La Revue de médecine interne
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Pulmonary hypertension (PH) is a possible complication of connective tissue diseases (CTDs), especially systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). It is defined by an elevation of the mean pulmonary arterial pressure above 20mmHg documented during a right heart catheterization (RHC). Due to their multiorgan involvement, CTDs can induce PH by several mechanisms, that are sometimes intricated: pulmonary vasculopathy (group 1) affecting arterioles (pulmonary arterial hypertension, PAH) and possibly venules (pulmonary veno-occlusive-like disease), left-heart disease (group 2), chronic lung disease (group 3) and/or chronic thromboembolic PH (group 4). ⋯ Strategies for PH screening and RHC referral have been extensively investigated for SSc-PAH but data are lacking in other CTDs. Therapeutic management of PH depends of the underlying mechanism(s): PAH-approved therapies in group 1 PH (with possible use of immunosuppressants, especially in case of SLE or MCTD); management of an underlying left-heart disease in group 2 PH; management of an underlying chronic lung disease in group 3 PH; anticoagulation, pulmonary endartectomy, PAH-approved therapies and/or balloon pulmonary angioplasty in group 4 PH. Regular follow-up is mandatory in all CTD-PH patients.
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Review
[Diagnosis and evaluation of metabolic dysfunction associated steatotic liver disease (MASLD)].
Non-alcoholic fatty liver disease (NAFLD) or recently called Metabolic Dysfunction Associated Steatotic Liver Disease (MASLD), is the leading cause of liver disease, with an estimated worldwide prevalence of 25%. MASLD is suspected, in a metabolic condition, in the presence of hepatic steatosis, moderate hepatic cytolysis or hyperferritinemia. ⋯ Only a small minority of patients with MASLD will develop advanced liver disease and require liver specialist. Given the high prevalence of MASLD, the identification of these patients cannot be envisaged without the taking part in the screening of all physicians (general practitioners and specialists).
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Hemophagocytic lymphohistiocytosis is a rare syndrome with a poor prognosis, characterized by an uncontrolled dysregulation of the immune system. The rarity of this disease makes it difficult to obtain large cohorts. In this study, we analyzed the data of 66 patients: the objective was to describe the epidemiological, clinical, biological and therapeutic characteristics and to compare our results with those already published. ⋯ The study of the clinico-biological, epidemiological and survival data of the patients in our cohort allowed us to confirm previously published data but also to discuss some of them.