Case Report: Is autoimmun hepatitis-primer biliary cholangitis overlap syndrome and toxic hepatitis present together?

Abstract

Objective: Overlap syndrome is described as one patient having two or more autoimmune liver diseases. The primary phenotype is autoimmune hepatitis (AIH) and secondarily has the features of cholestatic liver disease. When the patient with AIH has anti mitochondrial antibody (AMA) positivity and the histological features of bile duct damage, it’s diagnosed as Autoimmune Hepatitis Primary Biliary Cholangitis Overlap Syndrome (AIH-PBC Overlap). The prevelance of overlap syndrome is 2% in patients first diagnosed as AIH, and 19% when first diagnosed as PBC. Case Report: In our case; we presented a patient whom consulted to us with itching, jaundice and elevated liver function tests and while searching the ethiology, got interrupted with consumption of Amaranthus Retroflexus ( eng: Pigweed , tr: Hoşkıran otu) and got worsened symptoms but eventually diagnosed as Overlap syndrome. Conclusion: In most cases, a single cause is identified for acute liver dysfunction in a patient; however, this case aims to remind readers that in patients with impaired liver function, an overlap syndrome may coexist with a toxic agent

Keywords

• Autoimmune hepatitis
• primary biliary cholangitis
• overlap syndrome

Olgu Sunumu: Otoimmün hepatit primer biliyer kolanjit örtüşme sendromu ve toksik hepatit bir arada mı?

Abstract

Amaç: Bir hastada iki veya daha fazla otoimmün karaciğer hastalığının bulunduğu durumlara örtüşme sendromu denir. Asıl fenotipi Otoimmün Hepatittir (OİH) ve ikincil olarak kolestatik karaciğer hastalığı özelliklerine sahiptirler. OİH tanılı hastalarda anti mitokondriyal antikor (AMA) pozitiflik ve safra yolu hasarının histolojik özellikleri varsa OİH ve Primer Biliyer Kolanjit (PBK) örtüşme sendromu tanısı alırlar. Bu örtüşme sendromunun görülme sıklığı, başlangıçta OİH olarak tanı alanlarda %2, PBK olarak tanı alanlarda %19’dur. Olgu Sunumu: Olgumuzda; kaşıntı ve sarılık semptomları, karaciğer fonksiyon testlerinde yükseklik ile başvuran etiyoloji araştırması sırasında araya giren Hoşkıran otu alımı ile tablosu şiddetlenen ancak nihayetinde örtüşme sendromu tanısı konulan hasta sunulmaktadır. Sonuç: Sıklıkla bir hastada akut karaciğer fonksiyon bozukluğunun tek bir sebebi bulunur ancak bu vaka ile karaciğer fonksiyonları bozulan hastalarda örtüşme sendromu ile birlikte toksik bir ajan da olabileceği de okuyucuya hatırlatılmak istenmiştir.

Keywords

• Otoimmün hepatit
• primer biliyer kolanjit
• örtüşme sendromu

References

1. 1. Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report: Review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999; 31:929-38.
2. 2. https://pfaf.org/user/Plant.aspx?LatinName=Amaranthus+retroflexus
3. 3. Gonzalez RS, Washington K. Primary Biliary Cholangitis and Autoimmune Hepatitis. Surgical Pathology Clinics 2018; 11(2):329-349.
4. 4. Chazouilleres O, Wendum D, Serfaty L, et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 1998;28:296–301
5. 5. Kuiper EM, Zondervan PE, van Buuren HR. Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol 2010; 8:530-4.
6. 6. Zhang W, De D, Mohammed KA, Munigala S, Chen G, Lai JP, Bacon BR. New scoring classification for primary biliary cholangitis-autoimmune hepatitis overlap syndrome. Hepatol Commun. 2018; 20;2(3):245-253.
7. 7. Chazouillères O, Wendum D, Serfaty L, Rosmorduc O, Poupon R. Long term outcome and response to therapy of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. J Hepatol. 2006 ;44(2):400-6.
8. 8. Fan X, Zhu Y, Men R, Wen M, Shen Y, Lu C, Yang L. Efficacy and Safety of Immunosuppressive Therapy for PBC-AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study. Can J Gastroenterol Hepatol. 2018;2;2018:1965492.