Publications by authors named "Puechal X"
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 PDFArticle Synopsis
- The study aimed to assess the effectiveness of combining glucocorticoids (GCs) with cyclophosphamide (CYC) for treating patients with poor-prognosis eosinophilic granulomatosis with polyangiitis (EGPA) compared to using GCs alone.
- Data from a European multicenter database were analyzed, involving 209 patients and focusing on relapse rates and other related outcomes over 12 to 24 months.
- Results indicated that adding CYC significantly reduced the risk of relapse and related complications compared to GCs alone, suggesting a beneficial role for CYC in the treatment of poor-prognosis EGPA.
Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a heterogenous autoimmune disease. While traditionally stratified into two conditions, granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), the subclassification of ANCA-associated vasculitis is subject to continued debate. Here we aim to identify phenotypically distinct subgroups and develop a data-driven subclassification of ANCA-associated vasculitis, using a large real-world dataset.
View Article and Find Full Text PDFTargeted therapy has revolutionized the management of ANCA-associated vasculitis (AAV) over the last fifteen years. Rituximab, an approved induction and maintenance agent for severe AAV, is no less effective than cyclophosphamide as induction therapy and particularly useful in relapsing or refractory disease, or in women. In patients with relapsing AAV, granulomatosis with polyangiitis or PR3-ANCA, it is more effective than cyclophosphamide.
View Article and Find Full Text PDFLarge-vessel involvement in ANCA-associated vasculitis: A multicenter case-control study.
Semin Arthritis Rheum
August 2024
Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) primarily affects small vessels. Large-vessel involvement (LVI) is rare. We aimed to describe the characteristics of LVI, to identify associated risk factors, and to describe its therapeutic management.
View Article and Find Full Text PDFObjectives: Tropheryma whipplei infection can manifest as inflammatory joint symptoms, which can lead to misdiagnosis of inflammatory rheumatic disease and the use of disease-modifying antirheumatic drugs. We investigated the impact of diagnosis and treatment of Tropheryma whipplei infection in patients with inflammatory rheumatic disease.
Methods: We initiated a registry including patients with disease-modifying antirheumatic drugs-treated inflammatory rheumatic disease who were subsequently diagnosed with Tropheryma whipplei infection.
Objectives: B-cell depletion induced by rituximab (RTX) in ANCA-associated vasculitis (AAV) is a risk factor for hypogammaglobulinemia. Aggregating data on gammaglobulin levels kinetics during RTX and its association with the risk of relapse and severe infection is of interest.
Methods: Gammaglobulin levels were collected before induction therapy and during RTX maintenance therapy.
Objectives: Neutrophils play a key role in ANCA-associated vasculitis, both as targets of autoimmunity and as facilitators of vascular damage. In granulomatosis with polyangiitis (GPA), the data regarding the production of reactive oxygen species (ROS) in neutrophils are unclear. Further, recent data suggests that ROS production could have an anti-inflammatory effect through the regulation of inflammasomes and IL-1-related cytokines.
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- Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of small vessel vasculitis often causing severe asthma that depends on glucocorticoids for treatment.
- A study evaluated the effectiveness of mepolizumab at a dosage of 100mg every 4 weeks over 12 months for patients with EGPA-related asthma.
- Results showed that 62% of patients responded positively to mepolizumab, with responders having higher lung function and eosinophil levels compared to non-responders.
Article Synopsis
- - The study aimed to compare how effective and safe different treatment schedules with azathioprine (AZA) and rituximab (RTX) are in preventing relapses in patients with ANCA-associated vasculitis who are in complete remission.
- - Researchers conducted trials that compared 18-month fixed RTX to AZA, 18-month fixed RTX to 18-month tailored RTX, and extended 36-month RTX therapy versus placebo, tracking patient outcomes over 84 months.
- - Findings indicated that the 18-month fixed RTX regimen was significantly better at preventing major relapses compared to AZA and the tailored RTX approach, leading to a higher remission rate at the 84-month mark.
Article Synopsis
- This study focuses on describing the structure and harmonization of six antineutrophil cytoplasmic antibody-associated vasculitis (AAV) registries, emphasizing data quality and patient outcomes.
- The researchers used a specialized ontology to align data across registries and employed SPARQL for data retrieval, finding that over 5,000 AAV cases showed variations in data completeness and correctness.
- The findings revealed significant information on treatment methods and patient characteristics but highlighted challenges in comparing outcomes due to different recruitment settings and data quality issues.
Background: Benralizumab is effective in the treatment of eosinophilic asthma and is being investigated for the treatment of other eosinophil-associated diseases. Reports on the use of benralizumab for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) are limited to case reports and small case series.
Methods: We conducted a multicentre, retrospective study including EGPA patients treated with off-label benralizumab.
Article Synopsis
- A retrospective study on polyarteritis nodosa (PAN) analyzed data from 196 patients referred to a French study group between 2005 and 2019, highlighting significant changes in the disease's landscape.
- Main symptoms included constitutional, neurological, skin, and musculoskeletal issues, with 28% of cases being secondary PAN due to conditions like myelodysplastic syndrome and cancers; most patients (98.5%) were treated with glucocorticoids.
- The study found high relapse rates associated with older age and specific clinical features, with mortality linked to factors like age, necrotic purpura, kidney injury, and secondary PAN, emphasizing the need for improved patient management.
Article Synopsis
- A study was conducted to investigate the characteristics and outcomes of ANCA-associated vasculitis (AAV) caused by anti-thyroid drugs (ATDs), specifically focusing on microscopic polyangiitis (MPA).
- The research included 45 patients with ATD-induced AAV, and it was found that most had positive ANCA results, with skin complications and joint pain being the primary symptoms.
- Compared to primary MPA, ATD-induced MPA patients were younger, had more skin problems and fewer kidney issues, and experienced a lower risk of disease relapse after treatment.
Article Synopsis
- A study compared two ways of giving a medicine called tocilizumab to patients with a disease called Takayasu arteritis (TAK).
- They looked at 109 patients from different countries and found that both methods worked similarly well after 6 months, with about 69% showing improvement.
- However, patients who got tocilizumab as a shot under the skin had a higher chance of getting worse again compared to those who received it through an IV.
Objectives: To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests.
Methods: This retrospective multicentre case-control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests.
Objective: Interindividual variability in response to rituximab remains unexplored in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Rituximab pharmacokinetics (PK) and pharmacodynamics (PD) as well as genetic polymorphisms could contribute to variability. This ancillary study of the MAINRITSAN 2 trial aimed to explore the relationship between rituximab plasma concentration, genetic polymorphisms in PK/PD candidate genes, and clinical outcomes.
View Article and Find Full Text PDFObjective: To develop a score assessing the probability of relapse in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).
Methods: Long-term follow-up data from GPA and MPA patients included in five consecutive randomised controlled trials were pooled. Patient characteristics at diagnosis were entered into a competing-risks model, with relapse as the event of interest and death the competing event.
Article Synopsis
- - The study examined the characteristics of granulomatosis with polyangiitis (GPA) in patients who experienced induction failure, focusing on different treatments and how effective salvage therapies were from 2006 to 2021.
- - A total of 51 patients with GPA and induction failure were analyzed, revealing that those treated with intravenous cyclophosphamide often had more severe symptoms like relapsing disease and orbital masses compared to controls, while those on rituximab showed significant renal complications.
- - After trying salvage therapies, 69% of patients achieved remission, with a notable success in switching therapies; 50% of those inadequately responding to cyclophosphamide improved with rituximab, while patients progressing
Article Synopsis
- The study aimed to evaluate the prognostic value of chest high-resolution CT (HRCT) in patients with eosinophilic granulomatosis with polyangiitis (EGPA), a condition that hasn't been extensively analyzed using this imaging method.
- Researchers conducted a retrospective analysis of 46 EGPA patients from three French hospitals, assessing HRCT images for abnormal findings and correlating these with clinical outcomes.
- The results showed distinct HRCT patterns among patients, with those in one group (ground-glass pattern) having worse prognoses and higher rates of steroid dependency, whereas another group (bronchial pattern) exhibited better outcomes and higher remission rates, suggesting HRCT can inform better management strategies in EGPA.