[Mixed connective tissue disease and its management]. / La connectivite mixte et sa prise en charge.

Mixed connective tissue disease (MCTD) is a rare autoimmune condition. Since its first description 50 years ago, its mere existence has been debated, given that it shares features of other autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic sclerosis, inflammatory myopathy, rheumatoid arthritis and Sjogren's syndrome. Also, while antibodies to U1-RNP are essential for the diagnosis of MCTD, these antibodies may be expressed in other circumstances, such as in case of SLE. Nevertheless, the patient fulfilling criteria for MCTD needs specific management. In this review, we describe the clinical features and the potential complications of this complex disease, often wrongly disregarded as benign. We will also emphasize the recommended follow-up exams and address treatment, which is currently lacking formal recommendations.

La connectivite mixte (mixed connective tissue disease (MCTD)) est une maladie auto-immune rare. Dès sa description il y a cinquante ans, l'existence propre de la MCTD est débattue, car les limites avec d'autres maladies, comme le lupus érythémateux systémique (LES), la sclérodermie, les myopathies inflammatoires, la polyarthrite rhumatoïde et le syndrome de Sjögren, sont floues. Les anticorps anti-U1-RNP obligatoires au diagnostic de MCTD sont également exprimés dans d'autres circonstances, comme le LES. Quoi qu'il en soit, le patient présentant des critères de MCTD nécessite une prise en charge spécifique. Nous présentons ici les signes cliniques et complications potentielles d'une maladie longtemps estimée à tort comme d'évolution bénigne. Nous abordons aussi les examens de suivi recommandés et la thérapeutique, qui reste à ce jour mal définie.

[Understanding hypersensitivity reactions to monoclonal antibodies]. / Comprendre les réactions d'hypersensibilité aux anticorps monoclonaux.

Biologic drugs are complex molecules synthesized by a living organism. Their use is increasingly prevalent across all medical specialties, exposing a growing number of patients to potential adverse reactions. In this review, we discuss the new classification of hypersensitivity reactions, along with the specific characteristics of monoclonal antibodies. We also address the available diagnostic tools and discuss the management of those reactions, including for patients requiring the continuation of these biologic drugs.

Les médicaments biologiques sont des molécules complexes synthétisées par un organisme vivant. Ils sont de plus en plus utilisés dans toutes les spécialités médicales, exposant ainsi les patients à des réactions indésirables. Dans cet article, nous abordons la nouvelle classification des réactions d'hypersensibilité ainsi que les caractéristiques spécifiques des anticorps monoclonaux. Nous évoquons également les outils diagnostiques disponibles et discutons de la prise en charge, y compris pour les patients nécessitant la poursuite de l'administration des médicaments biologiques.

[Hymenoptera venom allergy: what's new in 2023]. / Hypersensibilité aux venins d'hyménoptères : le point en 2023.

Hymenoptera venom allergy is a central thematic in allergology. The recent limitation in the obtention of certain venom products has forced Swiss centers to adapt their diagnostic and therapeutical approaches. In this review, we will discuss diagnostics tools using recombinants serologies, recent recommendations for the screening of indolent systemic mastocytosis and the different immunotherapy protocols available for venom desensitization using aqueous and aluminum hydroxide-adsorbed purified venoms.

L'hypersensibilité aux venins d'hyménoptères est une thématique importante en allergologie. La disponibilité limitée des produits de désensibilisation a forcé les centres universitaires suisses à adapter leurs pratiques médicales diagnostique et thérapeutique. Dans cet article, nous discutons des différentes sérologies recombinantes disponibles, comment aborder le dépistage de la mastocytose systémique indolente et, finalement, les différents schémas de désensibilisation à base d'une formulation aqueuse ou dépôt adsorbé sur de l'hydroxyde d'aluminium.

[Difficult to treat auto-immune diseases : when glucocorticoids are not enough]. / Allergologie-immunologie - Maladies autoimmunes complexes : quand les glucocorticoïdes ne suffisent plus.

Glucocorticosteroids (GC) remain the mainstay of treatment in most systemic inflammatory diseases. GC have a broad anti-inflammatory action of rapid onset. The downsides of prolonged GC therapy are well established and include infections, osteoporosis and metabolic adverse effects, among others. In systemic sclerosis, GC are associated with an increased risk of scleroderma renal crisis and must be avoided. Adjunction of second-line immunosuppressive drugs may improve disease control and limit GC usage. We summarize here the findings of two studies published in 2021, one reporting the benefits of combining GC with mycophenolate mofetil in immune thrombocytopenia, the other suggesting that blockage of interleukin-6 may decrease disease progression in systemic sclerosis with lung involvement.

Les glucocorticoïdes (GC) demeurent le pilier du traitement de la plupart des maladies inflammatoires systémiques. Ils ont un effet anti-inflammatoire puissant et d'installation rapide. Les effets indésirables des GC sont bien connus : risque infectieux, ostéoporose, diabète et hypertension, entre autres. Dans la sclérose systémique, les GC sont à éviter, car associés à la survenue d'une crise rénale hypertensive. L'adjonction d'immunosuppresseurs comme deuxième ligne de traitement peut améliorer le contrôle de la maladie et limiter l'utilisation des GC. Nous résumons les résultats de deux études récentes, l'une rapportant les bénéfices de l'ajout de mycophénolate mofétil aux GC dans le purpura thrombopénique immun, l'autre suggérant que le blocage de l'interleukine 6 pourrait ralentir la progression de la sclérose systémique (ou sclérodermie) avec atteinte pulmonaire.

[Immune checkpoint inhibitors and renal toxicity†: update about an emerging pathology]. / Inhibiteurs de points de contrôle immunitaires et atteinte rénale†: mise au point sur une pathologie émergente.

Recently, comprehension of immune mechanisms involved in anti-tumor responses has permitted the development of new oncologic drugs called immune checkpoint inhibitors. These drugs act by restoring anti-tumor responses. With their increasing use, we note a rise in the incidence rate of immune related adverse events, which can affect many organs. Renal toxicity, more precisely tubulointerstitial nephritis, is still not well understood but an emerging complication.

Récemment, la compréhension des mécanismes immuns impliqués dans la réponse antitumorale a permis de développer de nouveaux traitements oncologiques, les inhibiteurs de points de contrôle immunitaires. L'action de ceux-ci repose sur une rupture des mécanismes de tolérance immune envers la tumeur. Avec leur développement, on observe l'apparition d'effets indésirables d'un nouveau genre, s'apparentant à une autoimmunité et touchant différents systèmes. La toxicité rénale, sous la forme d'une néphrite tubulo-interstitielle, est une complication encore méconnue mais émergeante.

Near fatal intoxication by nicotine and propylene glycol injection: a case report of an e-liquid poisoning.

BACKGROUND: Concern about intoxication by e-liquid is growing as calls to poison control centers have increased since their introduction. Only three cases of intoxication by injection have been reported worldwide. Our case is unique because of the precise follow-up of a patient who survived a lethal dose of self-injected e-liquid, without other co-intoxication. CASE PRESENTATION: A 51-year-old male presented to the Emergency Department after injecting himself intravenously (IV) in the forearm with 10 mL of e-liquid (1000 mg of nicotine diluted in propylene glycol). An agitation phase was followed by coma and bradypnoea requiring mechanical ventilation. The patient developed a transitory neurological impairment with the appearance of tetraparesis, gaze palsy and myoclonus due to nicotinic syndrome. The arterial blood gas (ABG) analysis confirmed uncompensated lactic acidosis with an elevated anion gap, which is an expected effect of propylene glycol. The toxicology screen indicated the presence of nicotine and cotinine in the blood and excluded the presence of concomitant intoxication. The patient recovered without sequelae. CONCLUSION: Even a small quantity of intravenous (IV) e-liquid can lead to an acute intoxication and fatal outcomes due to the toxic effects of nicotine. This case might help emergency doctors cope with acute intoxication by injection of e-liquid and increase their comprehension of the two main substances, nicotine and propylene glycol with overview of their pharmacodynamics and kinetic effects.

Performance increase in venous drainage for mini-invasive heart surgery: superiority of self-expanding cannulas.

OBJECTIVE: Originally, the Smartcanula principle (collapsed insertion and expansion in situ) was developed for venous drainage by gravity. However, in minimally invasive surgery, augmentation with either constrained force vortex pumps or vacuum is often used. The current study was set up to assess whether smaller diameters of self-expanding venous cannulas are sufficient in conjunction with venous drainage augmentation resulting in smaller access orifices. METHODS: To evaluate cannulas intended for cardiopulmonary bypass, an in vitro circuit was set up with silicone tubing between the test cannula encased in a lower reservoir, the centrifugal pump, and after an upper reservoir. Afterload was set arbitrarily at 60 mm Hg using a centrifugal pump. The pressure value was measured using Millar pressure transducers. Flow rate (Q) was measured using an ultrasonic flow meter calibrated with volume tank and timer. Revolutions per minute of the centrifugal pump were calibrated with a stroboscope. Data display and data recording were controlled using a Lab View application. Self-expanding (24F Smartcanula) and control (25F Biomedicus) cannulas were used. RESULTS: Sixty measurements were recorded. At pump speed of 1500, 1570, 2000, 2500, and 3000 rpm, the Q values were 3.6, 5.2, 6.6, 9.3, and 11.8 L/min for the 24F self-expanding cannula and 3, 4.3, 5.4, 7.5, and 9.3 L/min for the control cannula. The pressure values were 3.6, -5.4, -15.9, -45.3, and 80.6 mm Hg. Biomedicus 25F showed Q values from 16% to 19% less as compared with 24F Smartcanula. The pressure values were 6, 7, 4, 2, and 2 times more as compared with 24F Smartcanula. CONCLUSIONS: Our experimental evaluation demonstrated the superior performance of the Smartcanula with its self-expanding design in comparison with the reference commercially available standard cannulas. The Smartcanula with its small diameter is particularly welcome for minimally invasive surgery.