La Revue de medecine interne最新文献

[Hydroxychloroquine in systemic lupus erythematosus: Key updates]. 羟基氯喹治疗系统性红斑狼疮：最新进展。

La Revue de medecine interne Pub Date : 2025-06-05 DOI: 10.1016/j.revmed.2025.05.011

Ludovic Trefond

{"title":"[Hydroxychloroquine in systemic lupus erythematosus: Key updates].","authors":"Ludovic Trefond","doi":"10.1016/j.revmed.2025.05.011","DOIUrl":"https://doi.org/10.1016/j.revmed.2025.05.011","url":null,"abstract":"Hydroxychloroquine (HCQ) is a cornerstone treatment for systemic lupus erythematosus (SLE). Despite its long-standing use, recent studies have refined recommendations on dosage, toxicity, adherence, and benefits. The 2023 EULAR and 2024 KDIGO guidelines recommend a dose of 5mg/kg/day, with adjustments for renal impairment. However, reducing HCQ dose to≤5mg/kg/day increases the risk of moderate-to-severe flares (OR=6.04 [1.71-21.3]) and lupus-related hospitalizations (aOR=4.2 [1.45-12.19]). The prevalence of HCQ-related retinopathy reaches 8.6-11.5% after 15 years of use. Established risk factors include high daily doses, prolonged treatment, renal impairment, tamoxifen use, and pre-existing maculopathy. Recent studies have identified additional risks, including female sex (HR=3.83 [95% CI, 1.86-7.89]), darker skin phototypes (OR=5.5 [1.4-26.5]), serotonin-norepinephrine reuptake inhibitors (OR=6.6 [1.2-40.9]), an [HCQ]/[DCQ] ratio<7.2 (OR=8.4 [2.7-30.8]), and antiphospholipid syndrome (OR=8.9 [2.2-41.4]). The 2024 PNDS recommends annual ophthalmologic screening after five years of treatment. HCQ withdrawal significantly increases relapse risk, with severe flares occurring up to six times more frequently. A study on patients discontinuing HCQ due to retinopathy found a higher relapse rate compared to adherent patients (31.3 vs. 12.5%, OR=3.1 [1.2-8.2]). An algorithm for interpreting HCQ blood levels has been proposed, identifying levels below 200ng/mL as markers of poor adherence, correlating with an 80% missed-dose rate. Moreover, several studies confirm that HCQ is safe during pregnancy and does not increase the risk of congenital malformations. Finally, HCQ has been associated with a reduced risk of cardiovascular events in SLE. A study involving 52,883 patients found that HCQ use significantly lowered the incidence of cardiovascular events (OR=0.63 [0.57-0.69]). Recent evidence reinforces HCQ's essential role in SLE. Careful dose management, adherence monitoring, and ophthalmologic screening are crucial for optimizing treatment outcomes.","PeriodicalId":94122,"journal":{"name":"La Revue de medecine interne","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144251658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Fever in a 74-year-old man]. [74岁男子发烧]。

La Revue de medecine interne Pub Date : 2025-05-31 DOI: 10.1016/j.revmed.2025.05.014

L Frisson, F Aboubakar, G Zorzi, V Roelants, L Pothen, L Bamps, H Yildiz, A Benyamine

引用次数: 0

[Azygos continuation of the inferior vena cava associated with "H syndrome"]. [与“H综合征”相关的下腔静脉奇静脉延续]。

La Revue de medecine interne Pub Date : 2025-05-30 DOI: 10.1016/j.revmed.2025.05.013

Khaoula Boumeriem, Imrani Kaoutar, Ait Belhaj El Mahdi, Moatassim Billah Nabil, Nassar Itimad

引用次数: 0

[Recurrent blood problems in a 22 year-old woman]. [22岁女性复发性血液问题]。

La Revue de medecine interne Pub Date : 2025-05-29 DOI: 10.1016/j.revmed.2025.05.007

Nicolas Duhamel, Matthieu Buscot, Magalie Orlowski, Nihal Martis, Anaïs Roeser

引用次数: 0

What do patients with familial Mediterranean fever and the doctors who prescribe it know about colchicine? Results of a national survey of 255 patients and 203 doctors in France. 家族性地中海热患者和开处方的医生对秋水仙碱了解多少？这是一项对法国255名患者和203名医生进行的全国性调查结果。

La Revue de medecine interne Pub Date : 2025-05-27 DOI: 10.1016/j.revmed.2025.05.009

P Mertz, L Savey, M Delplanque, P Belamich, C Bayado, V Hentgen, S Georgin-Lavialle

引用次数: 0

[A lung mass]. [肺肿块]。

La Revue de medecine interne Pub Date : 2025-05-27 DOI: 10.1016/j.revmed.2025.05.012

Chaimae Abourak, Aya Laridi, Nidal El Hassani, Asmae Guennouni, Kaoutar Imrani, Ittimade Nassar

引用次数: 0

[Acid sphingomyelinase deficiency: A review]. [酸性鞘磷脂酶缺乏症：综述]。

La Revue de medecine interne Pub Date : 2025-05-24 DOI: 10.1016/j.revmed.2025.03.425

Martin Michaud, Wladimir Mauhin, Thomas Villeneuve, Olivier Lidove

{"title":"[Acid sphingomyelinase deficiency: A review].","authors":"Martin Michaud, Wladimir Mauhin, Thomas Villeneuve, Olivier Lidove","doi":"10.1016/j.revmed.2025.03.425","DOIUrl":"https://doi.org/10.1016/j.revmed.2025.03.425","url":null,"abstract":"Acid sphingomyelinase deficiency, formerly known as Niemann-Pick disease types B, A/B, and B, is a rare genetic disorder. It is an inherited autosomal recessive disease, linked to mutations in the SMPD1 gene. It is a lysosomal storage disease that leads to the accumulation of sphingomyelin mainly in macrophages, resulting in a multisystemic phenotype, primarily manifesting as hepatosplenomegaly and pulmonary involvement. The central nervous system may be affected, depending on the phenotype. Indeed, there is a phenotypic continuum among the three main forms that are distinguished: a chronic visceral form (formerly known as Niemann-Pick disease type B), a chronic neurovisceral form (intermediate form formerly known as Niemann-Pick disease type A/B), and an infantile neurovisceral form (formerly known as Niemann-Pick disease type A). The estimated overall prevalence is from 1 in 100,000 to 1 in 1,000,000 births. Clinical manifestations, as well as the age of onset of symptoms and/or diagnosis, vary depending on the form. The rarity of the disease and its nonspecific symptoms explain the diagnostic delay or failure to recognize the disease. Knowing and recognizing this disease is important, especially since there is an effective specific treatment through enzyme replacement therapy. In this article, we describe the clinical manifestations of acid sphingomyelinase deficiency, to understand when to suspect it, how to confirm it, and how to manage it.","PeriodicalId":94122,"journal":{"name":"La Revue de medecine interne","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144145280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Siltuximab as a salvage therapy in multi-refractory adult-onset Still's disease: A case series]. [西妥昔单抗作为多难治性成人发病斯蒂尔氏病的补救性治疗：一个病例系列]。

La Revue de medecine interne Pub Date : 2025-05-23 DOI: 10.1016/j.revmed.2025.05.002

Julie Moreau, Amélie Leurs, Emmanuel Ledoult, Sébastien Sanges, Pierre Quartier, Thibault Comont, David Launay, Eric Hachulla, Louis Terriou

{"title":"[Siltuximab as a salvage therapy in multi-refractory adult-onset Still's disease: A case series].","authors":"Julie Moreau, Amélie Leurs, Emmanuel Ledoult, Sébastien Sanges, Pierre Quartier, Thibault Comont, David Launay, Eric Hachulla, Louis Terriou","doi":"10.1016/j.revmed.2025.05.002","DOIUrl":"https://doi.org/10.1016/j.revmed.2025.05.002","url":null,"abstract":"Introduction: Adult-onset Still's disease (AOSD) is a systemic auto-inflammatory disorder characterized by a cytokine storm. Four major cytokines can be specifically targeted in severe, refractory or corticosteroid-dependent forms: interleukin 1 (IL-1), interleukin 6 (IL-6), interleukin 18 (IL-18), and Tumor necrosis factor alpha (TNFa). According to the French national protocol for adult-onset Still's disease, anti-IL-6 agents are the second-line of treatment after corticosteroids. We report here on the efficacy and safety of siltuximab prescribed off-label in patients with multi-refractory forms.Observations/results: These 4 patients, aged 24 to 74 years old, had polycyclic and inflammatory AOSD. The introduction of siltuximab was proposed after failure or poor tolerance of several lines of treatment. A partial or complete clinico-biological response was observed in all 4 patients.Conclusion: Siltuximab is a possible salvage treatment for multi-refractory AOSD.","PeriodicalId":94122,"journal":{"name":"La Revue de medecine interne","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144145291","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[From endemic to pandemic Mpox virus infection]. [从地方性到大流行的痘病毒感染]。

La Revue de medecine interne Pub Date : 2025-05-23 DOI: 10.1016/j.revmed.2025.05.008

Alexandre Robin, Yasmine Abi Aad, Nadia Valin, Romuald Cruchet, Pauline Naudion, Thibault Chiarabini, Quentin Richier

引用次数: 0

Immune checkpoint inhibitor-related eosinophilic fasciitis: 3 case reports with literature review. 免疫检查点抑制剂相关嗜酸性筋膜炎3例报告并文献复习。

La Revue de medecine interne Pub Date : 2025-05-20 DOI: 10.1016/j.revmed.2025.05.004

Mélanie Biteau, Vincent Sibaud, Alexandre Maria, Jérémie Dion, Emanuelle Uro-Coste, Aurore Siegfried, Andréa Pastissier, Pierre Cougoul, Karen Delavigne, Tthibault Comont, Valérian Rivet

{"title":"Immune checkpoint inhibitor-related eosinophilic fasciitis: 3 case reports with literature review.","authors":"Mélanie Biteau, Vincent Sibaud, Alexandre Maria, Jérémie Dion, Emanuelle Uro-Coste, Aurore Siegfried, Andréa Pastissier, Pierre Cougoul, Karen Delavigne, Tthibault Comont, Valérian Rivet","doi":"10.1016/j.revmed.2025.05.004","DOIUrl":"https://doi.org/10.1016/j.revmed.2025.05.004","url":null,"abstract":"Introduction: Immune checkpoint inhibitors (ICIs) are associated with a large spectrum of immune-related Adverse Events (irAEs). ICIs can exceptionally induce eosinophilic fasciitis (EF). Data of ICI-related EF (ICI-EF) are scarce without clear guidelines on both diagnosis and treatment.Methods: We conducted a literature review of all cases of patients over 18 years old with EF diagnosis following ICI treatment between 2016 and November 2023 and meeting Pinal-Fernandez's criteria. New cases from two departments (French Region of Occitania) were added to complete the cohort.Results: We selected 30 cases: 27 from literature review and 3 new cases diagnosed in Occitania. There was male predominance, with a sex ratio (M/F) of 1.3 with a median age of 57 years. The most common tumor was melanoma (50%). The time to onset was 10 months. Twenty-two patients (73%) had hypereosinophilia (mean 3300/mm3). A large majority (25/30, 83%) received at least one course of steroids therapy while immunosuppressive treatment was needed for 18 patients (72%). Rechallenge was proposed in 2 patients without recurrence of EF.Discussion: Clinical and biological characteristics appeared roughly similar between ICI-EF and idiopathic-EF. Management is currently based on clinical practice of idiopathic-EF with corticosteroids often used in first line of treatment. However, a second line (methotrexate of mycophenolate mofetil) was required in majority of patients as observed in idiopathic-EF.Conclusion: There is a significant reporting signal of EF following ICIs. A proactive management is required in all cases. A specific algorithm for treatment is finally proposed.","PeriodicalId":94122,"journal":{"name":"La Revue de medecine interne","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144121798","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0