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5.
Lupus ; 27(11): 1860-1863, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30028256

RESUMO

A 60-year-old female was diagnosed with progressive systemic sclerosis and interstitial lung disease of two months' duration. The patient was treated for Raynaud phenomenon with aspirin, nifedipine, colchicine, and naproxen. Two weeks after treatment, she developed widespread erythematous patches with blistering eruptions on the face, torso, and extremities, and also had erosion on the oral mucosa. Skin biopsy for histopathology and direct immunofluorescent studies were suggestive of lupus erythematosus. To the best of our knowledge, this is the first case of toxic epidermal necrolysis-like acute cutaneous lupus erythematosus in a patient with progressive systemic sclerosis.


Assuntos
Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Cutâneo/patologia , Escleroderma Sistêmico/complicações , Pele/patologia , Diagnóstico Diferencial , Feminino , Humanos , Pessoa de Meia-Idade , Doença de Raynaud/patologia , Síndrome de Stevens-Johnson/patologia
6.
Microvasc Res ; 118: 173-177, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29605552

RESUMO

OBJECTIVES: Despite increasing interest in nailfold capillaroscopy, objective measures of capillary structure and blood flow have been little studied. We aimed to test the hypothesis that structural measurements, capillary flow, and a combined measure have the predictive power to separate patients with systemic sclerosis (SSc) from those with primary Raynaud's phenomenon (PRP) and healthy controls (HC). METHODS: 50 patients with SSc, 12 with PRP, and 50 HC were imaged using a novel capillaroscopy system that generates high-quality nailfold images and provides fully-automated measurements of capillary structure and blood flow (capillary density, mean width, maximum width, shape score, derangement and mean flow velocity). Population statistics summarise the differences between the three groups. Areas under ROC curves (AZ) were used to measure classification accuracy when assigning individuals to SSc and HC/PRP groups. RESULTS: Statistically significant differences in group means were found between patients with SSc and both HC and patients with PRP, for all measurements, e.g. mean width (µm) ±â€¯SE: 15.0 ±â€¯0.71, 12.7 ±â€¯0.74 and 11.8 ±â€¯0.23 for SSc, PRP and HC respectively. Combining the five structural measurements gave better classification (AZ = 0.919 ±â€¯0.026) than the best single measurement (mean width, AZ = 0.874 ±â€¯0.043), whilst adding flow further improved classification (AZ = 0.930 ±â€¯0.024). CONCLUSIONS: Structural and blood flow measurements are both able to distinguish patients with SSc from those with PRP/HC. Importantly, these hold promise as clinical trial outcome measures for treatments aimed at improving finger blood flow or microvascular remodelling.


Assuntos
Capilares/patologia , Capilares/fisiopatologia , Microcirculação , Angioscopia Microscópica/métodos , Unhas/irrigação sanguínea , Doença de Raynaud/diagnóstico , Escleroderma Sistêmico/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Automação , Velocidade do Fluxo Sanguíneo , Estudos de Casos e Controles , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Doença de Raynaud/patologia , Doença de Raynaud/fisiopatologia , Fluxo Sanguíneo Regional , Reprodutibilidade dos Testes , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/fisiopatologia , Adulto Jovem
8.
Microvasc Res ; 113: 22-28, 2017 09.
Artigo em Inglês | MEDLINE | ID: mdl-28450106

RESUMO

OBJECTIVES: Raynaud's phenomenon (RP) can be the first manifestation of systemic sclerosis (SSc) or other connective tissue diseases (CTDs), often preceding an overt disease by years. It is not known if markers of endothelial damage are detectable in those RP patients who subsequently develop a CTD. METHODS: We studied 82 RP patients at their first evaluation to correlate the levels of endothelial markers with the subsequent development of an overt disease 36months later. We measured plasma levels of tissue-type plasminogen activator (t-PA) and von Willebrand factor (vWF), two markers of endothelial damage, and interleukin-6 (IL-6), a pro-inflammatory cytokine. Thirty sex- and age-matched healthy subjects (HS) served as controls. RESULTS: At baseline, 67 patients showed capillaroscopic normal pattern (CNP) and 15 patients, of which 11 were very early SSc, had capillaroscopic scleroderma pattern (CSP). Plasma levels of t-PA, vWF and IL-6 were higher in patients with CNP (p=0.0001) than in HS and even much higher in patients with CSP (p=0.0001). In patients with CNP and RP of recent onset (<18months), vWF plasma levels were higher when autoantibodies were present (p=0.020). After 36months, among 48 RP patients with CNP who remained in follow-up, 24 were diagnosed as primary and 24 as secondary RP. In secondary RP, basal levels of t-PA, IL-6 and particularly vWF were higher than in primary RP (p=0.005, p=0.004, p=0.0001 respectively) and HS (p=0.0001 for all). CONCLUSIONS: Our findings indicate that markers of endothelial damage are elevated in RP patients who subsequently develop SSc or other CTDs, even in the absence of capillaroscopic abnormalities.


Assuntos
Células Endoteliais/metabolismo , Interleucina-6/sangue , Doença de Raynaud/sangue , Ativador de Plasminogênio Tecidual/sangue , Fator de von Willebrand/metabolismo , Adulto , Idoso , Área Sob a Curva , Autoanticorpos/sangue , Biomarcadores/sangue , Estudos de Casos e Controles , Diagnóstico Precoce , Células Endoteliais/patologia , Feminino , Humanos , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Curva ROC , Doença de Raynaud/patologia , Fatores de Tempo , Regulação para Cima , Adulto Jovem
11.
Clin Rheumatol ; 36(7): 1637-1642, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27722973

RESUMO

This study aimed to detect by nailfold videocapillaroscopy (NVC) the presence of age-related capillary morphological patterns in a large cohort of subjects affected by primary Raynaud's phenomenon (PRP). NVC was performed in 877 patients affected by PRP, divided into three age groups: <35, 35-55 and >55 years. The following qualitative parameters were assessed and compared in the three groups of patients: apical dilations, irregular (non-homogeneous) dilations, venous branch dilations, microhaemorrhages, tortuosities and subpapillary venous plexus visibility. Patients with either irregular dilations or venous branch dilations were found significantly younger than those without (p < 0.0001). The presence of either irregular or venous branch dilations seems to exclude the presence of apical dilations. Patients with microhaemorrhages were found significantly younger than those without (p = 0.05), and 81 % of patients without microhaemorrhages did not show irregular and venous branch dilations. The subpapillary venous plexus seems more visible in subjects with age < 35, as well as in those with age > 55 years (p < 0.0001). A statistically significant negative correlation was found between presence of apical and irregular dilations (p < 0.0001), apical dilations and venous branch dilations (p = 0.02), apical dilations and tortuosities (p = 0.0005), microhaemorrhages and tortuosities (p < 0.0001) and venous branch dilations and tortuosities (p = 0.02). Finally, a statistically significant positive correlation was found between irregular and venous branch dilations (p < 0.0001), irregular dilations and microhaemorrhages (p < 0.0001) and venous branch dilations and microhaemorrhages (p < 0.0001). In conclusion, our study detected different age-related morphological capillary changes mainly in younger patients with PRP, as well as statistically significant correlations between the presence of different capillary variables.


Assuntos
Capilares/patologia , Angioscopia Microscópica/métodos , Unhas/irrigação sanguínea , Doença de Raynaud/patologia , Adulto , Fatores Etários , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
12.
J Microbiol Immunol Infect ; 50(6): 857-862, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26869017

RESUMO

BACKGROUND AND AIM: Raynaud's phenomenon (RP) is a microvascular disorder characterized by episodic peripheral vasospasm and ischemia and is commonly found in patients with autoimmune diseases (AID). The vasomotor homoeostasis and endothelial cells damage are involved in RP. Endothelial microparticles (EMPs) may act as a biomarker for endothelial damage. The aim of this study is to investigate the correlation between the levels of microparticles (MPs) and microvasculopathy in AID with RP. METHODS: Thirty-seven patients with AID and RP (RP group) and 27 patients with AID but without RP (non-RP group) were enrolled. The microvasculopathy score of RP was graded by nailfold capillary microscopy. The plasma levels of MPs were measured by flow cytometry utilizing specific labels for endothelial MPs (CD105 and CD144) and annexin V staining for phosphatidylserine bearing-MPs (annexin V+MPs). RESULTS: The levels of circulating EMPs (CD105+ p = 0.005, CD144+ p = 0.004), and the annexin V+ MPs (p < 0.001) were significantly elevated in the RP group compared with the non-RP group. Moreover, the high microvasculopathy scores were closely related with annexinV+ MPs levels in the RP group (p = 0.041). CONCLUSIONS: Levels of circulating EMPs and annexin V+ MPs are elevated in AID patients with RP indicate the endothelial damage and endothelial dysfunctions. In addition, levels of annexin V+ MPs can predict the severity of microvasculopathy in AID with RP.


Assuntos
Anexina A5/sangue , Doenças Autoimunes/patologia , Micropartículas Derivadas de Células/patologia , Células Endoteliais/patologia , Microvasos/patologia , Doença de Raynaud/patologia , Antígenos CD/sangue , Doenças Autoimunes/sangue , Doenças Autoimunes/complicações , Biomarcadores/sangue , Caderinas/sangue , Endoglina/sangue , Feminino , Humanos , Microvasos/citologia , Pessoa de Meia-Idade , Doença de Raynaud/sangue , Doença de Raynaud/complicações
13.
J Med Case Rep ; 10(1): 356, 2016 Dec 20.
Artigo em Inglês | MEDLINE | ID: mdl-27998297

RESUMO

BACKGROUND: Raynaud's phenomenon is a microvascular disorder that results in exaggerated vasoconstriction over vasodilatation secondary to an alteration in autonomic control. Though benign, it can result in severe ulceration and ultimately gangrene associated with disfiguration and permanent deformity. We present a case of severe secondary Raynaud's phenomenon in a black-African patient from a resource-limited setting, with focus on the difficulties encountered in the diagnosis and treatment. CASE PRESENTATION: A 43-year-old female Cameroonian farmer with a 7-year history of episodic paresthesia in her fingers and toes (when exposed to cold) presented to our emergency department with severe pain, ulceration, and "darkening" of her fingertips over a period of 2 days. An examination revealed bilateral ulceration and dry gangrene of her fingers and toes, based on which a diagnosis of secondary Raynaud's phenomenon due to a connective tissue disease was proposed. Results of paraclinical investigations were normal. Lifestyle modification along with a calcium channel blocker and phosphodiesterase type 5 inhibitor provided significant relief. CONCLUSIONS: An early diagnosis and knowledge on appropriate treatment of Raynaud's phenomenon is of vital importance to prevent permanent tissue damage and disability. Relying on biphasic color change for the diagnosis of Raynaud's phenomenon in black Africans can be potentially misleading.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Aconselhamento Diretivo , Dedos/patologia , Microcirculação/efeitos dos fármacos , Doença de Raynaud/patologia , Comportamento de Redução do Risco , Dedos do Pé/patologia , Adulto , Grupo com Ancestrais do Continente Africano , Analgésicos Opioides/uso terapêutico , Antibacterianos/uso terapêutico , Bloqueadores dos Canais de Cálcio/uso terapêutico , Cloxacilina/uso terapêutico , Doenças do Tecido Conjuntivo/patologia , Feminino , Gangrena , Humanos , Estilo de Vida , Nifedipino/uso terapêutico , Inibidores da Fosfodiesterase 5/uso terapêutico , Doença de Raynaud/complicações , Doença de Raynaud/terapia , Índice de Gravidade de Doença , Citrato de Sildenafila/uso terapêutico , Tramadol/uso terapêutico , Resultado do Tratamento
14.
Reumatismo ; 68(2): 109-11, 2016 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-27608801

RESUMO

Systemic sclerosis (SSc) is a connective tissue disease frequently associated with Raynaud's Phenomenon (RP). Among possible pharmacological treatments, phosphodiesterase 5 inhibitors are considered in cases of severe non -responsive RP. We present the case of a male SSc patient wh presented with critical finger ischemia and concomitant appearance of myocardial fibrosis after sudden interruption of sildenafil treatment.


Assuntos
Antirreumáticos/efeitos adversos , Cardiomiopatias/patologia , Dedos/irrigação sanguínea , Doença de Raynaud/tratamento farmacológico , Escleroderma Sistêmico/tratamento farmacológico , Citrato de Sildenafila/efeitos adversos , Síndrome de Abstinência a Substâncias , Antirreumáticos/uso terapêutico , Cardiomiopatias/etiologia , Humanos , Isquemia , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Doença de Raynaud/complicações , Doença de Raynaud/patologia , Fatores de Risco , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/patologia , Citrato de Sildenafila/uso terapêutico , Fatores de Tempo
15.
Clin Rheumatol ; 35(10): 2591-6, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27562035

RESUMO

CXCL4 is an antiangiogenic and immunomodulatory chemokine. We aimed to investigate serum levels of CXCL4 in primary Sjögren's syndrome (pSS), looking for associations with disease features. Thirty-nine consecutive pSS patients underwent clinical-serological assessment and nailfold videocapillaroscopy (NVC). Thirty-six patients and 30 controls affected by osteoarthritis were also investigated for serum levels of CXCL4 and soluble E-selectin (sE-selectin). CXCL4 was higher in pSS patients than in controls (1.79 [0.2-11.18] vs 1.023 ng/ml [0.02-14.45], p < 0.05), particularly in those without anti-La/SSB antibodies (2.89 [1.01-11.18] vs 1.69 ng/ml [0.2-2.72], p < 0.05), while it was lower in pSS patients with a focus score ≥1 at lip biopsy (1.44 [0.86-2.1] vs 2.24 ng/ml [1.64-3.25], p < 0.05) and clinically evident lymphadenopathy (1.53 [0.38-1.7] vs 2.08 ng/ml [1.45-3.03], p < 0.05). CXCL4 correlated with disease duration (r = 0.35, p < 0.05) and sE-selectin (r = 0.45, p < 0.01). Patients with Raynaud's phenomenon (RP) had more frequently abnormal CXCL4 levels than patients without RP (11/15 vs 3/21, p < 0.001), enlarged capillaries (14/16 vs 7/23, p < 0.001) and capillary loss at NVC (14/16 vs 6/23, p < 0.001). The hitherto unknown association of increased serum CXCL4 with features of microvascular impairment in pSS, along with the negative association with features of lymphocytic response (i.e., the absence of subset disease-specific autoantibodies, a low focus score, and the absence of lymphadenopathy) suggest clarifying the possible implication of this chemokine in pSS pathogenesis in larger studies.


Assuntos
Capilares/patologia , Linfonodos/patologia , Fator Plaquetário 4/sangue , Doença de Raynaud/sangue , Glândulas Salivares/patologia , Síndrome de Sjogren/sangue , Adulto , Idoso , Autoanticorpos/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Raynaud/etiologia , Doença de Raynaud/patologia , Síndrome de Sjogren/complicações , Síndrome de Sjogren/patologia
16.
AJR Am J Roentgenol ; 207(6): 1252-1256, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27575610

RESUMO

OBJECTIVE: The objectives of our study were to describe the MRI findings of pedal phalangeal bone marrow edema in patients with Raynaud phenomenon (RP) and discuss the clinical implications of these MRI findings. CONCLUSION: There is a progressive distal-to-proximal pattern of pedal phalangeal bone marrow edema on MRI in patients with RP. This knowledge may allow early diagnosis and treatment of rheumatologic disorders that are potentially associated with RP.


Assuntos
Doenças da Medula Óssea/diagnóstico por imagem , Medula Óssea/diagnóstico por imagem , Edema/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Doença de Raynaud/diagnóstico por imagem , Falanges dos Dedos do Pé/diagnóstico por imagem , Adolescente , Adulto , Medula Óssea/patologia , Doenças da Medula Óssea/etiologia , Doenças da Medula Óssea/patologia , Diagnóstico Diferencial , Edema/etiologia , Edema/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Raynaud/complicações , Doença de Raynaud/patologia , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Falanges dos Dedos do Pé/patologia
17.
Lupus ; 25(2): 214-6, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26377236

RESUMO

Pseudo-pseudo Meigs' syndrome (PPMS) has been reported to be a rare presentation of patients with systemic lupus erythematosus (SLE). However, such a presentation is not common in other forms of connective tissue disease. We presented a case of gross ascites, pleural effusion, and marked elevation of CA-125 level (PPMS-like features) that led to a diagnosis of MCTD. The patient responded to systemic steroid therapy.


Assuntos
Síndrome de Meigs/diagnóstico , Doença Mista do Tecido Conjuntivo/diagnóstico , Doença de Raynaud/patologia , Adulto , Anti-Inflamatórios/administração & dosagem , Diagnóstico Diferencial , Feminino , Humanos , Lúpus Eritematoso Sistêmico/patologia , Síndrome de Meigs/diagnóstico por imagem , Síndrome de Meigs/tratamento farmacológico , Síndrome de Meigs/patologia , Metilprednisolona/administração & dosagem , Doença Mista do Tecido Conjuntivo/tratamento farmacológico , Doença Mista do Tecido Conjuntivo/patologia , Prednisolona/administração & dosagem , Radiografia
18.
Int J Dermatol ; 55(1): 97-100, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25266415
20.
Clin Hemorheol Microcirc ; 63(4): 335-347, 2016 Oct 05.
Artigo em Inglês | MEDLINE | ID: mdl-26639766

RESUMO

OBJECTIVE: To explore whether polymorphic variants of the HTR1B gene are associated with the susceptibility of Raynauds' Phenomenon (RP) coursed by vibration. METHODS: 148 subjects exposed to vibration for more than 2 years were classified into either induced white finger (VWF) group (n = 72), or non-VWF group (n = 76). Vibration exposure levels were measured and assessed following ISO 5349-1:2001 protocol. All workers were genotyped by sequencing for the single nucleotide polymorphisms (SNPs) in the 5'-flanking and coding region of HTR1B. Genetic characteristics and linkage disequilibrium (LD) were analyzed with Haploview. Serum serotonin levels of each subject were detected using ELISA. The association between the susceptibility of vascular damage and genotype was analyzed via logistic regression. RESULTS: 7 known SNPs were obtained and their allele frequencies were inserted into the Hardy-Weinberg equilibrium. rs6297 variant genotype had an increased risk of VWF compared with wild genotype (OR = 2.14, 95% CI = 1.04- 4.58, P < 0.05). rs6298 mutant type (AG+GG) was found to have a significant interaction on vibration exposure LN(CEI), accounting for VWF occurrence. LN(5-HT) level is significantly different between the VWF group (x¯±s= 1.99±1.09 ng/mL) and the non-VWF group (x¯±s= 2.72±1.47 ng/mL). CONCLUSIONS: Serotonin levels may affect the progression of secondary RP. Polymorphic variants of the HTR1B gene are associated with the susceptibility of secondary RP in vibration-exposed occupational populations of Chinese Han people.


Assuntos
Braço/patologia , Mãos/patologia , Doenças Profissionais/genética , Doença de Raynaud/genética , Receptor 5-HT1B de Serotonina/genética , Vibração/efeitos adversos , Adulto , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Profissionais/patologia , Polimorfismo de Nucleotídeo Único , Doença de Raynaud/patologia
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