IgG4 Disease Revealed by a Type 1 Autoimmune Pancreatitis: A Case Report

Bagny Aklesso

Department of Gastroenterology and Hepatology, University Teaching Hospital, «Campus» of Lome, Togo.

Lawson-Ananissoh Laté Mawuli

Department of Gastroenterology and Hepatology, University Teaching Hospital, «Campus» of Lome, Togo.

Kogoe Lidawu Roland-Moïse *

Department of Gastroenterology and Hepatology, University Teaching Hospital, «Campus» of Lome, Togo.

Kaaga Yeba Laconi

Department of Internal Medicine, University Teaching Hospital, «Campus» of Lome, Togo.

Redah Venceslas Debehoma

Department of Gastroenterology and Hepatology, University Teaching Hospital, «Campus» of Lome, Togo.

Kanake Yendoukoa Yves

Department of Gastroenterology and Hepatology, University Teaching Hospital, «Campus» of Lome, Togo.

*Author to whom correspondence should be addressed.


Abstract

Aim:  To describe clinical, paraclinical and evolutive features of an IgG4 disease.

Presentation of Case: The patient was a male, 51 years old, seen in consultation in the gastro-enterology and hepatology department of University teaching hospital “Campus” of Lome for epigastric pain of sudden onset six days before admission, with transfixing irradiation, evolving intermittently in a febrile context. On admission, the patient has a subicterus. The evolution during hospitalization was marked by the sudden onset three days later of bilateral inflammatory polyarthritis of the knees and ankles. Biological examination revealed diabetes mellitus; lipasemia was elevated to 122U/l. There was a disturbance of the renal balance; and a significant increase of plasma IgG4 at 2.380 g/l. The abdominal ultrasound showed a globally hypertrophic pancreatic gland, of heterogeneous structure, without focal lesions or necrosis; without biliary lithiasis. An abdominal CT scan showed, after injection of contrast medium, a global hypertrophy of the pancreas with a corporal-caudal predominance, giving a classic "sausage" appearance; associated with a lack of enhancement of the pancreatic parenchyma.

Conclusion: Type 1 autoimmune pancreatitis is the pancreatic manifestation of IgG4 disease. It is a rare pathology of recent discovery, with a predominance in elderly and male subjects. The diagnosis must be evoked in front of a pancreatic pain associated with articular manifestations, or in case of absence of obvious etiology of a pancreatitis. This diagnosis is defined by recommandations of International Association of Pancreatology. The treatment is based on the use of corticosteroids unlike autoimmune pancreatitis type 2 which is self-limiting with a low risk of relapse and complications.

Keywords: Pancreas, pancreatitis, autoimmune, IgG4


How to Cite

Aklesso, B., Mawuli, L.-A. L., Roland-Moïse, K. L., Laconi, K. Y., Debehoma , R. V., & Yves, K. Y. (2023). IgG4 Disease Revealed by a Type 1 Autoimmune Pancreatitis: A Case Report. Asian Journal of Research and Reports in Gastroenterology, 6(1), 11–15. Retrieved from https://journalajrrga.com/index.php/AJRRGA/article/view/92

Downloads

Download data is not yet available.

References

Kamisawa T. IgG4-positive plasma cells specially infiltrate various organs in autoimmune pancreatitis. Pancreas. 2004;29:167–168.

Deshpande V, Chicano S, Finkelberg D, Selig M, Mino-Kenudson M, Brugge W. autoimmune pancreatitis: a systemic immune complex mediated disease. Am J Surg Pathol. 2006;30:1537–1545.

Stone JH, Khosroshahi A, Deshpande V, Stone J. IgG4-related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases. Arthritis Care Res. 2010;62:316–322.

Vonlaufen A, Frossard J-L. Pancréatite autoimmune. Rev Med Suisse. 2010;6:1662–6.

Kamisawa T, Notohara K, Shimosegawa T. Two clinocopathologic subtypes of autoimmune pancreatitis:LPSP and IDCP. 2010;139:22–5.

Zamboni G, Luttges J, Capelli P. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: A study on 53 resection specimens and 9 biopsy specimens. Virchows Archs. 2004;445:552–63.

Pearson RK, Longnecker DS, Chari ST. Controversies in clinical pancreatology: Autoimmune pancreatitis: Does it exist? Pancreas. 2003;2:1–13.

Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011;40:352–358.

Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, et al. Diagnosis of autoimmune pancreatitis: The Mayo Clinic experience. Clin Gastroenterol Hepatol. 2006;4:1010–16.

Takayama M, Hamano H, Ochi Y. Recurrent attacks of autoimmune pancreatitis result in pancreatic stone formation. Am J Gastroenterol. 2004; 99:932–7.

Okazaki K, Uchida K, Ohana M, Nakase H, Uose S, Inai M. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterol. 2000; 118:573–581.

Pickart T, Mayerle J, Lerch M. Autoimmune pancreatitis. Nat Clin Pr Gastroenterol Hepatol. 2007;4:14–23.

Nishimori I, Tamakoshi A, Kawa S. Influence of steroid therapy on the course of diabetes mellitus in patients with autoimmune pancreatitis: Findings from a nation wide survey in Japan. Pancreas. 2006;32:244–8.

Oseini A, Chaiteerakij R, Shire A, Ghazale A, Kaiya J, Moser C, et al. Utility of serum Immunoglobulin G4 in distinguishing Immunoglobulin G4-associated cholangitis from cholangiocarcinoma. Hepatology. 2011;54: 940–948.

Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.

Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol. 2006;41:626–31.