Publications by authors named "Beatrice Bibes"
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.
Efficacy and Safety of Rituximab-Based Treatments in Angioedema With Acquired C1-Inhibitor Deficiency.
J Allergy Clin Immunol Pract
January 2024
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
Background: The systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a very rare condition characterized by recurrent life-threatening episodes of vascular hyperpermeability in the presence of a monoclonal gammopathy. Extended intravenous immunoglobulin (IVIG) treatment is associated with fewer recurrences and improved survival, but the optimal treatment dosage and duration remain unknown.
Objective: We aim to evaluate the safety of IVIG tapering and withdrawal in patients with SCLS.
Background: Monoclonal gammopathy-associated systemic capillary-leak syndrome, also known as Clarkson disease, is a rare condition characterized by recurrent life-threatening episodes of capillary hyperpermeability in the context of a monoclonal gammopathy. This study was conducted to better describe the clinical characteristics, natural history, and long-term outcome of monoclonal gammopathy-associated systemic capillary-leak syndrome.
Methods: We conducted a cohort analysis of all patients included in the European Clarkson disease (EurêClark) registry between January 1997 and March 2016.
[Spontaneous intracranial hypotension: an unrecognized diagnosis].
Ann Med Interne (Paris)
March 2002
We report an observation of spontaneous intracranial hypotension occurring in a 32-year-old man. The diagnosis of intracranial hypotension is easy in a suggestive context: after a lumbar puncture or a diversion of CSF. Clinical and CSF cytological features can mimic chronic meningitis leading to false diagnosis and erroneous therapeutic.
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