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Coincidence of hereditary angioedema (HAE) with Crohn's disease
Authors:Farkas H  Gyeney L  Nemesánszky E  Káldi G  Kukán F  Masszi I  Soós J  Bély M  Farkas E  Füst G  Varga L
Affiliation: a Department of Oto-Rhino-Laryngology, National Institute of Rheumatology and Physiotherapy, Budapest, Hungaryb Department of Internal Medicine and Gastroenterology, National Institute of Rheumatology and Physiotherapy, Budapest, Hungaryc Department of Surgery, National Institute of Rheumatology and Physiotherapy, Budapest, Hungaryd Department of Radiology, National Institute of Rheumatology and Physiotherapy, Budapest, Hungarye Department of Pathology, National Institute of Rheumatology and Physiotherapy, Budapest, Hungaryf Immunpathology Laboratory, National Institute of Haematology and Immunology, Budapest, Hungaryg 3rd Department of Medicine, Semmelweis Medical University, Budapest, Hungary
Abstract:A patient with two diseases, based presumably on different immunopathological mechanisms, hereditary angioedema (HAE) and Crohn's disease, was followed for 8 years. For more than three years of this observation period, detailed laboratory data were also available and could be analyzed. Both diseases had severe courses requiring chronic treatment with danazol and sulfasalazine, respectively. During exacerbation of Crohn's disease, the levels of C4 was found to be significantly lower than during the periods free of symptoms of both diseases. This drop was probably due to an impaired C1-inhibitor activity. HAE attacks and acute exacerbation of Crohn's disease never occurred simultaneously. This finding may be a mere chance but may also indicate that the different immunopathological processes underlying HAE and Crohn's disease influence each other.
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