A multi-centre UK-based survey on angioedema secondary to acquired C1 inhibitor deficiency

作者：Hadeil Morsi, Aarnoud Huissoon, Alexandros Grammatikos, Andrew Whyte, Ania Manson, Anjali Ekbote, Anju Sivadasan, Anne Boulton, Archana Herwadkar, Ariharan Anantharachagan, Arthur Price, Cathal Steele, Catherine Stroud, Charu Chopra, Dilani Arnold, Efrem Eren, Elizabeth Cleave, Elizabeth Drewe, Emily Moon, Emily Zinser, Grant Hayman, Hana Alachkar, Harichandana Ghanta, Helen Bourne, Intisar Abdelhakam, John Dempster, Katie Townsend, Kavitha Sooriyakumar, Lorena Lorenzo, Magdalena Dziadzio, Manisha Ahuja, Maria Prasinou, Marina Frleta‐Gilchrist, Michael Zhang, Moira Thomas, Pavaladurai Vijayadurai, Prashantha Vaitla, Ravishankar Sargur, Richard Herriot, Robert Yellon, Sai Murng, Sara Drinkwater, Sarah Denness, Sarah Denman, Shuayb Elkhalifa, Sinisa Savic, Sorena Kiani‐Alikhan, Tanya Coulter, Tariq El‐Shanawany, Tasneem Rahman, Tomaz Garcez, Patrick Yong, Rashmi Jain · 发表于：Clinical & Experimental Immunology · 年份：2025 · DOI：10.1093/cei/uxae121 · 被引用次数：3 · 研究领域：Coagulation, Bradykinin, Polyphosphates, and Angioedema、Complement system in diseases、Hemophilia Treatment and Research

BACKGROUND: Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH) is very rare compared to its prototype, hereditary angioedema. An updated characterization of the AAE-C1-INH cohort in the UK is required to inform management. OBJECTIVES: To describe the disease burden of AAE-C1-INH, long-term prophylaxis (LTP) and the clinical, immunochemical, and treatment profiles of AAE-associated diseases in the UK. METHOD: Retrospective data on 117 AAE-C1-INH patients were collected using a national survey proforma across 25/34 Adult Clinical Immunology and Allergy centres in the UK. Other European cohorts were compared. RESULTS: The median age at AAE-C1-INH diagnosis was 65 years with 3.4% of patients diagnosed below 40 years. The median delay in diagnosis was 1 year. Antifibrinolytics and attenuated androgens showed comparable efficacy to LTP, at 88.9% and 89.5%, respectively. A haematological disorder was identified in 83.8% of AAE-C1-INH patients compared to 3.4% of autoimmune diseases. The predominant haematological disorders were splenic marginal zone lymphoma 34% followed by MGUS 16%. The severity of angioedema did not depend on the associated disease. Anti-C1INH-autoantibodies testing was limited to 23.1%. Rituximab monotherapy was effective in treating 9/9 splenic marginal zone lymphoma and 1/2 MGUS-associated AAE-C1-INH. Rituximab efficacy was independent of anti-C1INH-autoantibodies detection with response in 3/3 seronegative and 4/4 seropositive patients. CONCLUSION...