Abstract
We present the case of a 56-year-old woman diagnosed with primary biliary cholangitis (PBC). She has continuously taken 600 mg/day of ursodeoxycholic acid. Edema of the lower limbs manifested on July 20, 20XX; after 2 weeks, she manifested rapid weight gain and nettle rash on the limbs and trunk. She was admitted to our hospital on August 22. She had marked eosinophilia, hypoalbuminemia, anemia, non-pitting lower limbs edema, and nettle rash of the limbs and the trunk. We ruled out other diseases that may have caused the edema and suspected her with episodic angioedema with eosinophilia (EAE). The peripheral blood eosinophil count rapidly decreased after the administration of 30 mg prednisolone. The edema and nettle rash improved on the 7th day of admission, and the hypoalbuminemia and anemia improved on the 14th day. Prednisolone was tapered and discontinued, and there was no relapse of edema. We revised our diagnosis to non-recurrent EAE. She was diagnosed with asymptomatic PBC; therefore, anemia and hypoalbuminemia were considered not PBC but chronic inflammation and decrease in appetite. In this case, elevation of serum IgG4 was observed at onset and at remission. This suggests that IgG4 may be involved in the development of EAE in patients with chronic liver disease.
Similar content being viewed by others
References
Gleich GJ, Schroeter AL, Marcoux JP, et al. Episodic angioedema associated with eosinophilia. N Engl J Med. 1984;310:1621–6.
Carey EJ, Ali AH, Lindor KD. Primary biliary cirrhosis. Lancet. 2015;386:1565–75.
Gershwin ME, Ansari A, Mackay I, et al. Primary biliary cirrhosis: an orchestrated immune response against epithelial cells. Immunol Rev. 2000;174:210–25.
Golding PL, Smith M, Williams R. Multisystem involvement in chronic liver disease: studies on the incidence and pathogenesis. Am J Med. 1973;55:772–82.
Chikama R, Hosokawa M, Miyazawa T, et al. Nonepisodic angioedema associated with eosinophilia: report of 4 cases and review of 33 young female patients reported in Japan. Dermatology. 1998;197:321–5.
Katzen DR, Leiferman KM, Weller PF, et al. Hypereosinophilia and recurrent angioneurotic edema in a 21/2-year-old girl. Am J Dis Child. 1986;140:62–4.
Wolf C, Pehamberger H, Breyer S, et al. Episodic angioedema with eosinophilia. J Am Acad Dermatol. 1989;20:21–7.
Schiavino D, Gentiloni N, Murzilli F, et al. Episodic angioedema with eosinophilia (Gleich syndrome). Allergol Immunopathol. 1990;18:233–6.
Putterman C, Barak V, Caraco Y, et al. Episodic angioedema with eosinophilia: a case associated with T cell activation and cytokine production. Ann Allergy. 1993;70:243–8.
Beuers U, Gershwin ME, Gish RG, et al. Changingnomenclature for PBC: from ‘cirrhosis’ to ‘cholangitis.’ J Hepatol. 2015;63:1285–7.
Beuers U, Gershwin ME, Gish RG, et al. Changingnomenclature for PBC: From ‘cirrhosis’ to ‘cholangitis.’ Hepatology. 2015;62:1620–2.
Scheuer PJ. Primary biliary cirrhosis. Proc R Soc Med. 1967;60:1257–60.
Scheuer PJ. Pathologic features and evolution of primary biliary cirrhosis and primary sclerosing cholangitis. Mayo Clin Proc. 1998;73:179–83.
Gershwin ME, Mackay IR, Sturgess A, et al. Identification and specificity of a cDNA encoding the 70kd mitochondrial antigen recognized in primary biliary cirrhosis. J immunol. 1987;138:3525–31.
Toyoda M, Suzuki D, Uehara G, et al. Two cases of episodic angioedema associated with eosinophilia. Nihon Jinzo Gakkai Shi. 2001;43:44–8.
Stone JH, Zen Y, Deshpande V, et al. IgG4-related disease. N Engl J Med. 2012;366:539–51.
Wallace ZS, Naden RP, Chari S, et al. The 2019 American college of rheumatology/european league against rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020;79:77–87.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
Hideyuki Iwashita, Satoshi Shakado, Hideki Yasuda, Tarou Tanabe, Masashi Yamaguchi, Makoto Irie and Fumihito Hirai declare that they have no conflict of interest.
Human and animal rights
This article does not contain any studies directly involving human participants, as it is a case report with use of data collected from daily medical practice.
Informed consent
This case report has been published with the informed consent of the patient.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Iwashita, H., Shakado, S., Yasuda, H. et al. A case of episodic angioedema with eosinophilia in an elderly woman with primary biliary cholangitis. Clin J Gastroenterol 14, 836–841 (2021). https://doi.org/10.1007/s12328-021-01375-w
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12328-021-01375-w