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1.
Lupus ; 29(12): 1616-1622, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32605529

RESUMO

In this study, we aimed to evaluate long-term patient survival according to demographic data, clinical manifestations of systemic lupus erythematosus (SLE) and previous and current treatments, collected retrospectively. Patient selection required a minimum of four American College of Rheumatology revised criteria for SLE, biopsy-proven lupus nephritis (LN) available for reclassification according to the modified National Institutes of Health proposal for activity and chronicity indices and a minimum follow-up of at least three years since the last renal biopsy. Selection criteria were fulfilled in 25 patients followed for a median of 21 years. Based on the last renal biopsy, an equal number of patients were thus classified as class I/II and IV (n=8) and class III and V (n = 4). The mortality rate for LN was 14%. Having ever been diagnosed with glomerulonephritis (GN) type III or type IV but not class IV alone (p = 0.046), a higher histological chronicity index at the last renal biopsy (p = 0.022), not attaining renal remission one year after induction therapy (p = 0.004), end-stage renal disease on dialysis (p = 0.033) and the extra-renal Systemic Lupus International Collaborating Clinics Damage Index score (p = 0.017) were all significantly associated with mortality. Our results may provide important clues for strict observation protocols in particular categories of LN patients with long-standing disease.


Assuntos
Falência Renal Crônica/epidemiologia , Nefrite Lúpica/epidemiologia , Adolescente , Adulto , Anti-Infecciosos/uso terapêutico , Biópsia , Causas de Morte , Progressão da Doença , Feminino , Humanos , Falência Renal Crônica/mortalidade , Falência Renal Crônica/patologia , Nefrite Lúpica/tratamento farmacológico , Nefrite Lúpica/mortalidade , Nefrite Lúpica/patologia , Masculino , Portugal/epidemiologia , Indução de Remissão , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
3.
Acta Med Port ; 30(5): 368-372, 2017 May 31.
Artigo em Português | MEDLINE | ID: mdl-28865500

RESUMO

INTRODUCTION: Severity in systemic lupus erythematosus may vary from mild to even fatal consequences. There are no biomarkers to predict the disease's prognosis. The Systemic Lupus International Collaborating Clinics/ Systemic Damage Index defines systemic lupus erythematosus disease severity and is found to predict prognosis. OBJECTIVE: To test damage determinants in a single-centre systemic lupus erythematosus cohort. MATERIAL AND METHODS: Retrospectively followed systemic lupus erythematosus female patients (defined by the identification of at least four systemic lupus erythematosus American College of Rheumatology criteria - fulfillment 100%, n = 76) over the past five years. Age of onset, ethnicity, disease duration, number of American College of Rheumatology criteria at the end of follow-up, cumulative: renal, neuropsychiatric and articular phenotypes, hypertension, dyslipidaemia, smoking and Systemic Lupus Erythematosus Disease Activity Index 2K were correlated to the presence and degree of irreversible damage (Systemic Lupus International Collaborating Clinics Damage Index). Accumulation of American College of Rheumatology criteria was measured in a sub-group of patients followed from disease onset (within a year of the first symptom ascribed to systemic lupus erythematosus) (n = 39 - 51%); Systemic Lupus Erythematosus Disease Activity Index and Systemic Lupus International Collaborating Clinics Damage Index were performed. Statistical analysis was performed using Chi-square, Wilcoxon Mann-Whitney tests and Spearman correlation rho (Sig. 2-tailed p < 0.05). RESULTS: Systemic Lupus International Collaborating Clinics/Systemic Damage Index > 0 was present in 56.6% and significantly associated to a longer duration, a higher number of American College of Rheumatology criteria and a neuropsychiatric phenotype when compared with those with no damage. The final number of American College of Rheumatology criteria accrued was positively correlated to a higher disease activity over the past five years of follow-up (Spearman´s rho 0.02 and p < 0.05). There was no effect from other features. DISCUSSION AND CONCLUSION: Disease duration and number of American College of Rheumatology criteria predict Systemic Lupus International Collaborating Clinics/ Systemic Damage Index. neuropsychiatric disease has an impact on damage accrual.


Introdução: O lúpus eritematoso sistémico pode apresentar uma gravidade variável. Contudo, não existem biomarcadores que preveem o curso da doença. O dano é medido pelo índice Systemic Lupus International Collaborating Clinics/Systemic Damage Index que define a gravidade e prevê o seu prognóstico. Obetivo: Avaliação dos fatores que determinam dano nos doentes com lúpus eritematoso sistémico. Material e Métodos: Estudo retrospetivo, monocêntrico, em doentes com lúpus eritematoso sistémico (≥ 4 critérios do American College of Rheumatology ­ 100% dos doentes, n = 76), do sexo feminino, seguidos por um período ≥ 5 anos. Início da doença, etnia, duração, número de critérios American College of Rheumatology no final do seguimento, fenótipo renal, neuropsiquiátrico (e articular, co-morbilidades e Systemic Lupus Erythematosus Disease Activity Index -2K foram correlacionados com a presença e grau de dano medido pelo índice Systemic Lupus International Collaborating Clinics/Systemic Damage Index. A acumulação de critérios American College of Rheumatology foi objetivada num sub-grupo de doentes seguidos desde o início. A análise estatística utilizou o qui-quadrado, Wilcoxon Mann-Whitney e a correlação de Spearman (p < 0,05). Resultados: O Systemic Lupus International Collaborating Clinics Index era superior a 0 em 56,5% dos doentes. Estes doentes tinham um maior tempo de doença, um maior número de critérios American College of Rheumatology e um fenótipo neuropsiquiátrico, quando comparados com doentes sem dano (p < 0,05). Verificou-se uma correlação positiva entre o valor numérico de critérios American College of Rheumatology acumulados no final do seguimento e a atividade da doença nos últimos cinco anos (Spearman rho 0,02 e p < 0,05). Não se verificaram diferenças em relação às outras variáveis. Discussão e Conclusão: A duração da doença e o número de critérios do American College of Rheumatology acumulados conseguem prever a presença de dano. A doença neuropsiquiátrica teve impacto na morbilidade dos doentes com lúpus eritematoso sistémico, identificando um subgrupo em risco.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Adolescente , Adulto , Idoso , Criança , Feminino , Humanos , Pessoa de Meia-Idade , Morbidade , Prognóstico , Estudos Retrospectivos , Adulto Jovem
4.
BMJ Case Rep ; 20152015 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-26354836

RESUMO

We report a case of a 65-year-old man with systemic lupus erythematosus (SLE) and antiphospholipid syndrome, presenting palpable purpuric lesions, necrotic blisters and swelling ankles, after a previous tracheobronchitis episode. Laboratory data were remarkable for mild proteinuria and imaging studies were normal. A skin biopsy showed IgA deposits on superficial dermal capillaries and IgA vasculitis (IgAV) (former Henoch-Schönlein purpura) was assumed. The patient was treated with colchicine, deflazacort and azathioprine, but as a regression in the purpuric lesions was noted, a decline in renal function was detected. A kidney biopsy revealed mesangial proliferation with IgA deposition and IgAV nephritis was considered. Immunosuppressive treatment was adjusted, with progressive normalisation of renal function and disappearance of proteinuria over a monthly follow-up; after 6 months, total remission was achieved. To the best of our knowledge, this is the first reported case of IgAV in an adult patient with SLE.


Assuntos
Anti-Hipertensivos/administração & dosagem , Vasculite por IgA/diagnóstico , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico/patologia , Ramipril/administração & dosagem , Idoso , Humanos , Vasculite por IgA/tratamento farmacológico , Vasculite por IgA/imunologia , Vasculite por IgA/patologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Indução de Remissão
6.
ISRN Rheumatol ; 2012: 989218, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23227358

RESUMO

In order to establish if neuropsychiatric systemic lupus erythematosus (NPSLE) can be identified by any characteristic other than those used to diagnose the neuropsychiatric (NP) disease itself, we retrospectively reviewed 98 systemic lupus erythematosus (SLE) patients followed over a mean period of 10 years. NPSLE was identified in 22 patients. Stroke and generalized seizures were the most frequent NP manifestations. The NPSLE and non-NPSLE groups were similar with regard to demographic characteristics, ACR criteria, serum autoantibodies, and frequency of hypertension and hypercholesterolemia. Of note, compared to the non-NPSLE group, NPSLE was associated with a higher frequency of smoking (78 versus 26%), organ damage (73 versus 34%), and cumulative mortality rate (14 versus 7%). The series of patients was further analysed according to the presence of antiphospholipid syndrome (APS). Significantly, the interval between the onset of NP disease and SLE diagnosis was shorter in the APS(-) (0.3 ± 1 years) than in the APS(+) (5 ± 7 years) groups. Recurrence and/or persistence of NP events were only documented in the APS(-) group. Overall cumulative mortality was highest in NPSLE and in APS(+) patients with inadequate anticoagulation control, identifying an aspect that requires improved vigilance and the development of novel therapeutic modalities.

7.
BMJ Case Rep ; 20122012 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-22891010

RESUMO

Infective endocarditis can be associated with complex clinical presentations, sometimes with a difficult multi-disciplinary management. Actinobacillus actinomycetemcomitans belongs to the Haemophilus species, Actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens and Kingella species group, responsible for 5% to 10% of infective endocarditis in native heart valves. These organisms have slow fastidious growth pattern, often associated with negative cultures, and cause systemic embolism with abscess formation. The authors present the case of a 59-year-old man, admitted due to fever of unknown origin, with a personal history of obstructive hypertrophic cardiomyopathy and recent dental manipulation. The diagnosis of mitral valve's endocarditis was established after a transoesophageal ecocardiography, with a late isolation of A actinomycetemcomitans in blood culture. Despite the institution of antibiotic therapy, the patient suffered from multiple episodes of septic embolism: skin, mucosae, cerebral abscesses, spondylodiscitis and uveitis. He was submitted to heart surgery with miectomy and replacement of the native mitral valve by a mechanical prosthesis, while on antibiotics.


Assuntos
Aggregatibacter actinomycetemcomitans , Cardiomiopatia Hipertrófica/complicações , Endocardite Bacteriana/microbiologia , Doenças das Valvas Cardíacas/microbiologia , Valva Mitral , Infecções por Pasteurellaceae/complicações , Antibacterianos/uso terapêutico , Ecocardiografia Transesofagiana , Endocardite Bacteriana/diagnóstico , Endocardite Bacteriana/tratamento farmacológico , Febre de Causa Desconhecida/microbiologia , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Infecções por Pasteurellaceae/diagnóstico , Infecções por Pasteurellaceae/tratamento farmacológico
8.
BMJ Case Rep ; 20122012 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-22891014

RESUMO

Leprosy or Hansen's disease is a chronic granulomatous infectious disease caused by Mycobacterium leprae with a high prevalence in some developing countries however, it is rarely seen in non-endemic regions. Arthritis has been described in all types of Hansen's disease. Chronic arthritis is known to exist even in paucibacillary forms, resolved or treated disease and in patients without reaction, suggesting a perpetuated inflammatory process. In these cases leprosy can mimic some autoimmune diseases such as rheumatoid arthritis. When a patient with a history of leprosy presents with a symmetric, distal, polyarthritis the diagnosis may not be linear. Possibly it is a rheumatoid-like leprous arthritis with M leprae acting as the trigger element for the chronic process or it is an overlap condition, with a concomitant rheumatoid arthritis? A case report of a patient with a chronic inflammatory arthritis with 10 years of evolution is presented. The differential diagnosis between leprous and rheumatoid arthritis is discussed.


Assuntos
Artrite Infecciosa/diagnóstico , Artrite Reumatoide/diagnóstico , Hanseníase/complicações , Antirreumáticos/uso terapêutico , Artrite Infecciosa/microbiologia , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Diagnóstico Diferencial , Feminino , Humanos , Hansenostáticos/uso terapêutico , Hanseníase/tratamento farmacológico , Pessoa de Meia-Idade
10.
BMJ Case Rep ; 20122012 Apr 28.
Artigo em Inglês | MEDLINE | ID: mdl-22605004

RESUMO

Malignant lymphomas represent about 9% of cardiac neoplasms. Despite its life-threatening nature, the cardiac manifestations are often subclinical. In about 20% of deaths from lymphoma, cardiac involvement is found only in autopsy. The authors present the case of a 77-year-old female admitted due to intense back pain, vomiting, generalised pruritus, fatigue and weight loss. She had a personal history of hypertension and breast cancer was noted 10 years before admission. The thoracoabdominopelvic CT showed a mass in the left atrium with extension to the right atrium and inferior vena cava, and a paravertebral mass at D10-D11 with invasion of the spinal canal and hepatic hilum. The transthoracic paravertebral mass biopsy was compatible with a diffuse large B cell lymphoma. The patient developed a complete atrioventricular block, with haemodynamic instability, requiring urgent chemoreduction of the paracardiac mass and implantation of an epicardial pacemaker.


Assuntos
Bloqueio Cardíaco/diagnóstico , Bloqueio Cardíaco/etiologia , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/diagnóstico , Neoplasias do Mediastino/complicações , Neoplasias do Mediastino/diagnóstico , Idoso , Biópsia , Diagnóstico Diferencial , Feminino , Bloqueio Cardíaco/terapia , Humanos , Marca-Passo Artificial , Tomografia Computadorizada por Raios X
11.
BMJ Case Rep ; 20122012 Mar 08.
Artigo em Inglês | MEDLINE | ID: mdl-22605591

RESUMO

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder that is characterised by the presence of antiphospholipid antibodies and a common cause of vascular thromboembolic phenomena. The management of patients with APS is currently directed to antithrombotic medications. The international therapeutic guidelines recommend oral anticoagulation with warfarin indefinitely after the first thrombotic episode. However, therapeutic guidelines lack for a minority group of patients - the patients appropriately anticoagulated with recurrent thromboembolic phenomena. The authors present a clinical report that reveals the therapeutic and diagnostic complexity of this specific group of patients. Regarding recent studies, APS has been revealed as a complex syndrome with multiple pathophysiological mechanisms previously unknown. In this context, new therapeutic approaches have been defended and empirically experienced, with potentially promising results.


Assuntos
Síndrome Antifosfolipídica/complicações , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/etiologia , Trombose/diagnóstico , Trombose/etiologia , Anticorpos Antifosfolipídeos/análise , Broncoscopia , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Tomografia Computadorizada por Raios X
12.
BMJ Case Rep ; 20112011 Dec 21.
Artigo em Inglês | MEDLINE | ID: mdl-22670011

RESUMO

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a relatively rare entity. The therapeutic approach of patients with SAPHO syndrome has included multiple drugs with varying success and incoherence responses. The therapy is still empirical today. SAPHO syndrome is commonly treated with non-steroidal anti-inflammatory drugs, bisphophonates and non-biologic disease modifying antirheumatic drugs. Recent reports showed successful treatment with tumour necrosis factor α (TNF α) antagonists, but there is still a dark side of SAPHO syndrome including a subgroup of patient's refractory to all the treatments that have been empirically experienced. A clinical report of a patient with SAPHO syndrome with 12 years of evolution is described. All the therapeutic approaches, including anti TNF α therapy, have not prevented the clinical and radiographic progression of the disease. Given that the disease affects mostly younger patients, new therapeutic strategies are necessary in order to avoid potentially irreversible joint and bone lesions.


Assuntos
Síndrome de Hiperostose Adquirida , Síndrome de Hiperostose Adquirida/terapia , Adulto , Progressão da Doença , Humanos , Masculino
13.
BMJ Case Rep ; 20112011 Sep 13.
Artigo em Inglês | MEDLINE | ID: mdl-22679237

RESUMO

Buerger's disease or Thromboangiitis obliterans is a segmental inflammatory disease that affects the vessels and nerves of the extremities. It usually affects men below 45 years old and correlates with tobacco, as a predisposing factor. The authors present the case of a 34-year-old male, with ulcers in the fingertips with progressive worsening: acrocyanosis, slow healing, necrosis and finally loss of substance. Dorsalis pedis and posterior tibial pulses were not palpable. Personal history of heavy smoking was (20 pack-years). The angiography revealed proximal occlusion of the left posterior tibial and interosseal arteries, with distal circulation by the anterior tibial artery. He was submitted to disarticulation of the second left toe and therapy with pentoxifyline and iloprost infusion, calcium antagonist, antiplatelet drugs, statin and low molecular weight heparin (later replaced by oral anticoagulation). Improvement was seen of active vascular lesions and pain symptoms.


Assuntos
Tromboangiite Obliterante/diagnóstico , Tromboangiite Obliterante/tratamento farmacológico , Adulto , Angiografia , Aspirina/uso terapêutico , Atorvastatina , Bloqueadores dos Canais de Cálcio/uso terapêutico , Quimioterapia Combinada , Heparina de Baixo Peso Molecular/uso terapêutico , Ácidos Heptanoicos/uso terapêutico , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Iloprosta/uso terapêutico , Masculino , Nifedipino/uso terapêutico , Pentoxifilina/uso terapêutico , Inibidores da Agregação Plaquetária/uso terapêutico , Pirróis/uso terapêutico , Abandono do Hábito de Fumar
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