Publications by authors named "M Gerfaud-Valentin"
Background: The PEXIVAS (Plasma exchange and glucocorticoids in severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis) trial showed that a reduced-dose glucocorticoid regimen (redGC) was non-inferior to a standard-dose regimen (standGC) with respect to death or end-stage kidney disease (ESKD) in patients with ANCA-associated vasculitis (AAV). However, the primary endpoint did not include disease progression or relapse, cyclophosphamide was the main induction therapy and rituximab (RTX)-treated patients tended to have a higher risk of death or ESKD with redGC. We aimed to evaluate the real-world use of redGC.
View Article and Find Full Text PDFAn open prospective, multicenter study enrolled 48 selected patients with chronic immune thrombocytopenia who achieved complete response for 1 year on thrombopoietin receptor agonists, half of the patients maintained a sustained response off treatment 4 years after treatment discontinuation. NCT03119974.
View Article and Find Full Text PDFArticle Synopsis
- Porphyrias are genetic disorders that affect heme production, with acute hepatic porphyrias (AHP) like acute intermittent porphyria (AIP) often mimicking neurological conditions, leading to potential misdiagnoses.
- A 41-year-old woman experienced symptoms resembling neuralgic amyotrophy but was ultimately diagnosed with AIP after finding elevated levels of porphobilinogen and delta-aminolevulinic acid, following an initial misdiagnosis.
- This case highlights the importance of considering AIP in patients with acute neuropathies, particularly when they present with abdominal symptoms, to ensure timely and effective treatment.
Granulomatous myositis is a clinical-pathological entity, which has been rarely reported, mostly described in sarcoidosis. Currently, no clear and simple prognostic factor has been identified to predict granulomatous myositis evolution. The clinical, anatomopathological, imaging, and biological characteristics of 26 patients with granulomatous myositis were retrospectively collected to describe clinical presentation and outcomes of this condition.
View Article and Find Full Text PDFArticle Synopsis
- The study investigates the differences between cerebral amyloid angiopathy-related inflammation (CAA-RI) and biopsy-positive primary angiitis of the CNS (BP-PACNS), focusing on their clinical and radiologic presentations as well as relapse rates.
- It included 104 patients with CAA-RI and 52 with BP-PACNS, revealing that CAA-RI tends to show more white matter lesions and hemorrhagic features, while BP-PACNS is associated more with headaches and motor deficits.
- The results indicate significant differences in features between the two conditions, suggesting that they may require different diagnostic approaches and could have varying outcomes in terms of recurrence.