.15%). The lowest finger temperature improved from 20.3° C at inclusion to 26.4° C at 4 weeks (+30.04%) with Pycnogenol® in comparison with lower values (from 20.5 to 23.1 [+12.7%] in controls [P<0.05]). The fingertip skin flux increased significantly (+ 1.55 flux units) with Pycnogenol® (P<0.05), in controls just by +0.14 (ns). Supplementation with Pycnogenol® decreased oxidative stress and increased transcutaneous oxygen pressure (TcPO2) more than in controls. Compliance and tolerability were optimal. CONCLUSIONS: This pilot registry study suggests that Pycnogenol® may be used with significant advantages in primary, mild RS. Subjects using Pycnogenol® may control their symptoms and may avoid the need for more complex and, potentially dangerous or expensive treatments.


Assuntos
Antioxidantes/uso terapêutico , Suplementos Nutricionais , Flavonoides/uso terapêutico , Extratos Vegetais/uso terapêutico , Doença de Raynaud/tratamento farmacológico , Adulto , Feminino , Humanos , Projetos Piloto , Índice de Gravidade de Doença , Síndrome
18.
Turk J Med Sci ; 49(5): 1444-1449, 2019 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-31651110

RESUMO

Background/aim: Raynaud's phenomenon (RP) is not a rare health problem; global prevalence is about 3%­20%. Etiology and pathophysiology of this pathology has not been clarified. There are many precipitating factors resulting in RP. Hyperhomocysteinemia resulting from methylenetetrahydrofolate reductase (MTHFR) gene mutationmay have a role in its etiology. The aim of this study was to observe the frequency of RP in patients with MTFHR gene mutation and hyperhomocysteinemia. Possible relationships among vitamin B12, folic acid, complete blood count (leukocytes and platelets), and c-reactive protein levels and RP were also analyzed. Materials and methods: A total of 388 patients admitted to the internal medicine, hematology, and obstetric clinics of a university hospital between January 2012 and April 2013 ranging in age from 21 to 83 (mean age 38.16 ± 13.1) were enrolled in the study. Eighty-five (21.9%) of the patients were male and 303 (78.1%) were female. MTHFR gene mutation was analyzed in 388 patients; 52 (13.4%) were homozygous, 275 (70.9%) were heterozygous, and 61 (15.7%) were found to be negative for the MTHFR gene mutation and accepted as a control group. Vitamin B12, folic acid, complete blood count (leukocytes and platelets), and c-reactive protein levels were also analyzed. Results: Homocysteine levels were higher in both heterozygous and homozygous groups (P < 0.05). RP was more frequently observed in patients with elevated homocysteine levels (P < 0.05; X2 = 14.51). There was no significant relationship in other parameters studied. Conclusion: RP was more frequently observed in the groups with the MTHFR mutation and hyperhomocysteinemia. Serum homocysteine levels in patients with RP may be helpful for diagnosis.


Assuntos
Hiper-Homocisteinemia/complicações , Hiper-Homocisteinemia/genética , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Mutação , Doença de Raynaud/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Doença de Raynaud/epidemiologia , Adulto Jovem
19.
Med Sci Monit ; 25: 7391-7395, 2019 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-31576813

RESUMO

BACKGROUND We introduce a minimally invasive technique for the treatment of Raynaud disease - CT-guided percutaneous thoracic sympathetic chain radiofrequency thermocoagulation. MATERIAL AND METHODS Under CT guidance, the radiofrequency needle was punctured from the upper edge of the costotransverse joint to the anterior superior edge of the 4th capitulum costae and the lateral parietal pleura. After sensorial (1.5 mA, 50 Hz) and motorial (1.5 mA, 2Hz) testing to determine that there was no nerve innervation zone with muscle numbness and twitches, radiofrequency coagulation was set at 95°C for 300 s. RESULTS A total of 17 patients were enrolled in the treatment group. All the patients underwent CT-guided percutaneous thoracic sympathetic chain puncture of the needles to the upper edge of the 4th capitulum costae on both sides. The perfusion index (PI) of the fingers began to rise 30 s after radiofrequency thermocoagulation, and the palm temperature (T) began to rise after 90 s. At the end of treatment, PI increased by an average of 4.6-fold, and the T average rose by 3.6°C. Postoperative cold-water stimulation testing could no longer induce Raynaud disease. Follow-up was conducted for 1 to 15 months. Two patients were found to have recurrence at 9 months and 13 months, respectively. CONCLUSIONS CT-guided percutaneous thoracic sympathetic nerve chain radiofrequency coagulation can effectively treat Raynaud disease.


Assuntos
Eletrocoagulação/métodos , Terapia por Radiofrequência/métodos , Doença de Raynaud/terapia , Adulto , China , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Ondas de Rádio , Tomografia Computadorizada por Raios X , Resultado do Tratamento
20.
Lakartidningen ; 1162019 Sep 26.
Artigo em Sueco | MEDLINE | ID: mdl-31573670

RESUMO

Systemic sclerosis is an autoimmune systemic disease with an annual incidence in Sweden of only 20 cases per million and a standardised mortality rate of 3-4. Disease onset is usually preceded by a period with Raynaud's phenomenon, combined with structurally abnormal nailbed capillaries and accompanied by presence of scleroderma related autoantibodies. The presenting symptoms are skin thickness, puffy fingers, digital ulcers, dysphagia, joint stiffness and pain, and pruritus. Optimal management involves a number of specialists including allied health professionals. Early recognition, diagnosis and treatment are important. The dominating causes of death are cardiopulmonary.


Assuntos
Escleroderma Sistêmico , Autoanticorpos/imunologia , Humanos , Atenção Primária à Saúde , Doenças Raras/complicações , Doenças Raras/diagnóstico , Doenças Raras/patologia , Doenças Raras/terapia , Doença de Raynaud/etiologia , Encaminhamento e Consulta , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia
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