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1.
Ocul Immunol Inflamm ; : 1-9, 2019 Oct 31.
Artigo em Inglês | MEDLINE | ID: mdl-31671003

RESUMO

Background: This study aimed to assess the diagnostic relevance of CD4/CD8 ratio in cerebrospinal fluid (CSF) for the etiological diagnosis work-up of uveitis.Methods: We consecutively included patients who were referred to our department for the diagnostic workup of intermediate and/or posterior uveitis. Etiological diagnoses were established in a blind manner regarding CD4/CD8 ratio.Results: Fifty-two patients were included. A diagnosis of ocular sarcoidosis was made in 15 (29%) patients, 21% had another determined diagnosis while 50% remained of undetermined origin. Median CD4/CD8 ratio in CSF was 4.57 (IQR 3.39-5.47) in ocular sarcoidosis, 1.74 (1.60-3.18) in uveitis due to other determined cause (P = .008), and 2.83 (2.34-3.54) in those with uveitis of undetermined origin (P = .007). CD4/CD8 ratio >3.23 was associated with a diagnosis of ocular sarcoidosis.Conclusion: Determination of CD4/CD8 ratio in CSF can be useful for diagnosis work-up since a CD4/CD8 ratio >3.23 in CSF is associated with ocular sarcoidosis.

2.
Autoimmun Rev ; : 102403, 2019 Oct 19.
Artigo em Inglês | MEDLINE | ID: mdl-31639515

RESUMO

In systemic sclerosis (SSc), the use of corticosteroids (CS) is controversial due to their association with scleroderma renal crisis (SRC). However, patients with very early and early disease, characterised by main inflammatory component, may benefit from CS therapy. The aim of this review is to discuss pros and cons of CS treatment in SSc, providing current evidence about the use of CS in SSc. Moreover, we discuss also the underlying pathogenetic mechanisms that may be the background for the potential harms and efficacy of CS in SSc.

3.
Artigo em Inglês | MEDLINE | ID: mdl-31625621

RESUMO

Prednisone is an anti-inflammatory drug widely used in internal medicine and rheumatology, but dosing remains empirical. The active metabolite of prednisone is free prednisolone. The aim of this work was to build a population pharmacokinetic (PK) model that can predict free prednisolone concentrations in patients with inflammatory/immunologic conditions. A total of 107 patients from the department of internal medicine of Cochin hospital provided 343 observations. Blood samples drawn for biological analyses were used for drug determination. Total plasma prednisolone concentrations were measured by liquid chromatography-mass spectrometry and the data were modelled using Monolix. The pharmacokinetics was ascribed a one-compartment open model with 3 transit compartments standing for the absorption and metabolism process. The model used predicts free concentrations that served to derive total concentrations given published binding constants. Only size parameters influenced the pharmacokinetics. Free prednisolone CLU /F and VU /F, scaled allometrically on lean body weight, were respectively 26.7 L/h and 94.3 L for 50 kg LBW. CLU /F interindividual variability was 0.20. The additive and proportional residual variabilities were respectively 4.3 µg/L and 0.20. The results point out some dosing issues, i.e., the possibility of under- or over-dosage in thin or overweight patients respectively.

4.
BMJ Open ; 9(10): e029542, 2019 Oct 14.
Artigo em Inglês | MEDLINE | ID: mdl-31615796

RESUMO

OBJECTIVES: The Scleroderma Patient-centered Intervention Network (SPIN) Cohort uses the cohort multiple randomised controlled trial design to embed trials of online self-care interventions for people living with systemic sclerosis (SSc; scleroderma). To offer interventions to patients interested in using them, participants complete signalling items that query about the likelihood that patients would agree to participate in nine different hypothetical online programmes addressing common SSc-related problems. It is not known what factors influence patient-reported interest in participating in a particular online intervention and if intervention-specific signalling questions provide unique information or replicate broader characteristics, such as overall willingness to participate or self-efficacy. This study assessed factors that explain responses to intervention-specific signalling items. DESIGN: Cross-sectional survey. SETTING: SPIN Cohort participants enrolled at 42 centres from Canada, the USA, the UK, France, Spain and Mexico who completed study questionnaires from March 2014 to January 2018 were included. MEASURES: Demographic and disease characteristics, self-efficacy and symptoms related to each specific intervention were completed in addition to signalling items. General likelihood of using interventions was calculating by taking the mean score of the remaining signalling questions. PARTICIPANTS: 1060 participants with complete baseline data were included in the analyses. RESULTS: For all individual signalling questions, controlling for other variables, the mean of the remaining signalling questions was the strongest predictor (standardised regression coefficient ß from 0.61 (sleep) to 0.80 (self-management)). Smaller, but statistically significant, associations were found with the symptom associated with the respective signalling question and with general self-efficacy for 7 of 9 signalling questions. CONCLUSIONS: The main factor associated with patients' interest in participating in a disease-specific online self-care intervention is their general interest in participating in online interventions. Factors that may influence this general interest should be explored and taken into consideration when inviting patients to try online interventions.

5.
J Autoimmun ; : 102338, 2019 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-31570253

RESUMO

BACKGROUND: Progressive fibrosing interstitial lung disease (ILD) is rarely associated with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). This study focused on the outcomes of ILD patients with associated AAV (AAV-ILD). METHODS: AAV-ILD (cases: microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) with ILD) were compared to AAV patients without ILD (controls). ILD was defined as a usual interstitial pneumonia (UIP) or non-specific interstitial pneumonia (NSIP) pattern. Two controls were matched to each case for age (>or ≤65 years), ANCA status (PR3-or MPO-positive) and creatininemia (≥or <150 µmol/L). RESULTS: Sixty-two cases (89% MPO-ANCA+) were included. Median age at AAV diagnosis was 66 years. ILD (63% UIP), was diagnosed before (52%) or simultaneously (39%) with AAV. Cases versus 124 controls less frequently had systemic vasculitis symptoms. One-, 3- and 5-year overall survival rates, respectively, were: 96.7%, 80% and 66% for cases versus 93.5%, 89.6% and 83.8% for controls (p = 0.008). Multivariate analyses retained age >65 years (hazard ratio (HR) 4.54; p < 0.001), alveolar haemorrhage (HR 2.25; p = 0.019) and UIP (HR 2.73; p = 0.002), but not immunosuppressant use, as factors independently associated with shorter survival. CONCLUSION: For AAV-ILD patients, only UIP was associated with poorer prognosis. Immunosuppressants did not improve the AAV-ILD prognosis. But in analogy to idiopathic pulmonary fibrosis, anti-fibrosing agents might be useful and should be assessed in AAV-ILD patients with a UIP pattern.

6.
Presse Med ; 48(9): 919-930, 2019 Sep.
Artigo em Francês | MEDLINE | ID: mdl-31543394

RESUMO

Giant cell arteritis (GCA) is a large-vessel vasculitis involving the aorta and its main branches, especially supra aortic branches. Although much progress has been made, the pathophysiology remains incompletely understood. An initial trigger, suspected of infectious origin, lead to the maturation and recruitment of dendritic cells (DC). The lack of migration of these DC allows the local recruitment of T-lymphocytes (LT). These LT- CD4+ polarize in Type 1 helper (Th1), Th17 but also Th9. A qualitative and quantitative deficit in regulatory T cells (Treg) is observed under the influence of IL-21 overproduction. In addition, an imbalance in the Th17/Treg balance is favored by IL-6. The secretion of IFN-γ, IL-17, IL-6, IL-33 is responsible for a sustained local inflammatory reaction that is organized around tertiary lymphoid follicles. Locally recruited macrophages secrete reactive forms of oxygen together with VEGF and PDGF. These growth factors, together with neurotrophins and endothelin contribute to increase the proliferation of vascular smooth muscle cells (VSMCs). The imbalance between matrix metalloproteases (MMP)-2, MMP-9 and MMP-14 and tissue inhibitors of metalloproteases (TIMP)-1 and TIMP-2 also contribute to the remodeling process occurring in the vessel wall. Finally, arterial neovascularization contribute to the perpetuation of lymphocyte recruitment. This persistent remodeling is sometimes complicated by ischemic events responsible for the initial severity of the disease.


Assuntos
Arterite de Células Gigantes/fisiopatologia , Remodelação Vascular/fisiologia , Animais , Linfócitos B/fisiologia , Linfócitos T CD4-Positivos/fisiologia , Movimento Celular , Proliferação de Células , Células Dendríticas/fisiologia , Modelos Animais de Doenças , Predisposição Genética para Doença , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/etiologia , Arterite de Células Gigantes/patologia , Humanos , Interferon gama/metabolismo , Interleucina-17/metabolismo , Interleucina-33/metabolismo , Interleucina-6/fisiologia , Interleucinas/biossíntese , Ativação Linfocitária/fisiologia , Macrófagos/metabolismo , Metaloproteinases da Matriz/metabolismo , Músculo Liso Vascular/patologia , Neovascularização Patológica/fisiopatologia , Linfócitos T Reguladores/fisiologia , Células Th17/fisiologia
7.
Postgrad Med J ; 2019 Aug 29.
Artigo em Inglês | MEDLINE | ID: mdl-31467142

RESUMO

OBJECTIVES: This work aimed to study the prevalence and risk factors associated with well-being and career satisfaction among French internal medicine physicians and residents. METHODS: A total of 1689 French internal medicine physicians or trainees were surveyed to evaluate their workload, well-being and career satisfaction during February 2018. RESULTS: The response rate was 620/1689 (37%). The mean age of the participants was 37 years (±12); 49% of the participants were female, 27% worked in the Paris area, 74% worked in a university hospital and 49% were residents. Sixty-six per cent of the responders were satisfied with their work, and 66% would choose the internal medicine specialty again. However, 71% of the responders worked more than 50 hours a week, 21% worked more than 60 hours a week and 70% believed that they did not have enough time for personal/family activities. Twenty-five per cent of the responders had at least one sign of burnout (19% of the physicians in practice and 32% of the residents). Compared with the graduate physicians in practice, the residents worked more hours a week, had more activities at night, spent more time on administrative tasks, had a worse global appreciation of their work and felt that their work was less meaningful. In multivariate analysis, the factors associated with global satisfaction at work were autonomy and meaningful work. CONCLUSIONS: French internal medicine physicians have a high rate of career satisfaction. However, residents have a higher workload, less time for personal/family activities and feel that their work is less meaningful.

8.
Autoimmun Rev ; 18(9): 102353, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31323366

RESUMO

Recent studies show that relapsing polychondritis patients with tracheobronchial involvement are distinct from others in terms of clinical characteristics, therapeutic management, and disease evolution. Tracheobronchial involvement affects 20 to 50% of patients and may reveal the disease. It should be sought at the time of diagnosis and at each follow-up visit. Respiratory impairment is confirmed by computed tomography (CT) of the chest, including the cervical portion of the trachea, with end-inspiratory and dynamic expiratory scans, and pulmonary function tests. These investigations should be performed, even in asymptomatic patients, at the time of diagnosis, and repeated as necessary during follow-up. Bronchoscopy and a fortiori endoscopic intervention should be considered with caution and performed only by expert endoscopists after careful evaluation of the risks and benefits of such procedures, which can lead to damage or perforation of the airways and bronchospasm. Early detection and management of tracheobronchial involvement in relapsing polychondritis has significantly improved the prognosis of patients, especially with the development of interventional fiberoptic bronchoscopy. However, relapsing polychondritis-related morbidity and mortality are still elevated, particularly in tracheobronchial disease.


Assuntos
Broncopatias/etiologia , Policondrite Recidivante/complicações , Doenças da Traqueia/etiologia , Broncopatias/diagnóstico , Broncopatias/mortalidade , Broncopatias/terapia , Broncoscopia/métodos , Diagnóstico Diferencial , Diagnóstico Precoce , Intervenção Médica Precoce/métodos , Humanos , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/mortalidade , Policondrite Recidivante/terapia , Prognóstico , Sistema Respiratório/fisiopatologia , Análise de Sobrevida , Tomografia Computadorizada por Raios X , Doenças da Traqueia/diagnóstico , Doenças da Traqueia/mortalidade , Doenças da Traqueia/terapia
9.
Clin Exp Rheumatol ; 37 Suppl 117(2): 130-136, 2019 Mar-Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31162033

RESUMO

OBJECTIVES: Cardiovascular (CV) events are highly prevalent in systemic necrotising vasculitides (SNV). Visceral/subcutaneous adipose tissue (VAT/SAT) ratio has been shown to be associated with CV events in various diseases. We aimed to assess the relevance of abdominal adipose tissue measurement to predict major CV events (MCVEs) in SNV. METHODS: Patients with SNV were successively included in a longitudinal study assessing MCVEs and other sequelae. Dual x-ray absorptiometry was performed to evaluate abdominal adipose tissue. Patients were prospectively followed for MCVEs, defined as myocardial infarction, unstable angina, stroke, arterial revascularisation and/or hospitalisation for or death from CV causes. RESULTS: One hundred and twenty consecutive SNV patients were included and analysed (54 males, mean age 53±18 years). High CV risk was found in 28 (23.3%) patients. In univariate analysis, age, male gender, VDI, VAT/SAT ratio and serum troponin level were significantly associated with high CV risk, whereas age and VAT/SAT ratio remained independently associated with high CV risk. Variables associated with high tertile of VAT/SAT ratio included age and metabolic risk factors. After median follow-up of 42 months, 19 (16%) patients experienced MCVEs. Hazard ratios for incident MCVEs compared with 1st tertile of VAT/SAT ratio were 7.22 (1.02-51.3; p=0.048) and 9.90 (3.15-31.2; p=0.0002) in the 2nd and 3rd tertile, respectively. CONCLUSIONS: Abdominal visceral adipose tissue is a reliable surrogate marker of CV risk and predicts incident MCVEs in SNV patients. Abdominal adipose tissue should be probably evaluated routinely in these patients to assess CV risk.


Assuntos
Gordura Abdominal/metabolismo , Doenças Cardiovasculares , Vasculite Sistêmica/complicações , Tecido Adiposo/metabolismo , Adulto , Idoso , Doenças Cardiovasculares/diagnóstico , Feminino , Humanos , Gordura Intra-Abdominal , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Vasculite Sistêmica/metabolismo
10.
Artigo em Inglês | MEDLINE | ID: mdl-31219594

RESUMO

OBJECTIVES: To develop an International Classification of Functioning, Disability and Health (ICF) core set for SSc and to conceive a patient-centred ICF-based questionnaire assessing activities and participation in patients with SSc. METHODS: The construction of the ICF core set followed two steps. In the first step, meaningful concepts related to SSc were collected using data source triangulation from patients (n = 18), experts (n = 10) and literature (n = 174 articles). In the second step, concepts were linked to the best-matching ICF categories by one reviewer according to prespecified linking rules. Finally, patient-reported activities and participation categories of the ICF core set were translated into understandable questions. RESULTS: After linking concepts to ICF categories, 150 ICF categories were collected from focus groups, 22 from experts and 82 from literature. After fusion of the sources and removal of duplicates, the ICF core set included 164 categories: one at the first level, 157 at the second level and six at the third level, with 50 categories on body functions, 15 on body structures, 52 on activities and participation, and 47 on environmental factors. Patient-reported ICF categories on activities and participation were translated into a patient-centred ICF-based 65-item questionnaire. CONCLUSION: The present study proposes an ICF core set that offers a conceptual framework for SSc patients' care and health policy. Using a patient-centred approach, a patient-centred ICF-based questionnaire, the Cochin Scleroderma ICF-65 questionnaire, assessing activities and participation in patients with SSc, was conceived. TRIAL REGISTRATION: ClinicalTrials.gov, http://clinicaltrials.gov, NCT01848418.

12.
Kidney Int ; 96(2): 397-408, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31142442

RESUMO

Granulomatosis with polyangiitis (GPA) is an autoimmune vasculitis associated with anti-neutrophil-cytoplasmic antibodies (ANCA) against proteinase 3 leading to kidney damage. Neutrophils from those patients have increased expression of membrane proteinase 3 during apoptosis. Here we examined whether neutrophils from patients with GPA have dysregulated protein expressions associated with apoptosis. A global proteomic analysis was performed comparing neutrophils from patients with GPA, with healthy individuals under basal conditions and during apoptosis. At disease onset, the cytosolic proteome of neutrophils of patients with GPA before treatment was significantly different from healthy controls, and this dysregulation was more pronounced following ex vivo apoptosis. Proteins involved in cell death/survival were altered in neutrophils of patients with GPA. Several proteins identified were PR3-binding partners involved in the clearance of apoptotic cells, namely calreticulin, annexin-A1 and phospholipid scramblase 1. These proteins form a platform at the membrane of apoptotic neutrophils in patients with GPA but not healthy individuals and this was associated with the clinical presentation of GPA. Thus, our study shows that neutrophils from patients with GPA have an intrinsic dysregulation in proteins involved in apoptotic cell clearance, which could contribute to the unabated inflammation and autoimmunity in GPA. Hence, harnessing these dysregulated pathways could lead to novel biomarkers and targeted therapeutic opportunities to treat kidney disease.

13.
Artigo em Inglês | MEDLINE | ID: mdl-31056661

RESUMO

OBJECTIVE: In a previous controlled trial, 1-year adjunction of AZA to glucocorticoids (GC) for patients with non-severe, newly diagnosed eosinophilic granulomatosis with polyangiitis (EGPA) failed to lower remission failure, vasculitis relapse and isolated asthma/rhinosinus exacerbation rates, or cumulative GC use at month (M) 24. The aim of this study was to analyse longer-term outcomes to determine whether subsequent vasculitis relapse or isolated asthma/rhinosinus exacerbation (IARE) rates differed. METHODS: After M24, patients were followed prospectively, being treated based on physicians' best judgment. Flares and reasons for increased GC dose or immunosuppressant use were recorded, and reviewed according to randomization group to distinguish vasculitis relapses from IAREs according to EGPA Task Force recommendations. RESULTS: Fifty EGPA trial participants were followed for a median (interquartile range) of 6.3 (5.4-7.6) years; two (4%) died 11 months post-inclusion. By M24, vasculitis had relapsed in 21/49 (43%) patients and 14/50 (28%) had IAREs. Another patient died 4.8 years post-inclusion (infection). Among nine patients with subsequent vasculitis relapses, three had a major relapse and three had their first relapse after M24; among 25 patients with later IAREs, 17 occurred after M24. At 5 years, respective vasculitis relapse and IARE rates were 48% (95% CI 34.0, 62.6) and 56% (95% CI 41.7, 70.8), with no between-arm differences (P = 0.32 and 0.13). No entry clinical or biological parameter was associated with these outcomes during follow-up. CONCLUSION: These results confirmed that 1-year AZA and GC induction obtained good overall survival but no long-term benefit for non-severe EGPA patients. Vasculitis relapses, occurring mostly during the first 2 years, and IAREs, occurring throughout follow-up, require other preventive treatments. TRIAL REGISTRATION: ClinicalTrials.gov, https://clinicaltrials.gov, NCT00647166.

14.
J Rheumatol ; 2019 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-31043542

RESUMO

OBJECTIVE: Systemic sclerosis (SSc) is characterized by significant disability because of musculoskeletal involvement. Physical and occupational therapy (PT/OT) have been suggested to improve function. However, the rate of PT/OT use has been shown to be low in SSc. We aimed to identify demographic, medical, and psychological variables associated with PT/OT use in SSc. METHODS: Participants were patients with SSc enrolled in the Scleroderma Patient-centered Intervention Network (SPIN) Cohort. We determined the rate and indication of PT/OT use in the 3 months prior to enrollment. Multivariable logistic regression was used to identify variables independently associated with PT/OT use. RESULTS: Of the 1627 patients with SSc included in the analysis, 23% used PT/OT in the preceding 3 months. PT/OT use was independently associated with higher education (OR 1.08, 95% CI 1.04-1.12), having moderately severe small joint contractures (OR 2.09, 95% CI 1.45-3.03), severe large joint contractures (OR 2.33, 95% CI 1.14-4.74), fewer digital ulcerations (OR 0.70, 95% CI 0.51-0.95), and higher disability (OR 1.54, 95% CI 1.18-2.02) and pain scores (OR 1.04, 95% CI 1.02-1.06). The highest rate of PT/OT use was reported in France (43%) and the lowest, in the United States (17%). CONCLUSION: Despite the potential of PT/OT interventions to improve function, < 1 in 4 patients with SSc enrolled in a large international cohort used PT/OT services in the last 3 months. Patients who used PT/OT had more severe musculoskeletal manifestations and higher pain and disability.

16.
Autoimmun Rev ; 18(5): 493-500, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30844550

RESUMO

OBJECTIVE: To analyse the ocular manifestations of patients with GPA, their treatment and outcome. METHODS: Retrospective cohort study performed at the National Referral Center for Vasculitis, Cochin Hospital, Paris (France), from January 2005 to December 2015. Among 308 patients with a new diagnosis of GPA in accordance with the American College of Rheumatology classification criteria and/or revised Chapel Hill nomenclature definitions, we identified those with ocular involvement and a subsequent follow up in our center. RESULTS: The prevalence of ocular involvement in our GPA series was 38.6%; 63 patients were analysed with a median follow-up of 50.5 months (IQR: 17.8-82.8). Scleritis (18 patients, 28.6%) and episcleritis (18 patients, 28.6%) were the most common ophthalmologic manifestations, followed by orbital disease (13 patients, 20.6%). Bilateral involvement and visual acuity loss was seen in 29.1% and 16.7% of patients, respectively. Ocular involvement was the first GPA manifestation in 9 patients (14.3%), concomitant with systemic manifestation in 36 (57.1%), and occurred only during follow-up in 18 (28.6%). The indication for GPA treatment was suggested by ocular involvement in 12 patients (19.0%), by systemic features in 40 (63.5%) and by both ocular and systemic involvement in 11 (17.5%). Remission of ocular involvement was achieved in 51 patients (80.9%). In the remaining 12 (19.1%), symptoms persisted or even worsened, finally leading to rituximab (RTX) therapy in 8 of them (66.7%). Altogether, when used as first line or for refractory disease, ocular remission was achieved in 11 of the 12 cases (91.7%) treated with RTX versus 34 of the 47 cases (72.3%) treated with CYC (P = .260). Eye disease relapsed in 14 patients (22.2%). RTX allowed achievement of remission in 8 of them (57.1%). In the remaining six, other immunosuppressive drugs were used. CONCLUSIONS: Scleritis and episcleritis are the most common ocular manifestations in GPA, most of the time associated with other systemic manifestations. In >40% of cases, ocular manifestations were refractory to initial treatment or recurrent and led to RTX prescription, which appeared to be useful in these situations.


Assuntos
Oftalmopatias , Granulomatose com Poliangiite , Adulto , Estudos de Coortes , Oftalmopatias/diagnóstico , Oftalmopatias/epidemiologia , Oftalmopatias/etiologia , Oftalmopatias/terapia , Feminino , França/epidemiologia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/epidemiologia , Granulomatose com Poliangiite/terapia , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Rituximab/uso terapêutico , Resultado do Tratamento
17.
Radiology ; 291(2): 487-492, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30835186

RESUMO

Background Current imaging methods are not sensitive to changes in pulmonary function resulting from fibrosis. MRI with ultrashort echo time can be used to image the lung parenchyma and lung motion. Purpose To evaluate elastic registration of inspiratory-to-expiratory lung MRI for the assessment of pulmonary fibrosis in study participants with systemic sclerosis (SSc). Materials and Methods This prospective study was performed from September 2017 to March 2018 and recruited healthy volunteers and participants with SSc and high-resolution CT (within the previous 3 months) of the chest for lung MRI. Two breath-hold, coronal, three-dimensional, ultrashort-echo-time, gradient-echo sequences of the lungs were acquired after full inspiration and expiration with a 3.0-T unit. Images were registered from inspiration to expiration by using an elastic registration algorithm. Jacobian determinants were calculated from deformation fields and represented on color maps. Similarity between areas with marked shrinkage and logarithm of Jacobian determinants less than -0.15 were compared between healthy volunteers and study participants with SSc. Receiver operating characteristic curve analysis was performed to determine the best Dice similarity coefficient threshold for diagnosis of fibrosis. Results Sixteen participants with SSc (seven with pulmonary fibrosis at high-resolution CT) and 11 healthy volunteers were evaluated. Areas of marked shrinkage during expiration with logarithm of Jacobian determinants less than -0.15 were found in the posterior lung bases of healthy volunteers and in participants with SSc without fibrosis, but not in participants with fibrosis. The sensitivity and specificity of MRI for presence of fibrosis at high-resolution CT were 86% and 75%, respectively (area under the curve, 0.81; P = .04) by using a threshold of 0.36 for Dice similarity coefficient. Conclusion Elastic registration of inspiratory-to-expiratory MRI shows less lung base respiratory deformation in study participants with systemic sclerosis-related pulmonary fibrosis compared with participants without fibrosis. © RSNA, 2019 Online supplemental material is available for this article. See also the editorial by Biederer in this issue.

18.
Arthritis Rheumatol ; 71(6): 964-971, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30614663

RESUMO

OBJECTIVE: To generate a core set of items to develop classification criteria for scleroderma renal crisis (SRC) using consensus methodology. METHODS: An international, multidisciplinary panel of experts was invited to participate in a 3-round Delphi exercise developed using a survey based on items identified by a scoping review. In round 1, participants were asked to identify omissions and clarify ambiguities regarding the items in the survey. In round 2, participants were asked to rate the validity and feasibility of the items using Likert-type scales ranging from 1 to 9 (where 1 = very invalid/unfeasible, 5 = uncertain, and 9 = very valid/feasible). In round 3, participants reviewed the results and comments from round 2 and were asked to provide final ratings. Items rated as highly valid and feasible (median scores ≥7 for each) in round 3 were selected as the provisional core set of items. A consensus meeting using a nominal group technique was conducted to further reduce the core set of items. RESULTS: Ninety-nine experts from 16 countries participated in the Delphi exercise. Of the 31 items in the survey, consensus was achieved on 13, in the categories hypertension, renal insufficiency, proteinuria, and hemolysis. Eleven experts took part in the nominal group technique discussion, where consensus was achieved in 5 domains: blood pressure, acute kidney injury, microangiopathic hemolytic anemia, target organ dysfunction, and renal histopathology. CONCLUSION: A core set of items that characterize SRC was identified using consensus methodology. This core set will be used in future data-driven phases of this project to develop classification criteria for SRC.

19.
Crit Care ; 22(1): 328, 2018 Dec 04.
Artigo em Inglês | MEDLINE | ID: mdl-30514337

RESUMO

BACKGROUND: Acute respiratory failure related to diffuse alveolar hemorrhage (DAH) is a typical presentation of small-vessel vasculitis that requires prompt multidisciplinary management. The primary treatment is based on immunosuppressive drugs, whereas urgent plasma exchange has been proposed in case of life-threatening complications. We addressed the course of respiratory failure in 12 patients with ANCA-associated vasculitis-related DAH. PATIENTS AND METHODS: Observational retrospective case series performed in the medical ICU of a tertiary hospital in Paris, France. Consecutive patients with ANCA-associated DAH admitted to our ICU for acute respiratory failure and treated by plasmapheresis were included in the analysis. We evaluated the SpO2/FiO2 ratio and assessed the mechanical ventilation mode hourly for 7 days. RESULTS: Twelve patients were included. Five of them required invasive mechanical ventilation. All patients were treated by plasma exchange in addition to a combination of glucocorticoids and immunosuppressive agents. Oxygenation improved over the first 7 days following initiation of plasma exchange, as shown by a dramatic increase in the median SpO2/FiO2 ratio from 183 [interquartile 137-321] to 353 [239-432] (p = 0.003), along with a decrease in the level of ventilatory support. All but one patient survived. CONCLUSIONS: A multimodal induction regimen combining immunosuppressants and plasma exchange may rapidly reverse the respiratory dysfunction in ANCA-associated vasculitis-related DAH.

20.
Ocul Immunol Inflamm ; : 1-7, 2018 Nov 05.
Artigo em Inglês | MEDLINE | ID: mdl-30395752

RESUMO

PURPOSE: The diagnostic workup of uveitis is challenging, with 30 to 50% of cases remaining of undetermined etiology despite multiple investigations. Sarcoid granuloma-related increase of 1,25(OH)2D levels could be helpful for the diagnosis of ocular sarcoidosis. METHODS: Monocentric retrospective cohort study of patients for whom serum 25(OH)D and 1,25(OH)2D levels were measured during the etiologic workup of unexplained uveitis in a tertiary referral center. The diagnoses of uveitis' underlying diseases were established according to international diagnostic criteria. RESULTS: Fifty-nine patients were included. The diagnosis of defined, presumed or probable sarcoidosis was made in 37% of patients while 41% of cases remained of undetermined origin. The median serum levels of 25(OH)D in patients with ocular sarcoidosis and in those with uveitis due to another cause were 34.50 [21.2-40.8] and 43.20 [32.2-58.3] nmol/L (P=0.02), respectively. In the same subgroups of patients, the median serum levels of 1,25(OH)2D were 132.4 [107.4-163.9] and 108.0 [84.30-130.5] pmol/l (P=0.02), and the median 1,25(OH)2D/25(OH)D ratio was 4.17 [3.11-5.09] and 2.56 [1.54-3.37] (P=0.0007) respectively. A 1,25(OH)2D/25(OH)D ratio >3.5 was associated with the diagnosis of sarcoidosis with a 68 % sensitivity and a 78% specificity and, in univariate analysis, was associated with an abnormal chest CT-scan (OR=5.7, P=0.003), granulomas on bronchial biopsy (OR=14.7, P=0.007) and bronchoalveolar lavage fluid lymphocytosis (OR=12.4, P=0.0006). CONCLUSION: The measurement of serum 25(OH)D and 1,25(OH)2D levels is a useful tool in the etiological workup of patients with unexplained uveitis, since a high 1,25(OH)2D/25(OH)D ratio is suggestive of ocular sarcoidosis.

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