ABSTRACT
Uncertainty in inherent to every aspects of medical practice. As the concept of uncertainty in healthcare is still to explore, deciphering the determinants and the roots of this uncertainty would benefit from the insights of various disciplines, such as epistemology, sociology, mathematics, or philosophy. The urgent need to improve physician's ability to cope with uncertainty, has been recently highlighted by the COVID-19 pandemic. Besides, the concept of uncertainty tolerance has been proposed, and could serve as a relevant basis for approaching uncertainty, in medical education. Thus, we propose at first to discuss the uncertainty tolerance framework from Hillen et al. Then, from an educational perspective, we outline some avenues regarding how uncertainty tolerance could be thought, in a competence-based approach, and discuss several educational activities, which have proven efficient in promoting uncertainty tolerance among medical practitioners abroad.
Subject(s)
COVID-19 , Education, Medical , Humans , Uncertainty , Pandemics , COVID-19/epidemiology , Curriculum , Clinical CompetenceABSTRACT
Systemic sclerosis (SSc) is an autoimmune disease associated to fibrotic manifestations. Interstitial lung disease (SSc-ILD), one of the main fibrotic features of SSc, is the first cause of SSc-related death. The management of SSc-ILD has recently benefited from the results of key randomised controlled trials. French authorities have approved Nintedanib for the treatment of SSc-ILD, and tocilizumab has recently been approved by the Food and Drug Administration (FDA) in the United-States (US). These recent approvals challenge the management of this fibrotic manifestation of SSc. This narrative literature review, at the crossroad of internal medicine and pulmonology, discusses what could be an up-to date approach, in terms of diagnostic and therapeutic strategies for SSc-ILD, in the light of the results from recent clinical trials.
Subject(s)
Lung Diseases, Interstitial , Scleroderma, Systemic , Humans , Lung , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/therapy , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/therapySubject(s)
Bone Density Conservation Agents , Osteoporosis, Postmenopausal , Osteoporosis , Adrenal Cortex Hormones/therapeutic use , Bone Density Conservation Agents/therapeutic use , Bone and Bones , Diphosphonates , Female , Humans , Osteoporosis/drug therapy , Osteoporosis/prevention & control , Osteoporosis, Postmenopausal/drug therapy , Osteoporosis, Postmenopausal/prevention & control , PostmenopauseABSTRACT
Hypersensitivity Pneumonitis (HP) is a common immune-mediated interstitial lung disease (ILD) induced by repeated exposure to environmental antigens in susceptible individuals. The most commonly known forms are bird fancier's disease and farmer's lung. However, the antigens involved are widely diverse. Therefore, the list of causes of HP is frequently expanding. HP seems to be under-diagnosed owing to its highly heterogeneous presentation in both the non-fibrotic and fibrotic subtypes and could represent up to 15% of all ILDs encountered in clinical practice. However, the recognition of HP cases is essential to ensure appropriate therapy for the patient. Home health care workers' intervention is sometimes critical in this context. In case of confirmed exposure, the diagnosis could be considered with high confidence if the high-resolution computed tomography (HR-CT) shows a typical HP pattern associated with a lymphocytosis over 30% in the broncho-alveolar lavage (BAL). In all other situations, the patients should undergo further investigations and additional histopathological sampling should be considered and submitted to a multidisciplinary team discussion. After diagnosis, antigenic eviction is the rule whenever possible. Corticosteroid treatment is the first-line medical treatment for severe forms and aims to prevent the development of fibrosis. Anti-fibrotic therapy is now an option for patients with progressive ILD and failure of immunomodulatory/immunosuppressive therapies.
Subject(s)
Alveolitis, Extrinsic Allergic , Farmer's Lung , Lung Diseases, Interstitial , Alveolitis, Extrinsic Allergic/diagnosis , Alveolitis, Extrinsic Allergic/epidemiology , Alveolitis, Extrinsic Allergic/etiology , Bronchoalveolar Lavage , Humans , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/epidemiology , Lung Diseases, Interstitial/etiology , Tomography, X-Ray ComputedABSTRACT
We conducted an observational retrospective study of all adults hospitalized for documented varicella-zoster virus (VZV) meningitis or encephalitis during years 2000-2015 in one referral centre. Thirty-six patients (21 males, 15 females) were included, with meningitis (n = 21), or meningoencephalitis (n = 15). Median age was 51 years [interquartile range, 35-76], and 6 patients (17%) were immunocompromised. Aciclovir was started in 32 patients (89%), with a median dose of 11 mg/kg/8 h [10-15]. No patient died, but 12 (33%) had neurological sequelae at discharge. Age was the only variable associated with adverse outcome (OR 1.98 [1.17-3.35] per 10-year increment, P = 0.011).
Subject(s)
Central Nervous System Infections/virology , Herpes Zoster/virology , Herpesvirus 3, Human/physiology , Acyclovir/therapeutic use , Adult , Aged , Antiviral Agents/therapeutic use , Central Nervous System Infections/drug therapy , Central Nervous System Infections/immunology , Female , Herpes Zoster/drug therapy , Herpes Zoster/immunology , Herpesvirus 3, Human/genetics , Herpesvirus 3, Human/isolation & purification , Humans , Immunocompromised Host , Male , Middle Aged , Retrospective StudiesABSTRACT
INTRODUCTION: Libman-Sacks endocarditis is a rare complication of antiphospholipid syndrome. Anti-vitamin K therapy is the standard treatment, although valvular replacement surgery may be required in some severe cases. In the latest EULAR recommendations, it is advised not to use direct oral anticoagulants in the management of antiphospholipid syndrome, especially of high-risk profile. CASE REPORT: We present a case of a mitral Libman-Sacks endocarditis complicated with multiple strokes occurring in the setting of an antiphospholipid syndrome with triple positive antibody profile in a 63-year-old woman with multiple sclerosis. She was previously treated with apixaban for two years. Tinzaparin followed by prolonged warfarine treatment and two months of hydroxychloroquine resulted in valvular improvement. CONCLUSION: To our knowledge, this is the first case of Libman-Sacks endocarditis occurring during apixaban therapy in a patient with antiphospholipid syndrome. This severe case highlights the inefficiency of direct oral anticoagulants to prevent thrombotic events in the antiphospholipid syndrome.
Subject(s)
Antiphospholipid Syndrome , Endocarditis , Lupus Erythematosus, Systemic , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Antiphospholipid Syndrome/drug therapy , Endocarditis/complications , Endocarditis/diagnosis , Endocarditis/drug therapy , Female , Humans , Middle Aged , Pyrazoles/adverse effects , Pyridones/adverse effectsABSTRACT
OBJECTIVE: Numerous guidelines have been published on the management of venous thromboembolism (VTE). However, therapeutic decision-making may prove challenging in routine clinical practice. With this in mind, multidisciplinary team (MDT) meetings have been set up in Rennes University Hospital, France. This study sought to describe the situations discussed during MDT meetings and to assess whether the meetings bring about changes in the management of these patients. MATERIALS AND METHODS: A retrospective single-center study conducted at the Rennes University Hospital included cases presented from the beginning of the MDT meetings (February 2015) up to May 2017. RESULTS: In total, 142 cases were presented in 15 MDT meetings, corresponding to a mean of 10±4 cases per meeting. Of these, 129 related to VTE patients: 33 provoked VTEs, 22 unprovoked VTEs, 49 cancer-related VTEs, and 25 unspecified VTEs. MDT meetings led to significant changes in the anticoagulation type (therapeutic, prophylactic, or discontinuation) and duration, but not in the anticoagulant choice (direct oral anticoagulants, vitamin K antagonists, heparins, etc.). CONCLUSION: Requests for MDT meetings are made for all VTE types, and these meetings have an impact on VTE management.
Subject(s)
Anticoagulants/administration & dosage , Blood Coagulation/drug effects , Clinical Decision-Making , Cooperative Behavior , Interdisciplinary Communication , Patient Care Team , Venous Thromboembolism/drug therapy , Administration, Oral , Anticoagulants/adverse effects , Drug Administration Schedule , France , Hospitals, University , Humans , Patient Selection , Retrospective Studies , Treatment Outcome , Venous Thromboembolism/diagnosis , Venous Thromboembolism/etiologyABSTRACT
INTRODUCTION: Our work aimed to investigate the illnesses unrelated to systemic sclerosis (IUSS), diagnosed among patients with systemic sclerosis (SSc) throughout their follow-up in a referral tertiary care center. METHODS: All the patients with SSc followed in the Internal Medicine Department of the University Hospital between October, 2014 and December, 2015, were included. We specifically reviewed the medical records of the patients who exhibited IUSS, defined as an illness that could not be considered as a typical clinical manifestation or as a usual complication of the disease. RESULTS: Two hundred patients were included, and 38 IUSS were diagnosed among 31 SSc patients, over a 4â¯years median follow-up period. These diagnoses included vascular diseases (26%), heart diseases (21%), neoplasia (8%), infectious diseases (6%), autoimmune diseases (5%), endocrinopathies (5%), and others (24%). The median follow-up time before IUSS diagnosis was two years. Seventeen (45%) of these diagnoses were considered in patients showing suggestive clinical signs. A specific therapy was delivered in 25 cases (66%). Group comparisons revealed that dyslipidemia was more frequent in patients with IUSS (ORâ¯=â¯2.6 [1.1-1.5]; pâ¯=â¯0.014), while no differences were found for the other characteristics. Especially, no association between auto-antibodies specificity and the occurrence of IUSS was found. CONCLUSION: This study focused on IUSS in SSc patients and highlights the need for a polyvalent clinical approach all along the follow up of SSc patients.
Subject(s)
Scleroderma, Systemic/epidemiology , Adult , Aged , Cohort Studies , Comorbidity , Female , Follow-Up Studies , France/epidemiology , Humans , Male , Middle Aged , Monitoring, Physiologic/statistics & numerical data , Retrospective Studies , Scleroderma, Systemic/diagnosis , Tertiary Care CentersSubject(s)
Bacteremia/diagnosis , Foot Diseases/diagnosis , Gammaproteobacteria , Gram-Negative Bacterial Infections/diagnosis , Myiasis/diagnosis , Osteitis/diagnosis , Amputation, Surgical , Anti-Bacterial Agents/therapeutic use , Bacteremia/microbiology , Bacteremia/parasitology , Bacteremia/therapy , Bone Diseases, Infectious/diagnosis , Bone Diseases, Infectious/microbiology , Bone Diseases, Infectious/parasitology , Bone Diseases, Infectious/therapy , Chronic Disease , Combined Modality Therapy , Foot Diseases/microbiology , Foot Diseases/parasitology , Foot Diseases/therapy , Gammaproteobacteria/isolation & purification , Gammaproteobacteria/physiology , Gram-Negative Bacterial Infections/microbiology , Gram-Negative Bacterial Infections/parasitology , Gram-Negative Bacterial Infections/therapy , Ill-Housed Persons , Humans , Male , Middle Aged , Myiasis/microbiology , Myiasis/parasitology , Myiasis/therapy , Osteitis/microbiology , Osteitis/parasitology , Wound Infection/diagnosis , Wound Infection/microbiology , Wound Infection/parasitology , Wound Infection/therapySubject(s)
Medication Reconciliation , Pharmacy Service, Hospital , Hospitals , Humans , Internal Medicine , PharmacistsABSTRACT
Objective: To compare the clinical presentation and outcome of giant cell arteritis (GCA)-related aortitis according to the results of temporal artery biopsy (TAB).Method: Patients with GCA-related aortitis diagnosed between 2000 and 2017, who underwent TAB, were retrospectively included from a French multicentre database. They all met at least three American College of Rheumatology criteria for the diagnosis of GCA. Aortitis was defined by aortic wall thickening > 2 mm on computed tomography scan and/or an aortic aneurysm, associated with an inflammatory syndrome. Patients were divided into two groups [positive and negative TAB (TAB+, TAB-)], which were compared regarding aortic imaging characteristics and aortic events, at aortitis diagnosis and during follow-up.Results: We included 56 patients with TAB+ (70%) and 24 with TAB- (30%). At aortitis diagnosis, patients with TAB- were significantly younger than those with TAB+ (67.7 ± 9 vs 72.3 ± 7 years, p = 0.022). Initial clinical signs of GCA, inflammatory parameters, and glucocorticoid therapy were similar in both groups. Coronary artery disease and/or lower limb peripheral arterial disease was more frequent in TAB- patients (25% vs 5.3%, p = 0.018). Aortic wall thickness and type of aortic involvement were not significantly different between groups. Diffuse arterial involvement from the aortic arch was more frequent in TAB- patients (29.1 vs 8.9%, p = 0.03). There were no differences between the groups regarding overall, aneurism-free, relapse-free, and aortic event-free survival.Conclusion: Among patients with GCA-related aortitis, those with TAB- are characterized by younger age and increased frequency of diffuse arterial involvement from the aortic arch compared to those with TAB+, without significant differences in terms of prognosis.
Subject(s)
Aortitis/pathology , Giant Cell Arteritis/pathology , Temporal Arteries/pathology , Aged , Aortitis/diagnostic imaging , Aortitis/mortality , Biopsy , Female , Giant Cell Arteritis/diagnostic imaging , Giant Cell Arteritis/mortality , Humans , Male , Middle Aged , Retrospective Studies , Tomography, X-Ray ComputedABSTRACT
Introduction: Schnitzler syndrome is an auto-inflammatory disease defined by chronic urticarial eruption and monoclonal gammopathy. 18F fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) is often performed, but its utility in Schnitzler syndrome has not been specifically investigated. The aim of this study was to determine whether PET/CT is informative in the diagnosis and follow-up of Schnitzler syndrome relative to other imaging techniques, including bone scans.Patients and methods: Patients of this study were selected from the French cohort established by Néel et al. All patients with a diagnosis of Schnitzler syndrome (according to Strasbourg's and Lipsker's criteria) who had at least one PET/CT were included. Data were collected from medical records. PET/CT scans were all reviewed by a nuclear physician blinded to the clinical and imaging data.Results: Ten patients underwent at least one PET/CT scan and all had at least one 99mTechnetium bone scan during their follow-up. The most frequent PET/CT abnormalities were diffuse bone-marrow and/or increased femoral fluorodeoxyglucose uptake, but they did not correlate with disease activity. Conversely, bone-scan abnormalities, including mainly increased radiotracer uptake in long bones, appeared to strongly correlate with Schnitzler syndrome activity.Discussion: PET/CT does not appear to be useful for the diagnosis and follow-up of Schnitzler syndrome. However, bone scans appear to be more sensitive for diagnosis and may correlate with clinical activity. Bone scans may be well positioned to distinguish Schnitzler syndrome relapse from other aetiologies of bone, joint, or muscle pain.Conclusion: Bone scans may be favoured over PET/CT in Schnitzler syndrome.
Subject(s)
Bone and Bones/diagnostic imaging , Pain/diagnostic imaging , Positron Emission Tomography Computed Tomography/methods , Schnitzler Syndrome/diagnostic imaging , Tomography, X-Ray Computed/methods , Aged , Aged, 80 and over , Bone and Bones/immunology , Bone and Bones/pathology , Cohort Studies , Female , Fluorodeoxyglucose F18/pharmacokinetics , Humans , Immunoglobulin G/blood , Immunoglobulin M/blood , Male , Middle Aged , Pain/blood , Pain/immunology , Pain/pathology , Radiopharmaceuticals/pharmacokinetics , Schnitzler Syndrome/blood , Schnitzler Syndrome/immunology , Schnitzler Syndrome/pathologyABSTRACT
INTRODUCTION: Sideroblastic anemia is a rare cause of microcytic anemia, which is characterized by ring sideroblasts on bone marrow aspirate. This anemia can be congenital or acquired. CASE REPORT: We report the case of an alcoholic 49-year-old man who presented with a severe microcytic sideroblastic anemia related to pyridoxine (B6 vitamin) deficiency. Acid folic deficiency was associated. The blood count normalized within one month after vitamin supplementation. CONCLUSION: Pyridoxine deficiency must be sought in sideroblastic anemia in patients at risk.
Subject(s)
Anemia, Sideroblastic/drug therapy , Vitamin B 6 Deficiency/drug therapy , Vitamin B 6/therapeutic use , Alcoholism/complications , Alcoholism/diagnosis , Alcoholism/drug therapy , Anemia, Sideroblastic/complications , Anemia, Sideroblastic/diagnosis , Humans , Male , Middle Aged , Treatment Outcome , Vitamin B 6 Deficiency/complications , Vitamin B 6 Deficiency/diagnosisABSTRACT
BACKGROUND: Data on survival and prognosis factors in incident cohorts are scarce in systemic sclerosis (SStc). To describe survival, standardized mortality ratio (SMR), and prognosis factors in systemic sclerosis (SSc), we analyzed a multicenter French cohort of incident patients and performed a systematic review of the literature and meta-analysis. METHODS: A multicenter, French cohort study was conducted between January 1, 2000, and December 31, 2013. Patients were followed-up until July 1, 2016. A systematic review of the literature was carried out in MEDLINE and EMBASE up to July 2017. Meta-analysis was performed using all available data on SMR and hazard ratios of prognosis factors. RESULTS: A total of 625 patients (493 females, 446 lcSSc) were included. During the study period, 104 deaths (16.6%) were recorded and 133 patients were lost to follow-up. Overall survival rates at 1, 3, 5, and 10 years from diagnosis were 98.0%, 92.5%, 85.9%, and 71.7% respectively in the French cohort. Overall SMR was 5.73 (95% CI 4.68-6.94). Age at diagnosis > 60 years, diffuse cutaneous SSc, scleroderma renal crisis, dyspnea, 6-min walking distance (6MWD), forced vital capacity < 70%, diffusing capacity of the lungs for carbon monoxide < 70%, pulmonary hypertension (PH), telangiectasia, valvular disease, malignancy, anemia, and CRP > 8 mg/l were associated with a poorer survival after adjustment. Eighteen studies (11,719 patients) were included in the SMR meta-analysis and 36 studies (26,187 patients) in the prognosis factor analysis. Pooled SMR was 3.45 (95%CI 3.03-3.94). Age at disease onset, male sex, African origin, diffuse cutaneous SSc, anti-Scl70 antibodies, cardiac and renal involvement, interstitial lung disease, PH, and malignancy were significantly associated with a worse prognosis. Anti-centromere antibodies were associated with a better survival. CONCLUSIONS: Overall, our study highlights a high mortality rate in SSc patients and confirms previously described prognosis factors related to skin extension and organ involvement while identifying additional prognosis factors such as autoantibody status, telangiectasia, 6MWD, and valvular disease.
Subject(s)
Multicenter Studies as Topic , Scleroderma, Diffuse/epidemiology , Scleroderma, Systemic/epidemiology , Adult , Aged , Cohort Studies , Female , France/epidemiology , Humans , Incidence , Kaplan-Meier Estimate , Male , Middle Aged , Prognosis , Scleroderma, Diffuse/diagnosis , Scleroderma, Diffuse/mortality , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/mortality , Survival RateABSTRACT
INTRODUCTION: Though several assessment tools for resident professional skills based on workplace direct observation have been validated, they remain scarcely used in France. The objective of this study was to evaluate the reliability and the validity of a workbook including several assessment forms for different components of the professional competency. METHODS: Three assessment forms have been tested over a period of 6 months in a multicentric study including 12 French internal medicine departments: the French version of the mini-CEX, an interpersonal skills assessment form (OD_CR) and the multisource feedback form (E_360). Reliability has been assess using the intra-class correlation coefficient (ICC) and the Cronbach alpha coefficient. Arguments for validity have been provided looking at the ability of the forms to detect an increase in the scores over time and according to the level of experience of the resident. RESULTS: Twenty-five residents have been included. The Cronbach alpha was of 0.90 (n=70) with the mini-CEX, 0.89 with the OD_CR (n=62) and 0.77 with the E_360 (n=86). ICC showed a wide variation according to the items of the mini-CEX and the OD-CR probably due to the poor number of observations performed by residents. The scores of most of the items of these two forms increased between M1 and M6. The scores of the E_360 were high: 7.3±0.8 to 8.3±2.4 (maximum 9) and did not vary according to the level of experience. CONCLUSION: This study suggest that it would be difficult to ensure a sufficient reliability for professional skills assessment using these tools given our available current human and material resources. However, these assessment forms could be added to the resident portfolio as supports for the debriefing in order to document their progression during their formation.
Subject(s)
Educational Measurement/methods , Internal Medicine/education , Internship and Residency , Clinical Competence , Educational Measurement/standards , Educational Status , France , Humans , Internal Medicine/standards , Internship and Residency/standards , Prospective Studies , Reference Standards , Reproducibility of Results , Research Design , Students, Medical/statistics & numerical dataABSTRACT
Hormonal exposure in young women increases the risk of venous thromboembolic disease (VTE). Thrombophilia testing is often proposed in women of childbearing age before the initiation of contraception. However, the presence of a familial history of VTE has the potential to be more accurate than the presence of inherited thrombophilia. OBJECTIVE: To demonstrate an association between the risk of VTE in young women with hormonal exposure (pregnancy or oral contraceptive use) and the presence of a previous episode of VTE in their first-degree relatives, according to whether or not a detectable inherited thrombophilia was present. METHODS: We will perform a multicenter case-control cross-sectional study. The main risk factor is defined by the presence of a symptomatic VTE in young women with hormonal exposure. The principle variable is the presence of an objectively diagnosed episode of VTE in first-degree relatives. We will need to include 2,200 family members in 440 cases. EXPECTED RESULTS: We expect to improve understanding of the thrombotic risk in first-degree relatives of patients in hormonal context with or without a past history of VTE.
Subject(s)
Hormones/physiology , Venous Thromboembolism/etiology , Adolescent , Adult , Age Factors , Case-Control Studies , Cross-Sectional Studies , Family , Female , Hormones/blood , Humans , Middle Aged , Pregnancy , Pregnancy Complications, Cardiovascular/blood , Pregnancy Complications, Cardiovascular/epidemiology , Pregnancy Complications, Cardiovascular/etiology , Risk Factors , Sex Factors , Thrombophilia/complications , Thrombophilia/epidemiology , Thrombophilia/genetics , Venous Thromboembolism/blood , Venous Thromboembolism/epidemiology , Venous Thromboembolism/genetics , Young AdultABSTRACT
BACKGROUND: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc). METHODS: In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema. RESULTS: Rate of CPFE in SSc patients with CT scan was 3.6%, and 7.6% among SSc patients with ILD. CPFE-SSc patients were more likely to be male (75 % vs 18%, p < 0.0001), smokers (83 % vs 33%, p < 0.0001), and to have limited cutaneous SSc (53 % vs 24% p < 0.01) than ILD-SSc controls. No specific autoantibody was significantly associated with CPFE. At diagnosis, CPFE-SSc patients had a greater decrease in carbon monoxide diffusing capacity (DLCO 39 ± 13 % vs 51 ± 12% of predicted value, p < 0.0001) when compared to SSc-ILD controls, whereas lung volumes (total lung capacity and forced vital capacity) were similar. During follow-up, CPFE-SSc patients more frequently developed precapillary pulmonary hypertension (PH) (44 % vs 11%, p < 10-4), experienced more frequent unscheduled hospitalizations (50 % vs 25%, p < 0.01), and had decreased survival (p < 0.02 by Kaplan-Meier survival analysis) as compared to ILD-SSc controls. CONCLUSIONS: The CPFE syndrome is a distinct pulmonary manifestation in SSc, with higher morbidity and mortality. Early diagnosis of CPFE by chest CT in SSc patients (especially smokers) may result in earlier smoking cessation, screening for PH, and appropriate management.