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BACKGROUND: Clinical presentation of Lyme-associated uveitis is poorly described. We reported here a case series of seven patients with uveitis related to Lyme disease and a review of the literature. METHODS: A retrospective study in our university hospital between 1 May 2003 and 31 July 2016 on 1006 uveitis patients and review of Pubmed library. RESULTS: Seven patients (71.4% male, mean age = 53 (38-70)) were diagnosed with a Lyme-associated uveitis. All anatomical types of uveitis were found (four intermediate, three anterior, and three posterior uveitis); most were unilateral (n = 6; 85.7%), one granulomatous and two with synechiae. Peripheral retinal vasculitis was present in four patients. They all had a risk of exposure or extra-ophthalmological symptoms. Antibiotic and steroid treatment was rapidly effective in all patients. Four patients presented recurrences of uveitis, of whom two received a second antibiotic treatment, which is quite common in literature. Persistent or recurrence of symptoms can be explained by three hypotheses: (1) reinfection, (2) relapse of original infection, and (3) autoimmune reaction. CONCLUSION: Lyme-associated uveitis appears varied. Hyalitis and involvement of the posterior segment and retinal vasculitis seem to be rather frequent. Its prognosis is mainly good, even if inflammation can be resistant or recurring.
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Infecções Oculares Bacterianas/etiologia , Doença de Lyme/complicações , Uveíte/etiologia , Adulto , Idoso , Antibacterianos/uso terapêutico , Endoftalmite/diagnóstico , Endoftalmite/tratamento farmacológico , Endoftalmite/etiologia , Infecções Oculares Bacterianas/diagnóstico , Infecções Oculares Bacterianas/tratamento farmacológico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Doença de Lyme/diagnóstico , Doença de Lyme/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Recidiva , Vasculite Retiniana/diagnóstico , Vasculite Retiniana/tratamento farmacológico , Vasculite Retiniana/etiologia , Estudos Retrospectivos , Uveíte/diagnóstico , Uveíte/tratamento farmacológicoRESUMO
OBJECTIVE: To determine the ophthalmological and extra-ophthalmological clinical characteristics and visual prognosis of patients with sarcoid uveitis in different ethnic groups. METHODS: We retrospectively analysed the data from patients with sarcoid uveitis seen at two departments of Ophthalmology between December 2003 and December 2017. Patients presented biopsy-proven sarcoidosis and/or presumed sarcoid uveitis based on the following criteria: compatible thoracic imaging, associated with elevated angiotensin-conversion enzyme (ACE) and/or lymphocytic alveolitis on bronchoalveolar lavage fluid analysis (> 15% lymphocytes and CD4/CD8 > 3.5). Ophthalmological and general characteristics, as well as visual and global prognoses, were compared in three pre-defined ethnic groups: White Europeans, North Africans and Afro-Caribbeans. RESULTS: A total of 194 patients were included: 145 with biopsy-proven and 49 with presumed sarcoid uveitis. Overall, 68% were White Europeans while 20.6% were North Africans and 11.3% were Afro-Caribbeans. Sixty-nine per cent were women and the median age at presentation was 52.1 years. Median ages at first ocular manifestation of the disease in Afro-Caribbeans and North Africans were respectively 34.3 and 43.1 years, while it was 57.8 years in White Europeans (p < 0.001). Ocular involvement was bilateral in 77.8% (n = 151) of the cases and nearly half of the patients had panuveitis (48.5%). Anterior uveitis was more frequent in Afro-Caribbeans (59.1%; p < 0.0001), while White Europeans presented more frequently with intermediate uveitis. There was a significantly higher frequency of systemic involvement of sarcoidosis in North Africans while White Europeans showed a higher frequency of isolated ocular involvement at onset and during follow-up. Afro-Caribbeans, who had a complete visual recovery in 72.7% of the cases, had a better visual prognosis than other ethnic groups (p = 0.025). CONCLUSION: In this large European series of sarcoid uveitis, we observed ethnicity-related differences regarding uveitis clinical presentation and visual outcome. Although good overall, the visual prognosis seems to be better in Afro-Caribbeans than in other ethnic groups.
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Etnicidade , Sarcoidose/complicações , Uveíte/etnologia , Adulto , Biópsia , Feminino , Seguimentos , França/epidemiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Sarcoidose/diagnóstico , Sarcoidose/etnologia , Fatores de Tempo , Tomografia de Coerência Óptica , Uveíte/diagnóstico , Uveíte/etiologiaRESUMO
BACKGROUND: Uveitis is a frequent and early feature of sarcoidosis. As BTNL2 (butyrophilin-like 2) gene polymorphism was found linked with the susceptibility to sarcoidosis, we investigated whether a specific genotype of BTNL2 gene G16071A (or rs2076530) single-nucleotide polymorphism (SNP) would be associated with the risk of sarcoid uveitis in all patient subgroups. METHODS: The study compared the genotype frequencies of SNP G16071A of 135 patients with sarcoid uveitis (Sa+Uv+) with those of 196 patients with sarcoidosis without uveitis (Sa+Uv-), 81 patients with uveitis without sarcoidosis (Sa-Uv+), and 271 controls with no sarcoidosis nor uveitis (Sa-Uv-). Three hypothetical subgroups of patients with sarcoid uveitis (Sa+Uv+ cases) were considered: (1) subgroup I: patients aged <45 years of both sexes and all ethnic origins; (2) subgroup II: Caucasian women aged >45 years; and (3) subgroup III: all other patients. RESULTS: A statistically significant difference in genotype frequencies was found between the groups Sa+Uv- and Sa-Uv- (p=3.2×10-6) and between the groups Sa+Uv+ and Sa+Uv- (p=7.1×10-3). There was no difference between the three subgroups of Sa+Uv+ patients. There was a statistically significant difference in genotype frequencies between Sa+Uv- and Sa+Uv+ subgroup II (p=0.005) but no difference between Sa+Uv- and Sa+Uv+ subgroup I. CONCLUSION: No association was found between G16071A and the susceptibility to sarcoid uveitis. BTNL2 gene G16071A SNP seems to be a predisposing factor for sarcoidosis except in Caucasian postmenopausal women with sarcoid uveitis in whom the GG genotype prevails. These and future results will help in understanding differences between particular subgroups of patients with sarcoid uveitis.
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Butirofilinas/genética , Polimorfismo de Nucleotídeo Único , Sarcoidose/genética , Uveíte/genética , Adulto , Estudos de Casos e Controles , Primers do DNA/química , Feminino , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Fatores de Risco , Sarcoidose/diagnóstico , Uveíte/diagnósticoRESUMO
AIM: To assess the usefulness of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET/CT) and the predictive factors for the diagnosis of sarcoidosis in patients with uveitis who have normal thoracic tomography. METHODS: We retrospectively reviewed 67 consecutive patients with uveitis of unknown aetiology or a suspected sarcoidosis. All patients with normal thoracic tomography underwent an 18F-FDG PET/CT, which was blindly reinterpreted. We then assessed the proportion of positive 18F-FDG PET/CT and the impact on the final aetiology, using Abad's criteria for the diagnosis of intraocular sarcoidosis. RESULTS: 19 of the 67 patients (28.4%) had mediastinal hypermetabolic foci on their 18F-FDG PET/CT consistent with sarcoidosis. It identified a biopsy site in two cases, which were consistent with sarcoidosis. At the end of the study, six patients (10%) had a proven sarcoidosis, six patients (9%) were considered as having a presumed sarcoidosis and 18 patients (26.9%) as having indeterminate sarcoidosis. 18F-FDG PET/CT enabled the diagnosis of presumed sarcoidosis in these six patients. An older age at diagnosis (p=0.004) and the presence of synechiae (p=0.02) were significantly related to an abnormal 18F-FDG PET/CT, with a trend for an elevated ACE (p=0.0993). We established a nomogram to estimate the probability of having positive findings on the 18F-FDG PET/CT according to different predictive factors. CONCLUSION: 18F-FDG PET/CT enabled the diagnosis of intraocular sarcoidosis even in patients with a normal CT scan. Older age at diagnosis, presence of synechiae and elevated ACE are associated with positive findings on 18F-FDG PET/CT consistent with sarcoidosis.
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Fluordesoxiglucose F18/administração & dosagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Compostos Radiofarmacêuticos/administração & dosagem , Sarcoidose/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Uveíte/diagnóstico por imagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoidose Pulmonar/diagnóstico por imagemRESUMO
Purpose: Despite the huge advance in diagnostic technics, about one-third of uveitis is still considered of unknown etiology. In this study, we aimed to report their clinical features and to describe how a diagnosis has been finally reached for some patients.Methods: We retrospectively reviewed all patients with uveitis referred to our tertiary center between 2002 and 2016. The unknown etiology was admitted after a new ophthalmologic examination and a full work-up in internal medicine in our tertiary center.Results: Among 957 patients with uveitis, 355 had uveitis of unknown etiology. The clinical and epidemiological characteristics of this subgroup were no different from those with a known etiology. Out of 104 patients who were followed-up for more than 1 year, a diagnosis was finally achieved in 20 patients. The diagnosis was determined either because of the occurrence of a new clinical symptom (n = 10), a new/repeated non-ophthalmologic investigation (n = 7), or a new/repeated ophthalmic exam (n = 3).Conclusion: A prolonged follow-up, with repeated exams, may allow the determination of an etiology in about one-fifth of uveitis initially considered as idiopathic.
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Medição de Risco/métodos , Sarcoidose/complicações , Uveíte/diagnóstico , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Seguimentos , França/epidemiologia , Humanos , Incidência , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Sarcoidose/diagnóstico , Distribuição por Sexo , Microscopia com Lâmpada de Fenda , Fatores de Tempo , Tomografia Computadorizada por Raios X/métodos , Uveíte/epidemiologia , Uveíte/etiologia , Adulto JovemRESUMO
PURPOSE: To describe patients with new onset sarcoid uveitis occurring after an ophthalmic procedure and compare them with patients with sarcoid uveitis without ocular procedure. METHODS: Retrospective analysis of case records from patients with postophthalmic procedure sarcoid uveitis seen at our institution between April 2004 and October 2016. Patients with a previous history of uveitis were not included. Each patient was randomly matched with four controls from our incident cohort of new onset sarcoid uveitis without ophthalmic procedure. RESULTS: We identified 11 patients (8.5%) from our incident cohort of sarcoid uveitis (n=130), who were all women, with a postophthalmic procedure uveitis (mostly after cataract surgery (36%)). These patients were older (69.7 vs 52.7 years) and presented more synechiae than controls. After a mean follow-up of 30 (3-60) months, there was no significant difference between the postprocedure and the control group with regard to demography, clinical presentation, disease course, treatment, and outcome. CONCLUSIONS: Sarcoid uveitis has similar characteristics in patients with new onset sarcoid uveitis after or without ophthalmic procedure. As a consequence, ophthalmic intervention should be seen as a potential trigger of latent sarcoidosis.
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PURPOSE: ULISSE is the only study that prospectively assessed the efficiency of a standardized strategy, compared to an open strategy for the etiologic diagnosis of uveitis. Our aim was to evaluate the diagnostic yield of the tests prescribed in the ULISSE study to clarify their relevance. METHODS: ULISSE is a non-inferiority, prospective, multicenter and cluster randomized study. The standardized strategy is a two-steps strategy: in the first step, common standard tests were performed, and in the second step, tests were guided by the clinical and anatomic type of uveitis. We reported the relevance of the diagnostic tests used in the standardized strategy, as well as the profitability of the tests that were prescribed to more than twenty patients in each group. Based on diagnostic criteria, either an ophthalmologist, or an internist, established the profitability of a test by considering whether the test lead to a diagnosis or not. RESULTS: Among the 676 patients included (standardized 303; open 373), a diagnosis was made for 152 (50.4%) in the standardized group and 203 (54.4%) in the open group. The most common entities were HLA-B27 associated uveitis (22%), spondyloarthritis (11%), sarcoidosis (18%), tuberculosis (10.7%) and herpes virus infections (8.5%). Among the first step's systematic tests, tuberculin skin test was the most contributive investigation (17.1%), followed by chest X-ray (8.4%), C reactive protein and ESR (6.6% and 5.1%), complete blood count (2.2%) and VDRL (2.0%). The second step's most often contributive tests were: HLA B27 (56.3%), chest-CT (30.3%) and angiotensin converting enzyme (ACE) (16.5%). HLA B27 and ACE were significantly more contributive in the standardized group than in the open group. Immunological tests were never contributive. Among the free investigations, or among the investigations guided by clinical or paraclinical findings, the most often contributive tests were: Quantiferon® (24%), electrophoresis of serum protein (7.8%) and sacroiliac imagery (46.4%). Intracellular serologies (1.7%), serum calcium (2.1%) and hepatic tests (3.3%) were exceptionally contributive. Among the third intention tests, labial salivary gland biopsies were contributive in 17.9% of cases, but the profitability of other invasive investigations (anterior chamber tap, vitrectomy, bronchoscopy and lumbar puncture) or specialized imagery (18F-FDG PET, Brain MRI) could not be determined since these test were rarely performed. CONCLUSION: Only a few diagnostic tests are useful for the etiological assessment of uveitis. They are often cheap, simple, more often guided by the clinical findings, and lead to an etiological diagnosis in most patients. On the other hand, some tests are never or exceptionally contributive, such as immunological tests or intracellular serologies. Further studies are required to evaluate the profitability of third intention imagery and invasive investigations.
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Testes Diagnósticos de Rotina/métodos , Uveíte/diagnóstico , Uveíte/etiologia , Adulto , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Uveíte/patologiaRESUMO
AIMS: To determine the frequency and clinical presentation of Lyme disease in patients with uveitis and to assess the value of Borrelia burgdorferi serological testing. METHODS: Retrospective study on all patients with uveitis who were referred to our tertiary hospital were serologically tested for Lyme in our laboratory between 2003 and 2016. Screening consisted of determining B. burgdorferi serum IgG and IgM by ELISA method. The patient's serology was considered as positive if the ELISA-positive result in IgM and/or IgG was confirmed by an immunoblot positive in IgM and/or IgG. Lyme-associated uveitis was diagnosed based on serological results as well as response to antibiotics and exclusion of other diagnosis. RESULTS: Of the 430 patients with uveitis (60% women, mean age 49 years) fulfilling inclusion criteria, 63 (14.7%) had an ELISA-positive serology, confirmed by immunoblot for 34 patients (7.9%). The diagnosis of Lyme-associated uveitis was finally retained in seven patients (1.6%). These patients reported either a previous exposure including tick bite or forest walks (n=5), symptoms suggestive of Lyme disease (n=5) and resistance to local and/or systemic steroids (n=7). Among the remaining 27 positive patients, 22 had other established aetiologies and 5 other were unclassified. CONCLUSION: The seroprevalence of B. burgdorferi among our patients with uveitis was 7.9% compared with 6 to 8.5% in the general French population which leads to a low predictive value of serological testing. Its use should be reserved for patients with unexplained uveitis, an exposure history, systemic findings suggestive of Lyme disease and steroids resistance.
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Anticorpos Antibacterianos/sangue , Borrelia burgdorferi/imunologia , Infecções Oculares Bacterianas/diagnóstico , Doença de Lyme/diagnóstico , Uveíte/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Ensaio de Imunoadsorção Enzimática , Infecções Oculares Bacterianas/imunologia , Infecções Oculares Bacterianas/microbiologia , Feminino , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Doença de Lyme/imunologia , Doença de Lyme/microbiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Estudos Soroepidemiológicos , Centros de Atenção Terciária , Uveíte/imunologia , Uveíte/microbiologia , Adulto JovemRESUMO
INTRODUCTION: The off-label use of TNF antagonists in refractory sarcoidosis is increasingly reported but data on their efficacy and safety are still insufficient. OBJECTIVE: To report on efficacy and safety of TNF antagonists in severe and refractory sarcoidosis. METHODS: Examination of retrospective demographic, clinical, therapeutic, and adverse event data on 132 sarcoidosis patients (58% women; mean (min-max) age = 45.5 (14-78) years) given TNF antagonists (mainly infliximab, 91%) and investigation of response-linked factors. RESULTS: The overall clinical response (complete and partial) rate was 64%. TNF-antagonist efficacy (i.e., significant decrease of the ePOST score) was noted in cases with neurologic, heart, skin, and upper respiratory tract involvements. No significant difference in efficacy was found between anti-TNF used alone and TNF with immunosuppressant. The use of anti-TNF allowed reducing prednisone dosage at end of follow-up (p < 0.001). Adverse events were observed in 52% of the patients; they included infections (36%) and allergic reactions (8%) and required treatment interruption in 31 cases (23%). When TNF antagonists were interrupted, 13 patients experienced relapses within 14 months on average (median follow-up: 20.5 months). CONCLUSION: TNF antagonists were efficacious in about two-thirds of patients with severe/refractory sarcoidosis but their use led to a high rate of adverse events.
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Imunossupressores/uso terapêutico , Infliximab/uso terapêutico , Sarcoidose/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adolescente , Adulto , Idoso , Feminino , Humanos , Imunossupressores/efeitos adversos , Infliximab/efeitos adversos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
PURPOSE: To prospectively assess the efficiency of a standardized diagnostic approach, compared to an open strategy, for the etiologic diagnosis of uveitis. DESIGN: Noninferiority, prospective, multicenter, clustered randomized controlled trial. METHODS: Consecutive patients with uveitis, who visited 1 of the participating departments of ophthalmology, were included. In the standardized group, all patients had a minimal evaluation regardless of the type of uveitis (complete blood count, erythrocyte sedimentation rate, C-reactive protein, tuberculin skin test, syphilis serology, and chest radiograph) followed by more complex investigations according to ophthalmologic findings. In the open group, the ophthalmologist could order any type of investigation. Main outcome was the percentage of etiologic diagnoses at 6 months. RESULTS: Nine hundred and three patients with uveitis were included from January 2010 to May 2013 and the per-protocol population comprised 676 patients (open 373; standardized 303). Mean age at diagnosis was 46 years. Anatomic distribution of uveitis was as follows: anterior (60.8% and 72.3%, P = .0017), intermediate (11.7% and 12.3%, P = .8028), posterior (17.8% and 8.2%, P = .0004), and panuveitis (15.3% and 15.2%, P = .9596). An etiologic diagnosis was established in 54.4% of cases in the open group and 49.5% in the standardized group (P = .2029). The difference between both strategies (standardized minus open) was -4.9% (95% CI [-12.5%; 2.6%]). There were more investigations in the open group than in the standardized group (5371 vs 3759, P < .0001). CONCLUSION: The standardized strategy appears to be an efficient diagnostic approach for the etiologic diagnosis of uveitis, although its noninferiority cannot be proved.
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Técnicas de Diagnóstico Oftalmológico/normas , Ensaios Clínicos Pragmáticos como Assunto , Uveíte/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Acuidade VisualRESUMO
PURPOSE: To report the outcomes of initial methylprednisolone pulse then oral prednisone in the treatment of birdshot chorioretinopathy (BSCR). METHODS: We retrospectively reviewed medical records of 21 BSCR patients, treated with initial methylprednisolone, prednisone, or immunosuppressive therapy. Main outcome measures were changes in visual acuity, intraocular inflammation, concomitant immunosuppressive drug or intravitreal steroid, and adverse events. RESULTS: In total, 14 patients were initially treated with methylprednisolone; four patients with prednisone; and three with immunosuppressive drug. Intraocular inflammation was controlled in 10 of 14 patients (71%) at 1 year, with a mean dose of 6.2 mg/day prednisone. Patients treated initially by methylprednisolone had improved visual acuity at 1 year, but it was not significantly different from patients treated initially by prednisone. CONCLUSIONS: Corticosteroid therapy, including initial methylprednisolone pulse then prednisone, was effective in stabilizing vision and decreasing inflammation in most patients with BSCR.
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Coriorretinite/tratamento farmacológico , Glucocorticoides/administração & dosagem , Metilprednisolona/administração & dosagem , Prednisona/uso terapêutico , Administração Oral , Adulto , Idoso , Coriorretinopatia de Birdshot , Coriorretinite/diagnóstico , Coriorretinite/fisiopatologia , Combinação de Medicamentos , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Pulsoterapia , Estudos Retrospectivos , Resultado do Tratamento , Acuidade Visual/fisiologiaRESUMO
AIMS: To determine the long-term visual and systemic outcomes of uveitis patients with biopsy-proven sarcoidosis. METHODS: A retrospective study of biopsy-proven sarcoid uveitis, with a 3-year minimum follow-up, seen at Lyon University Hospital, between April 2004 and January 2016. RESULTS: A total of 83 patients were included, with a median age at onset of 52 (37-62) years and an unbalanced gender ratio (women 77.1%). Thirty-one patients had original systemic sarcoidosis in addition to ocular localisation, whereas 52 initially presented with isolated sarcoid uveitis. Among the latter, 7.7% (n=4) developed an extraocular disease after a median follow-up duration of 60 (44-110) months. The systemic spread in these patients included cutaneous sarcoids (n=2), arthritis (n=1) and multiple mononeuritis (n=1). Complete visual recovery was obtained for 60.2% of all patients and 89.2% had retrieved best-corrected visual acuity (BCVA) >20/50 in both eyes. A unilateral loss of BCVA of worse than 20/200 was documented in two patients in the isolated sarcoid uveitis group. No patient suffered from bilateral severe visual impairment or blindness. Factors linked to a poor visual prognosis, defined by BCVA ≤20/50 in at least one eye, were chronic macular oedema (p=0.009) and persistent ocular inflammation (p=0.0005). CONCLUSIONS: In this large European series of biopsy-proven sarcoidosis to date, with a prolonged follow-up, sarcoid uveitis was suggestive of a favourable systemic and visual outcome. Clinically isolated uveitis that revealed sarcoidosis remained a strictly ocular condition in most cases.
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Segmento Anterior do Olho/patologia , Biópsia/métodos , Sarcoidose/diagnóstico , Uveíte/diagnóstico , Acuidade Visual , Adulto , Progressão da Doença , Feminino , Angiofluoresceinografia/métodos , Seguimentos , Fundo de Olho , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Sarcoidose/fisiopatologia , Fatores de Tempo , Tomografia de Coerência Óptica/métodos , Tomografia Computadorizada por Raios X , Uveíte/fisiopatologiaRESUMO
Backgroung: This study investigated the efficacy and safety of TNF antagonists in sarcoid uveitis in unselected cases. Design: This is a multicentre study on patients with sarcoidosis who received TNF antagonists in pneumology and internal medicine departments in France. We present here the subgroup of patients with biopsy-proven sarcoid uveitis included in the nationwide registry STAT (Sarcoidosis treated with TNF AnTagonists). Results: Among the 132 patients included in this multicenter study, 18 patients with refractory uveitis were treated as a first-line TNF antagonist with infliximab (n=14), adalimumab (n=3) and certolizumab (n=1). Before anti-TNF initiation, the median duration of sarcoidosis was 42 months and 83% of the patients have been treated with at least one immunosuppressive drug. Six patients switched for a second-line TNF antagonist. After a mean time under treatment of 29 months, the treatment resulted in a significant decrease of the ophthalmic extrapulmonary Physician Organ Severity Tool (ePOST) (mean score: 4.2 vs. 2.6) scores and a steroid sparing effect (29.4±20.7 vs. 6.2±5.2 mg/d). Overall, the ophthalmic response, either complete or partial, was 67%. Nine patients (50%) presented adverse events, including severe infectious complications in 5 patients, which required anti-TNF treatment interruption in 6 cases (33%). Among the 7 responder patients who discontinued anti-TNF therapy, 71% relapsed. Finally, 12 patients (67%) could continue TNF antagonist treatment. Conclusions: TNF antagonists were efficient in 67% of biopsy-proven refractory sarcoid uveitis. Severe adverse events, mainly infectious complications, were frequent. The high frequency of relapses after anti-TNF-α discontinuation requires a close patient follow-up thereafter. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 74-80).
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We evaluated mortality rates and underlying causes of death among French decedents with sarcoidosis from 2002 to 2011.We used data from the French Epidemiological Centre for the Medical Causes of Death to 1) calculate sarcoidosis-related mortality rates, 2) examine differences by age and gender, 3) determine underlying and nonunderlying causes of death, 4) compare with the general population (observed/expected ratios), and 5) analyse regional differences.1662 death certificates mentioning sarcoidosis were recorded. The age-standardised mortality rate was 3.6 per million population and significantly increased over the study period. The mean age at death was 70.4â years (versus 76.2â years for the general population). The most common underlying cause of death was sarcoidosis. Sarcoidosis decedents were more likely to be males when aged <65â years. When sarcoidosis was the underlying cause of death, the main other mentions on death certificates were chronic respiratory and cardiovascular diseases. The overall observed/expected ratio was >1 for infectious disease, tuberculosis and chronic respiratory disease, and <1 for neoplasms. We observed a north-south gradient of age-standardised mortality ratio at the country level.Despite the limitation of possibly capturing the more severe cases of sarcoidosis, this study may help define and prioritise preventive interventions.
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Sarcoidose/mortalidade , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Causas de Morte , Feminino , França/epidemiologia , Humanos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Distribuição por Sexo , Adulto JovemRESUMO
BACKGROUND AND OBJECTIVES: Progressive multifocal leukoencephalopathy (PML) is a rare, JC-virus-mediated, demyelinating disease with a high mortality rate. As no recommended treatment exists, mirtazapine, a potential blocker of virus entry into cells, has been empirically used. METHODS: We analysed existing data on mirtazapine's efficacy to treat PML by systematically reviewing the literature since 2005, when it was first used. RESULTS: Searches in PubMed, EBSCO, SCOPUS and Google Scholar between January 2005 and December 2015, identified five cohort studies and 74 case reports. No statistically significant effect of mirtazapine on PML outcome was observed in the cohort studies. From studying the case reports, mortality rate for PML was associated with the underlying circumstances, such as an older age, the use of an immunosuppressant, or PML occurring in patients with a haematological malignancy or a transplant. CONCLUSIONS: Except for natalizumab-associated PML, we did not highlight any potential benefit of mirtazapine on disease outcomes. Further interventional studies are needed to confirm that 5-HT2AR inhibition is relevant to treat PML.
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Leucoencefalopatia Multifocal Progressiva/tratamento farmacológico , Mianserina/análogos & derivados , Humanos , Imunossupressores/administração & dosagem , Vírus JC/isolamento & purificação , Mianserina/uso terapêutico , Mirtazapina , Natalizumab/efeitos adversosRESUMO
Parenchymal lung involvement (PLI) in adult-onset Still's disease (AOSD) has seldom, if ever, been studied. We examine here retrospective cohort AOSD cases and present a review of the literature (1971-2014) on AOSD-related PLI cases.Patients with PLI were identified in 57 AOSD cases. For inclusion, the patients had to fulfill Yamaguchi or Fautrel classification criteria, show respiratory symptoms, and have imaging evidence of pulmonary involvement, and data allowing exclusion of infectious, cardiogenic, toxic, or iatrogenic cause of PLI should be available. This AOSDâ+âPLI group was compared with a control group (non-PLI-complicated AOSD cases from the same cohort).AOSDâ+âPLI was found in 3 out of the 57 patients with AOSD (5.3%) and the literature mentioned 27 patients. Among these 30 AOSDâ+âPLI cases, 12 presented an acute respiratory distress syndrome (ARDS) and the remaining 18 another PLI. In the latter, a nonspecific interstitial pneumonia computed tomography pattern prevailed in the lower lobes, pulmonary function tests showed a restrictive lung function, the alveolar differential cell count was neutrophilic in half of the cases, and the histological findings were consistent with bronchiolitis and nonspecific interstitial pneumonia. Corticosteroids were fully efficient in all but 3 patients. Ten out of 12 ARDS cases occurred during the first year of the disease course. All ARDS-complicated AOSD cases received corticosteroids with favorable outcomes in 10 (2 deceased). Most PLIs occurred during the systemic onset of AOSD.PLI may occur in 5% of AOSDs, of which ARDS is the most severe. Very often, corticosteroids are efficient in controlling this complication.
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Pneumopatias/etiologia , Pneumopatias/patologia , Doença de Still de Início Tardio/complicações , Doença de Still de Início Tardio/patologia , Corticosteroides/uso terapêutico , Humanos , Pneumopatias/diagnóstico por imagem , Pneumopatias/tratamento farmacológico , Doença de Still de Início Tardio/diagnóstico por imagem , Doença de Still de Início Tardio/tratamento farmacológicoRESUMO
Hemophagocytic lymphohistiocytosis (HLH) is a critical condition that may lead to organ failure and early death. The aim of this retrospective observational study was to describe a cohort of HLH patients admitted to intensive care unit (ICU) and investigate the risk factors of early death.A positive HLH diagnosis was defined by an HScore ≥ 169. Univariate and multivariate analyses were carried out to investigate hospital and 28-day mortality risk factors. Between January 2002 and July 2014, 71 HLH cases were seen at our institution.The overall 28-day mortality (start at ICU admission) and hospital mortality were 38% and 68%, respectively. The factors associated with increased 28-day mortality were the sequential organ failure assessment score at ICU admission (Pâ<â.001) and advance in age (P = 0.03). The factors associated with increased hospital mortality were a high sequential organ failure assessment score at ICU admission (Pâ<â0.01), advance in age (Pâ=â0.04), and the presence of lymphoma-related HLH or HLH of unknown origin (Pâ<â0.01).Organ failure overtops the classical early-death risk factors in adult ICU-admitted HLH patients. This failure and the subsequent early death may be prevented by timely specific cytotoxic therapies and the control of the underlying disease.
Assuntos
Linfo-Histiocitose Hemofagocítica/mortalidade , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Temperatura Corporal , Feminino , Mortalidade Hospitalar , Humanos , Unidades de Terapia Intensiva , Masculino , Pessoa de Meia-Idade , Escores de Disfunção Orgânica , Estudos Retrospectivos , Fatores de RiscoRESUMO
In 1802 the Hôtel-Dieu in Lyons was incorporated in the so-called Hospices Civils de Lyon. This allowed the expansion and renovation of buildings, as well as the improvement of the conditions of hygiene and comfort of the patients. This hospital was devoted only to the most severely ill or injured adults. 1100 patients were treated by seven doctors, a main surgeon and his deputy, residents and sisters. Broadly speaking the evolution of surgery can be divided into two periods: that of before anesthesia and septic surgery and that of antiseptic and aseptic surgery. We have to mention Gensoul and the resection of the maxillary before anesthesia, Bonnet and Ollier who were devoted to osteo-articular surgery (Ollier's disease), Poncet who built the first aseptic theater, Jaboulay and the resident Carrel who were transplantation's pioneers, Bouveret (paroxysmal tachycardia and Bouveret syndrome), Destot who did the first medical use of X-rays in 1895.
Assuntos
Hospitais/história , Pacientes Internados/história , Adulto , França , História do Século XIX , HumanosRESUMO
AIMS: To determine the clinical features of patients with uveitis with biopsy-proven sarcoidosis, document differences in these features according to ethnicity, age and sex, and assess the diagnostic value of biochemical and imaging examinations. METHODS: Retrospective study of 83 biopsy-proven sarcoid uveitis cases seen at two Departments of Internal Medicine and two Departments of Ophthalmology between April 2004 and March 2014. RESULTS: Caucasian patients presented with uveitis at a later age than non-Caucasian (58 years vs 41â years; p=0.001) and had more often a chronic form (78.3% vs 43.8%; p=0.01). Women had higher rates of chronic macular oedema than men (48.3% vs 14.3%; p=0.01). There were no statistically significant differences between patients aged ≤50 years and patients aged >50â years. ACE levels were high (>62â U/L) in 61.7% and lysozyme levels high (>16.7â mg/L) in 83.9% of tested patients. Chest X-rays and CTs were suggestive of sarcoidosis in 62.8% and 91.2% of cases, respectively. Among 21 patients with positive tomography and negative X-rays, 13 were Caucasian women >50â years. Endoscopic ultrasound-guided fine-needle aspiration of intrathoracic nodes contributed to the diagnosis in 7 patients with normal labial salivary gland and transbronchial biopsies. Any of the enzyme tests together with any of the imaging tests identified 100% of the patients. CONCLUSIONS: In this largest European series of biopsy-proven sarcoidosis to date, the outstanding diagnostic ability of enzyme test plus imaging test couple suggests that the recourse to invasive procedures should be limited to patients with ocular involvement that would justify systemic treatments.