La Revue de médecine interne
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Hydroxychloroquine (HCQ), a synthetic antimalarial, is recognized for its immunomodulatory, anti-inflammatory and vascular-protective effects. In 20-30% of cases of primary obstetrical antiphospholipid syndrome (APS), the combination of antiplatelet aggregation and prophylactic anticoagulation fails to prevent obstetrical complications, a situation referred to as refractory obstetrical APS. This is partly due to the pro-inflammatory effects of antiphospholipid antibodies (aPL) binding to decidual and trophoblastic cells, which compromise embryonic implantation and placentation. ⋯ A few clinical studies of low scientific evidence suggest a benefit of HCQ in CIUE, but further data are needed. Finally, pre-eclampsia (PE) is another pregnancy-related condition at risk of recurrence, and its pathogenesis also seems to involve an imbalance in immune responses. HCQ's antioxidant properties could have a positive effect on endothelial dysfunction, a key component of PE.
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VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic), recently described, due to a somatic mutation of the UBA1 gene and often associated with hemopathy, is characterized by systemic symptoms close to those described in Still's disease or relapsing polychondritis. There are also patients with hemopathy, presenting inflammatory symptoms reminiscent of those of VEXAS syndrome but without mutation of the UBA1 gene. ⋯ The role of somatic mutations in the pathophysiology of autoinflammatory diseases associated with hematologic diseases must be better understood in order to better characterize them and develop targeted treatments.
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The platypnea orthodeoxia syndrome is a rare clinical entity combining positional dyspnea and arterial oxygen desaturation during the transition to orthostatism, reversible on return to decubitus. The most frequent etiology of this syndrome is the presence of a patent foramen ovale (PFO) responsible for a right-to-left intracardiac shunt, the severity of which results in significant functional disability and a risk of death from hypoxia. ⋯ The platypnea orthodeoxia syndrome associated with a patent foramen ovale often suffers from delayed diagnosis. Advanced age and co-morbidities should not prevent patients from undergoing percutaneous PFO closure, as the clinical benefit is important.
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The Script Concordance Tests (SCTs) are an examination modality introduced by decree in the French National Ranking Exam for medical students in 2024. Their objective is to evaluate clinical reasoning in situations of uncertainty. In practice, SCTs assess the impact of new information on the probability of a hypothesis formulated a priori based on an authentic clinical scenario. ⋯ Instead, the distribution of responses from a panel of experienced physicians is used to establish the question's scoring scale. Literature data suggest that physicians, even experienced ones, like most humans, often exhibit biased intuitive probabilistic reasoning. These biases raise questions about the relevance of using expert panel responses as scoring scales for SCTs.