Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 118
Filtrar
1.
Cornea ; 38(7): 918-920, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30969261

RESUMO

PURPOSE: To describe a case of bilateral multifocal stromal crystalline keratopathy in the setting of relapsing polychondritis (RP). METHODS: Case report. RESULTS: We describe a 31-year-old woman who presented with ocular inflammation, bilateral auricular chondritis, and nasal chondritis, meeting the clinical criteria of RP. We illustrate her auricular cartilaginous abnormalities, saddle nose deformity, scleritis, and discrete mid-stromal opacities in both corneas that extend through the central cornea. Uniquely, her opacities feature a marked crystalline component as demonstrated on photography, anterior segment optical coherence tomography, and confocal microscopy. CONCLUSION: A central keratopathy is not typically reported in patients with RP. In this case report, we describe a unique diffuse bilateral nummular mid-stromal crystalline keratitis that extends into the central cornea and further define it using multimodal imaging.


Assuntos
Técnicas de Diagnóstico Oftalmológico , Policondrite Recidivante/diagnóstico por imagem , Adulto , Cristalinas/análise , Feminino , Humanos , Microscopia Confocal , Imagem Multimodal , Fotografação , Tomografia de Coerência Óptica
2.
Ann Thorac Surg ; 108(3): 897-904, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30910657

RESUMO

BACKGROUND: Placement of uncovered self-expandable metallic stents was found to successfully alleviate critical airflow limitation in patients with relapsing polychondritis (RP) with central airway involvement by several reports. However, the long-term outcome of airway metallic stenting in patients with RP remain unclear. METHODS: We retrospectively analyzed patients with RP who underwent airway metallic stenting with the use of fiberoptic bronchoscopy between September 1, 2009, and October 1, 2017, in Shanghai. Outcome measurements, including modified Medical Research Council (mMRC) dyspnea score, 6-minute walk distance (6MWD), spirometry, and bronchoscopic findings, as well as adverse events after stent placement, were collected. RESULTS: A total of 27 patients were included; the median patient age was 58 years (range: 41 to 74 years), and 19 were men (70.4%). Nineteen uncovered self-expandable metallic stents were placed in the trachea and 39 in the main bronchi. The median follow-up time was 50.5 months (range: 6 to 100 months). The baseline forced expiratory volume in 1 second (FEV1) percentage predicted (%pred), FEV1/forced vital capacity (FVC), and peak expiratory flow (PEF) was 24.2 ± 3.7, 27.2 ± 5.6, and 0.99 ± 0.21 L/min, respectively. One day after the procedure, improvement from baseline in FEV1 %pred, FEV1/FVC, and PEF was 17.9 ± 8.9 (p = 0.001), 19.8 ± 10.9 (p = 0.002), and 0.69 ± 0.44 L/min (p = 0.001), respectively. Changes in the following variables were also statistically and clinically significant: 6MWD of 193.7 ± 83.4 m; mMRC dyspnea score of -1.2 ± 0.4 points (both p < 0.05). The improvements were maintained at 5 years: a mean change in FEV1 %pred, FEV1/FVC, PEF, 6MWD, and mMRC score was 19.5 ± 6.7, 13.9 ± 5.0, 0.82 ± 0.40 L/min, 134.7 ± 66.2 m, and -0.83 ± 0.29 points, respectively. Cough, foreign body sensation, mucus production, and granulomas were common adverse events, occurring in 48.1% (13 of 27), 40.7% (11 of 27), 29.6% (8 of 27), and 25.9% (7 of 27) of the subjects. However, none of those complications were severe enough to require urgent bronchoscopic interventions. CONCLUSIONS: Airway metallic stenting in patients with RP with central airway involvement resulted in long-term clinical benefits in lung function, exercise tolerance, and dyspnea with an acceptable safety profile.


Assuntos
Obstrução das Vias Respiratórias/cirurgia , Broncoscopia/métodos , Policondrite Recidivante/diagnóstico por imagem , Policondrite Recidivante/cirurgia , Qualidade de Vida , Stents , Adulto , Obstrução das Vias Respiratórias/diagnóstico por imagem , Obstrução das Vias Respiratórias/etiologia , Estudos de Coortes , Feminino , Seguimentos , Humanos , Masculino , Metais , Pessoa de Meia-Idade , Segurança do Paciente , Policondrite Recidivante/complicações , Desenho de Prótese , Implantação de Prótese , Testes de Função Respiratória , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Capacidade Vital/fisiologia
3.
Sichuan Da Xue Xue Bao Yi Xue Ban ; 49(5): 803-807, 2018 Sep.
Artigo em Chinês | MEDLINE | ID: mdl-30378346

RESUMO

OBJECTIVE: To analyze the characteristics of clinical manifestation, imaging, fiber-bronchoscope finding and pulmonary function testing in the patients of relapsing polychodriti with respiratory symptoms. METHODS: The clinical data of 33 patients with RP in West China Hospital of Sichuan University from May 2007 to May 2017 were analyzed retrospectively. The patients were divided into respiratory group (21 cases) and non-respiratory group (12 cases) based on whether the respiratory symptoms were main involvement, and the data of the two groups were statistically analyzed. RESULTS: Among the 33 patients, the most vulnerable system was respiratory system, accounting for 63.6%. The other involvement areas were as follows: ears 48.5%, joints 30.3%, skin 15.2%, noses and eyes 12.1% respectively. The chief complaints in the respiratory group were cough, polypnea, and fever. The major manifestations of the non-respiratory group were pain and swelling of ears and nose, joints. CT showed that the wall of trachea and main bronchus were thickened, calcified, and narrowed in 13 cases (61.9%) of the respiratory group. while 3 patients (25.0%) found thickened bronchus wall without bronchostenosis in non-respiratory group . Bronchoscopy showed that 86.7% (13/15) of the patients were found airway abnormal in the respiratory group, and none of the patients in the non-respiratory group had. In the test of pulmonary function, the respiratory group had lower one second forced expiratory volume (FEV1), [FEV1/ forced vital capacity (FVC)] and peak expiratory flow (PEF), and all the differences were significant (P<0.05). CONCLUSION: CT, broncho-scopy, and pulmonary function tests could provide early evidence to diagnosis of relapsing polychondritis, which is lack of specificity of diagnosis, especially respiratory system symptom onset.


Assuntos
Broncoscopia , Policondrite Recidivante/diagnóstico por imagem , Testes de Função Respiratória , China , Humanos , Estudos Retrospectivos
4.
Clin Rheumatol ; 37(1): 251-255, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28361234

RESUMO

Aseptic meningitis is an extremely rare neurologic complication of relapsing polychondritis (RP). We reported a case of a 58-year-old Chinese female with intractable headache, puffy ears, pleocytosis, and cranial magnetic resonance imaging (MRI) showing thickened and enhanced meninges. She was finally diagnosed of aseptic meningitis due to RP after full exclusion of infectious causes. She gradually developed neurosensory hearing loss, vertigo, and saddle nose while glucocorticosteroid therapy and combined cyclophosphamide could not control her headache. Ultimately, cyclosporin A was tried showing a good response. Only 18 previous cases were found in the literature and the clinical manifestation, cerebrospinal fluid (CSF) characteristics, imaging features, and therapy considerations of RP-related aseptic meningitis were summarized by reviewing the literature. Aseptic meningitis due to RP is a rare condition of undetermined pathoetiology. Its diagnosis is primarily based on clinical manifestations combined with CSF and MRI examinations plus adequate exclusion of possible infections. Corticosteroid is the basic therapy but choice of protocol should be individualized.


Assuntos
Encéfalo/diagnóstico por imagem , Meningite Asséptica/etiologia , Policondrite Recidivante/complicações , Ciclofosfamida/uso terapêutico , Ciclosporina/uso terapêutico , Dexametasona/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Imagem por Ressonância Magnética , Meningite Asséptica/diagnóstico por imagem , Meningite Asséptica/tratamento farmacológico , Pessoa de Meia-Idade , Policondrite Recidivante/diagnóstico por imagem , Policondrite Recidivante/tratamento farmacológico , Resultado do Tratamento
5.
Clin Nucl Med ; 43(1): 25-27, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29189373

RESUMO

A 78-year-old man had fever, persistent wheezing, and serum C-reactive protein elevation. Malignant lymphoma was suspected because of mediastinal lymph nodes swelling on CT and soluble interleukin 2 receptor elevation. Symmetric F-FDG uptake in the tracheobronchial tree and bilateral auricles was observed on PET/CT. He was finally diagnosed as having relapsing polychondritis by auricular cartilage biopsy. F-FDG PET/CT may have crucial role in evaluating the extent of inflammation and deciding the biopsy site of relapsing polychondritis.


Assuntos
Fluordesoxiglucose F18 , Achados Incidentais , Policondrite Recidivante/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Idoso , Biópsia , Humanos , Masculino , Policondrite Recidivante/patologia
7.
Prog Transplant ; 27(3): 321-323, 2017 09.
Artigo em Inglês | MEDLINE | ID: mdl-29187087

RESUMO

Relapsing polychondritis is a rare multisystem autoimmune disease characterized by recurrent inflammation and destruction of different cartilaginous and proteoglycan-rich structures like cardiovascular system. We reported a case of heart transplantation in a 20-year-old man having a relapsing polychondritis with severe multiple cardiovascular involvement. The immunosuppressive treatment used to prevent graft rejection allowed to turn out the inflammatory disease. Cardiac transplantation may be considered as last resort treatment option in relapsing polychondritis with intractable extensive heart lesions.


Assuntos
Transplante de Coração , Policondrite Recidivante/cirurgia , Humanos , Masculino , Policondrite Recidivante/diagnóstico por imagem , Policondrite Recidivante/patologia , Adulto Jovem
8.
Medicine (Baltimore) ; 96(46): e8734, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29145318

RESUMO

INTRODUCTION: Fever of unknown origin (FUO) is a common initial presentation leading to a diagnostic challenge. PATIENT CONCERNS: A 3-month history of moderate-to-high fever was reported in an otherwise healthy 54-year-old man. Enhanced computed tomography (CT) scans of his chest showed a remarkable progressive enlargement of bilateral cervical, supraclavicular, hilar, and mediastinal lymph nodes within 2 weeks. Bronchofibroscopy manifested obvious luminal stenosis with swelling, thick pale mucosa, and disappearing of structures of trachea cricoid cartilage, followed by a 18F-fluorodeoxyglucose positron-emission tomography-computed tomography (18F-FDG PET/CT) with intense symmetric FDG uptake in larynx, tracheobronchial tree, and hilar, mediastinal, and axillary lymph nodes being demonstrated. DIAGNOSIS: A diagnosis of relapsing polychondritis (RP) was finally reached. INTERVENTIONS: The patient received methylprednisolone 40 mg daily with a gradual tapering in a 4-month follow-up. OUTCOMES: The patient experienced no relapse of fever and lymph nodes enlargement in the 4-month follow-up. LESSONS: Even though long-term fever with multiple lymphadenectasis usually lead to a diagnosis of lymphoma, the bronchoscopic features and evidence from 18F-FDG PET/CT in this case were much more approximate to RP, indicating an importance of a sensible differential diagnosis of RP in patients who present with nonspecific features such as FUO and lymph nodes enlargement. Keeping a high index of clinical suspicion in these patients can help recognize uncommon of RP and promote diagnosis and treatment. Our case highlights the significance of 18F-FDG PET/CT in helping reaching the diagnosis of RP in this condition. This report provides new data regarding the diagnostic difficulties of this rare type of autoimmune disease, and further investigations are needed as cases accumulate.


Assuntos
Febre de Causa Desconhecida/etiologia , Fluordesoxiglucose F18 , Policondrite Recidivante/complicações , Policondrite Recidivante/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons/métodos , Compostos Radiofarmacêuticos , Fluordesoxiglucose F18/farmacocinética , Humanos , Linfonodos/diagnóstico por imagem , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Policondrite Recidivante/patologia , Compostos Radiofarmacêuticos/farmacocinética
12.
Medicine (Baltimore) ; 96(30): e7503, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28746194

RESUMO

BACKGROUND: Relapsing polychondritis (RP) is a rare autoimmune disease of unknown etiology that may affect multiple cartilage throughout the body. CASE REPORT: We report on a middle-aged man presented with cough, chest tightness, and fever of unknown origin, 18F-fluorodeoxyglucose positron emission tomography/computed tomography (F-FDG PET/CT) was performed. And the imaging shows multiple increased FDG accumulation in tracheobronchial tree and all intercostal cartilages, as well as in nasal, right auricule, laryngeal cartilage. Based on the findings, the diagnosis of RP was made. CONCLUSION: Our case demonstrates that FDG PET/CT is an useful diagnostic tool to accurately determine the extent of inflammation throughout the body and to guiding the selection of a biopsy site.


Assuntos
Fluordesoxiglucose F18 , Policondrite Recidivante/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Diagnóstico Precoce , Humanos , Masculino , Pessoa de Meia-Idade , Policondrite Recidivante/tratamento farmacológico , Policondrite Recidivante/patologia
14.
Semin Nucl Med ; 47(4): 408-424, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28583280

RESUMO

FDG is a tracer for visualizing glucose metabolism. PET/CT using FDG is widely used for the diagnosis of cancer, because glycolysis is elevated in cancer cells. Similarly, active inflammatory tissue also exhibits elevated glucose metabolism because of glycolysis in activated macrophages and proliferating fibroblasts. Elevated FDG uptake by active inflammatory tissues, such as those affected by arthritis, vasculitis, lymphadenitis, and chondritis, has enabled the diagnosis of inflammatory diseases using FDG-PET/CT. Rheumatoid arthritis (RA) is a systemic, chronic inflammation of the joints resulting in synovitis. Several clinical studies of RA have demonstrated that FDG uptake in affected joints reflects the disease activity of RA, with strong correlations between FDG uptake and various clinical parameters having been noted. Furthermore, the use of FDG-PET for the sensitive detection and early monitoring of the response to RA therapy has been reported. RA is sometimes associated with subclinical vasculitis, which is related to systemic inflammation. FDG-PET/CT can be used to evaluate subclinical vasculitis in the aorta or carotid artery. Polymyalgia rheumatica (PMR) is an autoimmune musculoskeletal disease of unknown etiology characterized by pain and stiffness in the shoulder, neck, and pelvic girdle, but not in the small finger joints in the hands, together with fever, fatigue, and weight loss. There is no specific test for PMR, and its diagnosis is based on clinical diagnostic criteria and the exclusion of other diseases with similar symptoms. However, FDG-PET/CT reveals a characteristic FDG uptake by the bursitis in ischial tuberosity, greater trochanter, lumbar or cervical spinous process, and scapulohumeral joint. A combination of FDG-PET/CT findings showed a high diagnostic value for PMR in a differential diagnosis from RA. FDG-PET/CT is also very useful for evaluating large vessel vasculitis, which is often associated with PMR. Relapsing polychondritis is a rare multisystem disease of unknown etiology involving cartilaginous and proteoglycan-rich structures. Its rarity and diversity of symptoms often result in a delayed diagnosis. FDG-PET/CT reveals unique FDG uptake findings for chondritis in the auricular, nasal, trachea, bronchial tree, and costal cartilage and in the cartilage of joints. Thus, the spread of knowledge regarding these very specific FDG-PET/CT findings could promote the early diagnosis and improved disease control of relapsing polychondritis.


Assuntos
Artrite Reumatoide/diagnóstico por imagem , Policondrite Recidivante/diagnóstico por imagem , Polimialgia Reumática/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Fluordesoxiglucose F18 , Humanos
15.
BMJ Case Rep ; 20172017 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-28619741

RESUMO

We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness. Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis. Our patient had a constellation of symptoms which posed a diagnostic challenge. Finally, a diagnosis of relapsing polychondritis with several unusual features was made. Overlap with Takayasu's arteritis and granulomatosis with polyangitis, which has been reported rarely in the literature, cannot be excluded.


Assuntos
Otopatias/diagnóstico , Gangrena/diagnóstico , Doenças Nasais/diagnóstico , Policondrite Recidivante/diagnóstico , Pioderma Gangrenoso/diagnóstico , Diagnóstico Diferencial , Otopatias/complicações , Otopatias/diagnóstico por imagem , Feminino , Gangrena/complicações , Gangrena/diagnóstico por imagem , Humanos , Doenças Nasais/complicações , Doenças Nasais/diagnóstico por imagem , Policondrite Recidivante/complicações , Policondrite Recidivante/diagnóstico por imagem , Pioderma Gangrenoso/complicações , Pioderma Gangrenoso/diagnóstico por imagem , Adulto Jovem
16.
J Dermatol ; 44(7): 826-829, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28266051

RESUMO

Relapsing polychondritis (RP) is a rare autoimmune-mediated disease characterized by inflammation involving cartilaginous tissues. We report here a case of RP in a 38-year-old Japanese man with 13-year duration of psoriasis vulgaris treated with topical steroids and vitamin D3 . The patient presented with tender swelling and erythema of both auricles, and the antibody to type II collagen was detected. The biopsy specimen revealed a dense mixed cell infiltration over the auricular cartilage. We reviewed eight cases with the association of RP and psoriasis, and in all cases the clinical course of psoriasis did not correlate with that of RP. The severity of RP was mild in the majority of cases, and our case was unique in that the patient had no joint symptoms. Adalimumab treatment was effective for both RP and psoriasis. Fat-suppressed contrast-enhanced magnetic resonance imaging was beneficial, not only to demonstrate subclinical inflammation in the nasal septum, but also to subjectively assess the improvement of RP.


Assuntos
Adalimumab/uso terapêutico , Antirreumáticos/uso terapêutico , Glucocorticoides/uso terapêutico , Policondrite Recidivante/tratamento farmacológico , Psoríase/tratamento farmacológico , Administração Cutânea , Adulto , Autoanticorpos/sangue , Doenças Autoimunes/etiologia , Biópsia , Proteína C-Reativa/análise , Colecalciferol/uso terapêutico , Colágeno Tipo II/imunologia , Cartilagem da Orelha/diagnóstico por imagem , Cartilagem da Orelha/imunologia , Cartilagem da Orelha/patologia , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Metaloproteinase 3 da Matriz/sangue , Policondrite Recidivante/diagnóstico por imagem , Policondrite Recidivante/imunologia , Policondrite Recidivante/patologia , Psoríase/sangue , Psoríase/imunologia , Índice de Gravidade de Doença
19.
Int J Neurosci ; 127(2): 124-134, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26887435

RESUMO

Relapsing polychondritis (RPC) is a rare, immune-mediated condition affecting approximately 3.5 per million population per year. Neurological involvement in RPC is still rarer and is presumed to be the result of a vasculitic process, although this is seldom confirmed in the literature. We present two cases of RPC complicated by cognitive dysfunction with contrasting clinical trajectories. Our findings suggest that there are two clinical phenotypes of cognitive dysfunction in RPC. The first is a fulminant, multisystem presentation with sub-acute cognitive decline mimicking central nervous system vasculitis, and we provide histopathological evidence of this process occurring. The other is an insidious cognitive decline without associated constitutional or systemic symptoms.


Assuntos
Transtornos Cognitivos/complicações , Policondrite Recidivante/etiologia , Idoso , Encéfalo/patologia , Transtornos Cognitivos/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Policondrite Recidivante/diagnóstico por imagem
20.
Acta Clin Belg ; 72(2): 130-132, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27075792

RESUMO

We present the case of a 39-year-old man with a severe glottis stenosis. The saddle nose, images of laryngotracheal stenosis and the (FDG) positron-emission tomography/computed tomography lead to a final diagnosis of relapsing polychondritis. In the patient a coexistent myelodysplastic syndrome was diagnosed. Moreover, the elevated total IgG4 exceeding 135 ml/dl requested additional immunochemistry for detection of IgG4-bearing plasma cells in the biopsies. The patient underwent an allogenic stem cell transplantation and died on day 40 after the transplantation because of an acute steroid-resistent graft vs host.


Assuntos
Glote/patologia , Nariz/patologia , Policondrite Recidivante/diagnóstico por imagem , Adulto , Constrição Patológica/etiologia , Humanos , Masculino , Policondrite Recidivante/complicações , Policondrite Recidivante/patologia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA