Dermatomyosite à anticorps anti-MDA5 sévère associée à un cancer et contrôlée par inhibiteur de JAK

Dermatomyositis is an idiopathic inflammatory myopathy with various clinical and serological profiles, including poor prognosis forms for which aggressive immunosuppressive treatment is warranted. We report the case of a 60-year-old woman referred to our hospital for an anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis (MDA5 DM) with rapidly progressive interstitial pneumonia, typical cutaneous features and muscular impairment. Treatment with high-dose methylprednisolone, cyclophosphamide and gamma globulin was performed, but the patient remained corticodependant. Blood detection of positive interferon signature justified the administration of an anti-JAK1/2, leading to the clinical remission and the regression of the interferon signature. After 12 months of follow up, a small cell carcinoma was discovered, raising the question of a paraneoplastic syndrome, for which the most recent datas are quite reassuring for this kind of MDA5 DM. The presentation of this case is of twofold interest: describing one of the first report of successful treatment of intereronopathy MDA5 DM with ruxolitinib and highlighting an association with a cancer, which is not expected for this phenotype of dermatomyositis.     

Fibrillation atriale et allongement du QT sur hypomagnésémie secondaire à un traitement par inhibiteur de la pompe à protons

Proton pump inhibitors are widely prescribed but their long-term use can expose patients to adverse effects. Some of these are not very well known, including hypomagnesemia. Hypomagnesemia can be manifested by cardiac complications such as supraventricular arrhythmia or QT prolongation, increasing the risk of torsade de pointe. We present a case of atrial fibrillation triggered by severe hypomagnesemia secondary to proton pump inhibitor use and exacerbated by thiazide diuretic treatment. Conversion to sinus rhythm showed a prolonged corrected QT interval. We approach the pathophysiology and the electrophysiologic effects of hypomagnesemia.     

Rétinopathie sévère aux antipaludéens de synthèse

Introduction

Antimalarial drugs are largely used for the treatment of various systemic diseases. They can cause toxic retinopathy, which can lead to blindness.

Sténose tricuspide fonctionnelle sévère occasionnée par une importante végétation suite à une endocardite sur sonde de pacemaker

Lead-related infective endocarditis with negative hemocultures constitutes a severe condition potentially associated with a pejorative prognosis. It may induce a functional tricuspid stenosis caused by an important obstructive vegetation. We report the case of an 82 year-old woman in whom the lead-related endocarditis produced massive vegetation causing a severe functional tricuspid stenosis. We describe the medical history, diagnosis and treatment.     

Colchicine et inflammation oculaire non sévère hors maladie de Behcet : 16 cas et revue de la littérature

IntroductionColchicine is a narrow therapeutic margin drug that does not have the adverse effects of corticosteroids and immunosuppressants. Its use in non-severe ocular inflammatory disease excluding Behcet's disease has not been studied.MethodsWe included patients seen in the internal medicine department of Dijon University Hospital consecutively between September 2020 and September 2021 if they had received colchicine during their pathology. Patients with suspected Behçet's disease were excluded. Treatment efficacy was studied in patients with at least one year of disease progression who had received more than one year of colchicine. Successful treatment was defined as a 50 % reduction in the number of annual relapses on colchicine.ResultsSixteen patients were included (9 women and 7 men). They had recurrent anterior uveitis (n = 10), recurrent scleritis (n = 5) and intermediate uveitis. Opthalmological involvement was neither severe nor complicated. All patients combined, the annual relapse ratio (ARR) decreased from 1.8 (0.8–3.5) to 0.3 (0–1.6), (P = 0.06). Colchicine was considered effective in three of 10 analyzable patients. In only one patient, treatment was stopped for adverse effects after six weeks.ConclusionIn view of the interesting benefit-risk ratio of colchicine, it seems appropriate to focus on this molecule in non-granulomatous anterior uveitis and non-severe recurrent scleritis.     

Actualités physiopathologiques et thérapeutiques dans l’asthme sévère

Asthma is an inflammatory airway disease which presentation is highly heterogeneous. Last two decades provided new clinical and basic data concerning asthma physiopathology that make global understanding much complex. Phenotypes based on clinical settings and paraclinical investigations from large cohorts confirm old paradigm (eosinophilic vs. non-eosinophilic asthma) but also introduce new concepts (obesity-related asthma, late onset asthma, etc.). Conversely, improvement of big data analytics allows to initiate new cohorts aiming at better understanding the pathophysiology underlying those phenotypes and unraveling new ones. However, clinical and therapeutic impacts of those big data need to be further detailed. In parallel, biotherapies and innovative techniques as bronchial thermoplasty become available for severe asthmatic patients who did not respond to specific treatment in the past. Development of a personalized medicine in severe asthma becomes an important challenge for tomorrow. This review will focus on new pathophysiological concepts arisen from large cohorts and new therapeutic strategies available and in progress for severe asthma.