Publications by authors named "C Blanchard-Delaunay"
Background: Angioedema (AE) due to acquired C1-inhibitor deficiency (AAE-C1-INH) is a rare disease associating recurrent edema of mucosa and skin. Several underlying diseases have been reported, mainly lymphoproliferative diseases and monoclonal gammopathy. However, 15 to 20% of patients never exhibit such a hematological condition.
View Article and Find Full Text PDFAngioedema due to Acquired C1-Inhibitor Deficiency Associated With Monoclonal Gammopathies of Undetermined Significance Characteristics of a French National Cohort.
J Allergy Clin Immunol Pract
December 2024
Article Synopsis
- MGUS-associated angioedema due to acquired C1 inhibitor deficiency (AAE-C1-INH) hasn't been specifically characterized before, prompting this study to explore its biological and clinical features over 30 years in France.
- In a study of 41 patients, most had anti-C1INH antibodies, and treatments included acute management and long-term prophylaxis, with a significant number developing malignant blood disorders like lymphoma or myeloma.
- The study found a link between the remission of angioedema and the underlying hematological malignancy, emphasizing the need for regular hematological evaluations in patients with MGUS-AE-C1-INH.
Article Synopsis
- Angioedema due to acquired C1-inhibitor deficiency (AAE-C1-INH) can result from excess consumption of C1-INH or the presence of anti-C1-INH antibodies, commonly linked to lymphoid cancers or monoclonal gammopathies.
- A study in France assessed the effectiveness of rituximab for treating 55 patients with AAE-C1-INH, revealing that 34 patients achieved remission after an average of nearly 4 years of follow-up.
- The results indicated that patients without anti-C1-INH antibodies had a better chance of remission; those with lymphoma and those treated with rituximab and chemotherapy experienced lower rates of relapse, suggesting that rituximab
Importance: Results of randomized clinical trials have demonstrated rituximab's noninferiority to cyclophosphamide as induction therapy for antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), with neither treatment having a specific advantage for granulomatosis with polyangiitis (GPA). However, post hoc analysis results have suggested that rituximab might be more effective than cyclophosphamide in inducing remission in patients with proteinase 3-positive AAV.
Objective: To compare the effectiveness of rituximab and cyclophosphamide in inducing GPA remission in a large population of unselected patients.
Article Synopsis
- Hereditary angioedema (HAE) is a serious condition causing unpredictable swelling attacks, and over the past decade, new treatments for long-term prophylaxis (LTP) have emerged based on a better understanding of its biology.
- A study conducted in France in 2020 surveyed physicians from 25 expert centers about their practices, treatments, and the unmet needs of HAE patients; ultimately focusing on 20 centers with active patient files.
- Of the 714 patients with C1 inhibitor deficiency, 59.2% were on LTP, but many still faced unmet needs related to treatment tolerability, efficacy, and cost; physicians expressed a desire for more effective, better-tolerated, and affordable therapies