La Revue de medecine interne最新文献

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.

Fibrillary glomerulonephritis (FGN) is a glomerular disease described since 1977, with a prevalence in renal biopsies of less than 1%. It presents as renal failure, proteinuria, haematuria and hypertension in middle-aged adults. It is defined histologically, using light microscopy, which reveals organised deposits of fibrils measuring around 20nm, which are negative for Congo red staining. Electron microscopy, the first gold standard for diagnosis, has now been superseded by immunohistochemistry using the anti-DNAJB9 antibody. The discovery of this molecule has revolutionised the diagnosis of GNF, thanks to its excellent sensitivity and specificity (98% and 99% respectively). The association of GNF with hepatitis C virus, autoimmune diseases, neoplasia or haemopathy is debated. Renal prognosis is guarded, with 50% of patients progressing to end-stage renal failure within 2 to 4years of diagnosis. In the absence of randomised controlled trials, the recommended treatment is based on nephroprotective measures, corticosteroid therapy and possibly a second-line immunosuppressant such as rituximab. After renal transplantation, recovery or recurrence is possible. The pathophysiology of the disease is still poorly understood, and further studies are needed.

Introduction: Q fever is a zoonosis caused by Coxiella burnetii. Acute infection is mainly asymptomatic. In other cases it mainly causes a flu-like illness, a pneumonia, or an hepatitis. We present an atypical case of an acute Q fever revealed by a massive pleural effusion.

Case report: We report the case of a 43-year-old man referred to our hospital for an acute respiratory distress. Further analyses showed an exudative eosinophilic pleural effusion, associated with a pulmonary embolism and a deep femoral vein thrombosis. Aetiologic explorations revealed an acute Q fever (IgM and IgG against C. burnetii phase II antigens) associated with anti-phospholipids. The outcome was favorable with vitamin K antagonists, doxycycline, and hydroxychloroquine, till the negativation of the anti-phospholipid antibodies.

Discussion and conclusion: During acute C. burnetii infections, anti-phospholipid antibodies are highly prevalent but thrombotic complications are rare. The 2023 ACR/EULAR APS criteria restricts the diagnosis of APS, as in our case of acute severe infection. In front of an atypical pneumonia and/or thrombotic events, screening of C. burnetii and anti-phospholipid antibodies could be useful. Given its low level of evidence, prolongated treatment by doxycycline, hydroxychloroquine ± anticoagulant for C. burnetii's associated anti-phospholipid syndrome is discussed, but succeeded in our case.

Three prospective randomized studies have demonstrated the efficacy of autologous hematopoietic stem cell (HSC) transplantation in systemic sclerosis (SSc) on survival. These results encourage us to offer this therapy to patients who have a rapidly progressive disease and who have early symptoms but no advanced visceral involvement. HSC autograft can thus be discussed in patients with diffuse cutaneous SSc with a duration of the disease since the first visceral manifestations (cutaneous, cardiac, digestive, pulmonary, or renal) excluding Raynaud's phenomenon of less than 5 years. However, the indications for HSC autograft in SSc validated at European level and in the national diagnostic and care protocol (PNDS) are broader and some of these indications are debatable, in particular in patients with worsening diffuse interstitial lung disease. These indications are discussed in a reasoned way, taking into account the level of evidence and the toxicity of the HSC autograft.