Table 2.
Summary of systematically reviewed clinical cases (primary sclerosing cholangitis/autoimmune hepatitis overlap syndrome)
| Ref. | Country | Sex | Age | Clinical presentation | IBD | Co-morbidities | Antibodies | HLA | Treatment | Relapse | Outcome | Miscellaneous |
| Wurbs et al[3], 1995 | Germany | F | 28 | Fever, Choluria, Weight Loss, Fatigue | N | None | pANCA, SMA | DR | Steroids, AZA | N | Recovery | |
| Lawrence et al[4], 1994 | United States | M | 39 | Nausea, Emesis, Fatigue, Hepatomegaly, Occult stool blood | UC | Cirrhosis | SMA | NA | Steroids, AZA, Cyclosporine A | N | Recovery | |
| Nalepa et al[5], 2017 | Poland | M | 10 | Jaundice, Diarrhea, Abdominal Pain, Hepatomegaly, Splenomegaly, Ascites, Hematemesis | UC | Cirrhosis, Esophageal Varices | ANA, SMA | NA | Steroids, AZA, UDCA, MSM | Y | Recovery | Liver transplantation |
| Luketic et al[6], 1997 | United States | F | 38 | Jaundice, Nausea, Fatigue, Ascites, Hematemesis | N | None | ANA | NA | Steroids, AZA | Y | Recovery | Liver transplantation |
| Mueller et al[7], 2018 | Germany | F | 15 | Vomiting, Fatigue | N | None | ANA, pANCA, SMA, AMA | NA | Steroids, UDCA | N | Recovery | |
| Guerrero-Hernández et al[8], 2007 | Mexico | F | 22 | Jaundice, Choluria, Fatigue | N | None | ANA, pANCA | NA | Steroids, AZA, UDCA | N | Recovery | |
| Takiguchi et al[9], 2002 | Japan | F | 36 | Fever | N | None | ANA, pANCA | A24, A31, B35, B61, Cw4, DR4 | Steroids, AZA, UDCA | Y | Recovery | |
| McNair et al[10], 1998 | United Kingdom | M | 38 | Jaundice, Watery Stools, Abdominal Pain, Weight Loss | UC | Encephalopathy | ANA, LKM1, pANCA, SMA | B8, DR3 | Steroids, AZA | Y | Death | |
| McNair et al[10], 1998 | United Kingdom | F | 20 | Jaundice, Itching | N | None | ANA, pANCA, SMA | ND | Steroids, AZA, UDCA | Y | Recovery | |
| McNair et al[10], 1998 | United Kingdom | M | 26 | Dyspnea, Jaundice | N | None | ANA, pANCA | A1, B8, DR3 | Steroids, AZA | Y | Recovery | |
| McNair et al[10], 1998 | United Kingdom | M | 14 | Jaundice, Diarrhea, Abdominal Pain | UC | None | ANA, pANCA, SMA | A1, B8, DR3 | Steroids, AZA | Y | Recovery | |
| McNair et al[10], 1998 | United Kingdom | M | 18 | Jaundice, Diarrhea, Abdominal Pain, Weight Loss | N | None | pANCA, SMA | ND | Steroids, AZA, UDCA | N | Recovery | |
| Man et al[11], 2017 | Romania | M | 13 | Jaundice, Hepatomegaly, Splenomegaly | N | Esophageal Varices | SMA | NA | Steroids, AZA, UDCA, Mycophenolate Mofetil | Y | Recovery | |
| Malik et al[12], 2010 | United States | F | 22 | Diarrhea, Abdominal Pain | CD | None | NA | NA | Steroids, AZA, UDCA, Mycophenolate Mofetil | Y | Recovery | |
| Lamia et al[13], 2012 | Tunisia | M | 4 | Hematuria, Diarrhea, Hepatomegaly, Splenomegaly | NSIC | None | ANA, pANCA, SMA | NA | Steroids, AZA, UDCA, 6-MP | Y | Recovery | |
| Lee et al[14], 2005 | Malaysia | F | 5 | Jaundice, Itching, Steatorrhea | N | None | ANA, SMA | NA | Steroids, UDCA | N | Recovery | |
| Santos et al[15], 2012 | Colombia | M | 36 | Jaundice, Hematemesis, Abdominal Pain, Hepatomegaly, Ascites | N | Cirrhosis, Encephalopathy, Esophageal Varices | ANA | NA | Steroids, AZA, UDCA | Y | Recovery | Liver transplantation |
| Santos et al[15], 2012 | Colombia | F | 35 | Headache, Jaundice, Fatigue | UC | Esophageal Varices | ANA, pANCA, SMA | NA | Steroids, UDCA, MSM | Y | Recovery | |
| Santos et al[15], 2012 | Colombia | F | 45 | Jaundice, Choluria, Acholia, Hepatomegaly | NSIC | Hypothyroidism | ANA, SMA | NA | Steroids, AZA, UDCA | N | Recovery | |
| Saltik-Temizel et al[16], 2004 | Turkey | M | 11 | Jaundice, Itching, Abdominal Pain, Hepatomegaly, Splenomegaly, Fecal Occult Blood | UC | None | pANCA, SMA | NA | Steroids, AZA, UDCA, MSM | Y | Recovery | |
| Gopal et al[17], 1999; Nagral et al[18], 1999 | India | F | 14 | Jaundice, Hepatomegaly, Splenomegaly, Ascites | N | Cirrhosis, Esophageal Varices | ANA | NA | Steroids, D-penicillamine | Y | Recovery | Liver transplantation |
| Lüth et al[19], 2009 | Germany | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA | |
| Farid et al[20], 2015 | Bahrain | F | 11 | Jaundice, Nausea, Vomit, Abdominal Pain | UC | Cirrhosis | NA | NA | Steroids, AZA | Y | Death | Liver transplantation |
| Floreani et al[21], 2005 | Italy | F | 26 | NA | NA | NA | NA | NA | NA | NA | NA | |
| Floreani et al[21], 2005 | Italy | M | 19 | NA | NA | NA | NA | NA | NA | NA | NA | |
| Floreani et al[21], 2005 | Italy | M | 32 | NA | NA | NA | NA | NA | NA | NA | NA | |
| Floreani et al[21], 2005 | Italy | M | 27 | NA | NA | NA | NA | NA | NA | NA | NA | |
| Floreani et al[21], 2005 | Italy | F | 15 | NA | NA | NA | NA | NA | NA | NA | NA | Liver transplantation |
| Floreani et al[21], 2005 | Italy | F | 15 | NA | NA | NA | NA | NA | NA | NA | NA | |
| Floreani et al[21], 2005 | Italy | F | 16 | NA | NA | NA | NA | NA | NA | NA | NA | |
| Gohlke et al[22], 1996; Zenouzi et al[23], 2014 | Germany | M | 19 | NA | N | Esophageal Varices | ANA, pANCA, SMA | A1, A32, B8, Cw3, Cw7, DR3, DR4 | Steroids, AZA, UDCA | Y | Recovery | |
| Gohlke et al[22], 1996; Zenouzi et al[23], 2014 | Germany | M | 28 | NA | UC | Esophageal Varices | ANA, pANCA | A1, A32, B7, B8, Cw7, DR3, DR4, DR52, DR53, DQ2, DQ3 | Steroids, AZA, UDCA | Y | Recovery | |
| Gohlke et al[22], 1996; Zenouzi et al[23], 2014 | Germany | M | 18 | NA | N | Cirrhosis, Esophageal Varices | ANA, pANCA, SMA | A1, A25, B8, DR3 | Steroids, AZA, UDCA | Y | Recovery | Liver transplantation |
| Abdo et al[24], 2002 | Canada | M | 15 | Jaundice, Fatigue | UC | None | ANA, SMA | NA | Steroids, AZA | Y | Recovery | |
| Abdo et al[24], 2002 | Canada | M | 51 | Abdominal Pain, Weight Loss, Fatigue | N | None | ANA, SMA | NA | Steroids, AZA, UDCA | Y | Recovery | |
| Abdo et al[24], 2002 | Canada | M | 54 | Abdominal Pain, Fatigue, Splenomegaly | UC | Cirrhosis, Alcohol-induced Pancreatitis | NA | NA | Steroids, AZA, UDCA, MSM | Y | Recovery | |
| Abdo et al[24], 2002 | Canada | F | 25 | Jaundice, Itching, Abdominal Pain, Fatigue, Hepatomegaly | N | None | ANA, SMA | NA | Steroids, AZA, UDCA | Y | Recovery | |
| Abdo et al[24], 2002 | Canada | F | 23 | Fatigue | UC | None | ANA, SMA | NA | Steroids, AZA, UDCA, MSM | Y | Recovery | |
| Abdo et al[24], 2002 | Canada | M | 20 | Jaundice, Abdominal Pain, Weight Loss, Hepatomegaly, Splenomegaly | N | Cirrhosis | ANA, SMA | NA | Steroids, AZA, UDCA | Y | Recovery | Liver transplantation |
| van Buuren et al[25], 2000 | Netherlands | M | 7 | NA | UC | None | ANA, SMA | NA | Steroids, AZA | NA | Recovery | |
| van Buuren et al[25], 2000 | Netherlands | M | 14 | NA | CD | None | ANA, SMA | NA | Steroids, AZA | NA | Recovery | |
| van Buuren et al[25], 2000 | Netherlands | F | 21 | NA | UC | None | ANA, pANCA | NA | Steroids, AZA | NA | Recovery | |
| van Buuren et al[25], 2000 | Netherlands | F | 22 | NA | CD | None | ANA, SMA | NA | Steroids, AZA | Y | Recovery | Liver transplantation |
| van Buuren et al[25], 2000 | Netherlands | M | 20 | NA | UC | None | SMA | NA | Steroids, AZA, UDCA | NA | Recovery | |
| van Buuren et al[25], 2000 | Netherlands | M | 23 | NA | UC | Cirrhosis, Esophageal Varices | ANA, pANCA | NA | Steroids, AZA, UDCA | NA | Recovery | |
| van Buuren et al[25], 2000 | Netherlands | M | 37 | NA | N | None | ANA | NA | Steroids, AZA, UDCA | NA | Recovery | |
| van Buuren et al[25], 2000 | Netherlands | M | 54 | NA | CD | None | ANA, SMA | NA | Steroids, AZA, UDCA | Y | Recovery | Liver transplantation |
| van Buuren et al[25], 2000 | Netherlands | F | 44 | Jaundice | UC | Hepatic Insufficiency | pANCA | NA | Steroids, AZA, UDCA | Y | Recovery | Liver transplantation |
| Li et al[26], 2017 | China | M | 52 | Jaundice, Itching | N | Rheumatoid Arthritis | NA | NA | Steroids | N | Recovery | |
| Gharibpoor et al[27], 2017 | Iran | M | 26 | Jaundice, Itching, Choluria, Acholia, Abdominal Pain, Weight Loss, Hepatomegaly | N | None | ANA, SMA | NA | Steroids, AZA, UDCA | N | Recovery | |
| Sander et al[28], 2007 | Germany | M | 24 | CD | None | ANA, SMA | B8, DR4 | Steroids, AZA, UDCA | Y | Recovery | ||
| Smolka et al[29], 2016 | Czech Republic | M | 16 | Jaundice | N | None | ANA, pANCA | NA | NA | NA | NA | |
| Smolka et al[29], 2016 | Czech Republic | M | 17 | Diarrhea, Abdominal Pain | UC | None | pANCA | NA | NA | NA | NA | |
| Smolka et al[29], 2016 | Czech Republic | F | 15 | Fatigue | N | None | pANCA | NA | NA | NA | NA | |
| Smolka et al[29], 2016 | Czech Republic | M | 14 | UC | None | NA, pANCA, SMA | NA | NA | NA | NA | ||
| Smolka et al[29], 2016 | Czech Republic | F | 16 | CD | None | pANCA | NA | NA | NA | NA | ||
| Smolka et al[29], 2016 | Czech Republic | F | 10 | Fever, Weight Loss, Fatigue | NSIC | None | LKM1, pANCA | NA | NA | NA | NA | |
| Smolka et al[29], 2016 | Czech Republic | M | 12 | Abdominal Pain | UC | None | pANCA | NA | NA | NA | NA | |
| Smolka et al[29], 2016 | Czech Republic | F | 9 | Melena, Fatigue | UC | None | ANA, pANCA | NA | NA | NA | NA | |
| Smolka et al[29], 2016 | Czech Republic | M | 3 | Diarrhea, Abdominal Pain | UC | None | ANA, pANCA | NA | NA | NA | NA | |
| Smolka et al[29], 2016 | Czech Republic | F | 9 | N | None | ANA, pANCA, SMA | NA | NA | NA | NA | ||
| Smolka et al[29], 2016 | Czech Republic | F | 15 | Itching, Diarrhea, Abdominal Pain | CD | None | ANA, pANCA | NA | NA | NA | NA | |
| Griga et al[30], 2000 | United Kingdom | F | 24 | Diarrhea | CD | None | ANA, pANCA | NA | Steroids, MSM, UDCA | N | Recovery | |
| Griga et al[30], 2000 | United Kingdom | M | 28 | Jaundice, Itching | N | None | ANA, pANCA | B8, DR4 | Steroids, UDCA | N | Recovery | |
| Warling et al[31], 2014 | Belgium | M | 29 | Jaundice, Fatigue | UC | Membranous Glomerulonephritis | pANCA | DR3 | Steroids, AZA, UDCA, MSM, 6-MP | Y | Recovery | |
| Hyslop et al[32], 2010 | United States | NA | 40 | NA | UC | None | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 24 | NA | UC | None | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 53 | NA | CD | Cirrhosis | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 37 | NA | UC | None | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 32 | NA | UC | Cirrhosis | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 61 | NA | CD | Cirrhosis | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 52 | NA | CD | None | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 26 | NA | CD | Cirrhosis | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 33 | NA | UC | None | ANA | NA | NA | NA | NA | |
| Hyslop et al[32], 2010 | United States | NA | 44 | NA | UC | Cirrhosis | ANA | NA | NA | NA | NA | |
| Fukuda et al[33], 2012 | Japan | M | 72 | Ascites | N | Cirrhosis, Hepatocellular Carcinoma | ANA, AMA | DRB1*0405, DRB1*0901 | Steroids, UDCA | Y | Death | |
| Hatzis et al[34], 2001 | Greece | F | 46 | Fever, Arthralgia, Fatigue, Splenomegaly | N | Nephrectomy for Reflux Nephropathy | ANA, pANCA, SMA | A3, A11, B16, B35, Cw4, DR13, DR14, DR52, DQ6 | Steroids, AZA, UDCA, Antibiotics | N | Recovery | |
| Thakker et al[35], 2010 | India | F | 9 | Fever, Jaundice, Itching, Arthralgia, Fatigue, Hepatomegaly, Splenomegaly | N | None | ANA | NA | Steroids, UDCA | N | Recovery | |
| Koskinas et al[36], 1999 | Greece | M | 18 | Fever, Jaundice, Hematochezia, Fatigue, Hepatomegaly, Splenomegaly, Ascites | UC | Pyoderma Gangrenosum | NA | A2, A32, B7, B21, B49, Bw4, Bw6, DR6, DR10, DR13 | Steroids, AZA, UDCA, MSM, Antibiotics | Y | Recovery | |
| Lucas et al[37], 2007 | United States | M | 18 | Fecal incontinence, Abdominal Pain | UC | NA | NA | NA | NA | NA | NA | |
| Protzer et al[38], 1996 | Switzerland | M | 22 | Jaundice, Nausea, Diarrhea, Abdominal Pain, Fatigue | UC | PIGCH | SMA | A1, A2, B8, B44, Cw5, Cw7, DR3, DR52, DQ2 | Steroids, UDCA | Y | Recovery | |
| Protzer et al[38], 1996 | Switzerland | F | 32 | N | PIGCH | pANCA | A2, A28, B55, B67, Cw3, DR4, DR11, DQ2, DQ3 | Steroids, AZA | Y | Recovery | ||
| Protzer et al[38], 1996 | Switzerland | M | 28 | N | Cirrhosis, PIGCH | ANA | A1, B8, DR3 | Steroids, AZA | Y | Death | ||
| Protzer et al[38], 1996 | Switzerland | M | 26 | N | PIGCH | ANA | A1, B8, DR3 | Steroids, UDCA | Y | Recovery | ||
| Hong-Curtis et al[39], 2004 | United States | F | 34 | Jaundice, Itching, Fatigue | UC | Anemia | ANA | NA | Steroids, UDCA, Antibiotics | Y | Recovery | |
| Simão et al[40], 2012 | Portugal | M | 15 | Itching | N | None | ANA, AMA | NA | Steroids, AZA, UDCA | Y | Recovery | |
| Larsen et al[41], 2012 | Denmark | M | 10 | Vomiting, Diarrhea, Abdominal Pain, Weight Loss | CD | None | pANCA, SMA | NA | Steroids, AZA, UDCA | N | Recovery | |
| Guerra et al[42], 2016 | Peru | F | 22 | Jaundice, Choluria, Fatigue, Splenomegaly, Ascites | N | Cirrhosis, Esophageal Varices | ANA | A2, A11, B35, B60, DR9, DR13 | Steroids, UDCA | Y | Recovery | Liver transplantation |
| Ng et al[43], 2011 | Australia | F | 33 | NA | NA | NA | NA | NA | UDCA | NA | NA | |
| Igarashi et al[44], 2017 | Japan | F | 19 | N | None | ANA | NA | Steroids, UDCA | Y | Recovery | ||
| Igarashi et al[44], 2017 | Japan | M | 61 | N | Renal Cell Carcinoma | NA | NA | Steroids, UDCA | Y | Recovery | ||
| Gargouri et al[45], 2013 | Tunisia | M | 10 | Jaundice, Abdominal Pain, Fatigue, Hepatomegaly, Splenomegaly | N | Esophageal Varices | pANCA | NA | Steroids, AZA, UDCA | N | Recovery | |
| Patrico et al[46], 2013 | Italy | F | 7 | Fever, Acholia, Hepatomegaly | N | None | LKM1 | NA | Steroids, AZ | Y | Recovery |
M: Male; F: Female; NA: Not available; ND: Not determined, IBD: Inflammatory bowel disease; CD: Crohn’s Disease, UC: Ulcerative Colitis, NSIC: Non Specific Inflammatory Colitis, OS: Overlap syndrome, PSC: Primary sclerosing cholangitis, AIH: Autoimmune hepatitis, PIGCH: Post-infantile Giant Cell Hepatitis, MSM: Mesalamine, SFZ: Sulfasalazine, UDCA: Ursodeoxycholic Acid, AZA: Azathioprine, 6-MP: 6-Mercaptopurine, IFX: Infliximab, ADM: Adalimumab, LKM1: Liver kidney microsome type 1 antibody, AMA: Anti-mitochondrial antibodies, ANA: Antinuclear antibody, SMA: Smooth muscle antibodies, pANCA: Perinuclear anti-neutrophil cytoplasmic antibodies.