Volume 34, Issue 2, November 2021, Pages 141–144
Rokhsi Soukaina1, Tarik Adioui2, Benayad Aourarh3, Abdelfettah Touibi4, Hasna Igorman5, Sanaa Berrag6, Mouna Tamzaourte7, and Aziz Aourarh8
1 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
2 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
3 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
4 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
5 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
6 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
7 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
8 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
Original language: English
Copyright © 2021 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Objective: To report an uncommon presentation of a rare case of autoimmune polyglandular syndrome type IIIb in a patient presenting as well with Small Duct Primary Sclerosing Cholangitis. Clinical Presentation and Intervention: A 42-year-old man presented with jaundice and intermittent fever. Blood tests showed macrocytic anaemia due to vitamin B 12 deficiency compatible with Biermer’s disease. A thyroid function test was consistent with hyperthyroidism compatible with Basedow’s disease. And Liver biopsy revealed signs compatible with Small Duct Sclerosing Cholangitis. A final diagnosis of Small Duct Sclerosing Cholangitis with Biermer’s disease and Basedow’s disease, which constituted autoimmune polyglandular syndrome type IIIb, was made and the patient was treated with L-thyroxine, vitamin B 12 injection and Ursodeoxycholic acid with a impressive improvement during his follow up. Conclusion: This case showed a rare combination between APS type IIIb and Small Duct Sclerosing Cholangitis and that the presence of one autoimmune endocrine disease should prompt clinicians to look for other coexisting autoimmune diseases which may be asymptomatic.
Author Keywords: Small-duct, Sclerosing Cholangitis, type IIIb, Autoimmune, Polyglandular, Syndrome.
Rokhsi Soukaina1, Tarik Adioui2, Benayad Aourarh3, Abdelfettah Touibi4, Hasna Igorman5, Sanaa Berrag6, Mouna Tamzaourte7, and Aziz Aourarh8
1 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
2 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
3 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
4 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
5 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
6 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
7 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
8 Department of Gastroenterology, Military Hospital Mohammed V, Rabat, Morocco
Original language: English
Copyright © 2021 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Objective: To report an uncommon presentation of a rare case of autoimmune polyglandular syndrome type IIIb in a patient presenting as well with Small Duct Primary Sclerosing Cholangitis. Clinical Presentation and Intervention: A 42-year-old man presented with jaundice and intermittent fever. Blood tests showed macrocytic anaemia due to vitamin B 12 deficiency compatible with Biermer’s disease. A thyroid function test was consistent with hyperthyroidism compatible with Basedow’s disease. And Liver biopsy revealed signs compatible with Small Duct Sclerosing Cholangitis. A final diagnosis of Small Duct Sclerosing Cholangitis with Biermer’s disease and Basedow’s disease, which constituted autoimmune polyglandular syndrome type IIIb, was made and the patient was treated with L-thyroxine, vitamin B 12 injection and Ursodeoxycholic acid with a impressive improvement during his follow up. Conclusion: This case showed a rare combination between APS type IIIb and Small Duct Sclerosing Cholangitis and that the presence of one autoimmune endocrine disease should prompt clinicians to look for other coexisting autoimmune diseases which may be asymptomatic.
Author Keywords: Small-duct, Sclerosing Cholangitis, type IIIb, Autoimmune, Polyglandular, Syndrome.
How to Cite this Article
Rokhsi Soukaina, Tarik Adioui, Benayad Aourarh, Abdelfettah Touibi, Hasna Igorman, Sanaa Berrag, Mouna Tamzaourte, and Aziz Aourarh, “Small-duct Primary Sclerosing Cholangitis associated with type IIIb Autoimmune Polyglandular Syndrome: A rare combination,” International Journal of Innovation and Applied Studies, vol. 34, no. 2, pp. 141–144, November 2021.
