Systematic Reviews
Copyright ©The Author(s) 2020.
World J Clin Cases. Sep 26, 2020; 8(18): 4075-4093
Published online Sep 26, 2020. doi: 10.12998/wjcc.v8.i18.4075
Table 2 Summary of systematically reviewed clinical cases (primary sclerosing cholangitis/autoimmune hepatitis overlap syndrome)
Ref.CountrySexAgeClinical presentationIBDCo-morbiditiesAntibodiesHLATreatmentRelapseOutcomeMiscellaneous
Wurbs et al[3], 1995GermanyF28Fever, Choluria, Weight Loss, FatigueNNonepANCA, SMADRSteroids, AZANRecovery
Lawrence et al[4], 1994United StatesM39Nausea, Emesis, Fatigue, Hepatomegaly, Occult stool bloodUCCirrhosisSMANASteroids, AZA, Cyclosporine ANRecovery
Nalepa et al[5], 2017PolandM10Jaundice, Diarrhea, Abdominal Pain, Hepatomegaly, Splenomegaly, Ascites, HematemesisUCCirrhosis, Esophageal VaricesANA, SMANASteroids, AZA, UDCA, MSMYRecoveryLiver transplantation
Luketic et al[6], 1997United StatesF38Jaundice, Nausea, Fatigue, Ascites, HematemesisNNoneANANASteroids, AZAYRecoveryLiver transplantation
Mueller et al[7], 2018GermanyF15Vomiting, FatigueNNoneANA, pANCA, SMA, AMANASteroids, UDCANRecovery
Guerrero-Hernández et al[8], 2007MexicoF22Jaundice, Choluria, FatigueNNoneANA, pANCANASteroids, AZA, UDCANRecovery
Takiguchi et al[9], 2002JapanF36FeverNNoneANA, pANCAA24, A31, B35, B61, Cw4, DR4Steroids, AZA, UDCAYRecovery
McNair et al[10], 1998United KingdomM38Jaundice, Watery Stools, Abdominal Pain, Weight LossUCEncephalopathyANA, LKM1, pANCA, SMAB8, DR3Steroids, AZAYDeath
McNair et al[10], 1998United KingdomF20Jaundice, ItchingNNoneANA, pANCA, SMANDSteroids, AZA, UDCAYRecovery
McNair et al[10], 1998United KingdomM26Dyspnea, JaundiceNNoneANA, pANCAA1, B8, DR3Steroids, AZAYRecovery
McNair et al[10], 1998United KingdomM14Jaundice, Diarrhea, Abdominal PainUCNoneANA, pANCA, SMAA1, B8, DR3Steroids, AZAYRecovery
McNair et al[10], 1998United KingdomM18Jaundice, Diarrhea, Abdominal Pain, Weight LossNNonepANCA, SMANDSteroids, AZA, UDCANRecovery
Man et al[11], 2017RomaniaM13Jaundice, Hepatomegaly, SplenomegalyNEsophageal VaricesSMANASteroids, AZA, UDCA, Mycophenolate MofetilYRecovery
Malik et al[12], 2010United StatesF22Diarrhea, Abdominal PainCDNoneNANASteroids, AZA, UDCA, Mycophenolate MofetilYRecovery
Lamia et al[13], 2012TunisiaM4Hematuria, Diarrhea, Hepatomegaly, SplenomegalyNSICNoneANA, pANCA, SMANASteroids, AZA, UDCA, 6-MPYRecovery
Lee et al[14], 2005MalaysiaF5Jaundice, Itching, SteatorrheaNNoneANA, SMANASteroids, UDCANRecovery
Santos et al[15], 2012ColombiaM36Jaundice, Hematemesis, Abdominal Pain, Hepatomegaly, AscitesNCirrhosis, Encephalopathy, Esophageal VaricesANANASteroids, AZA, UDCAYRecoveryLiver transplantation
Santos et al[15], 2012ColombiaF35Headache, Jaundice, FatigueUCEsophageal VaricesANA, pANCA, SMANASteroids, UDCA, MSMYRecovery
Santos et al[15], 2012ColombiaF45Jaundice, Choluria, Acholia, HepatomegalyNSICHypothyroidismANA, SMANASteroids, AZA, UDCANRecovery
Saltik-Temizel et al[16], 2004TurkeyM11Jaundice, Itching, Abdominal Pain, Hepatomegaly, Splenomegaly, Fecal Occult BloodUCNonepANCA, SMANASteroids, AZA, UDCA, MSMYRecovery
Gopal et al[17], 1999; Nagral et al[18], 1999IndiaF14Jaundice, Hepatomegaly, Splenomegaly, AscitesNCirrhosis, Esophageal VaricesANANASteroids, D-penicillamineYRecoveryLiver transplantation
Lüth et al[19], 2009GermanyNANANANANANANANANANA
Farid et al[20], 2015BahrainF11Jaundice, Nausea, Vomit, Abdominal PainUCCirrhosisNANASteroids, AZAYDeathLiver transplantation
Floreani et al[21], 2005ItalyF26NANANANANANANANA
Floreani et al[21], 2005ItalyM19NANANANANANANANA
Floreani et al[21], 2005ItalyM32NANANANANANANANA
Floreani et al[21], 2005ItalyM27NANANANANANANANA
Floreani et al[21], 2005ItalyF15NANANANANANANANALiver transplantation
Floreani et al[21], 2005ItalyF15NANANANANANANANA
Floreani et al[21], 2005ItalyF16NANANANANANANANA
Gohlke et al[22], 1996; Zenouzi et al[23], 2014GermanyM19NANEsophageal VaricesANA, pANCA, SMAA1, A32, B8, Cw3, Cw7, DR3, DR4Steroids, AZA, UDCAYRecovery
Gohlke et al[22], 1996; Zenouzi et al[23], 2014GermanyM28NAUCEsophageal VaricesANA, pANCAA1, A32, B7, B8, Cw7, DR3, DR4, DR52, DR53, DQ2, DQ3Steroids, AZA, UDCAYRecovery
Gohlke et al[22], 1996; Zenouzi et al[23], 2014GermanyM18NANCirrhosis, Esophageal VaricesANA, pANCA, SMAA1, A25, B8, DR3Steroids, AZA, UDCAYRecoveryLiver transplantation
Abdo et al[24], 2002CanadaM15Jaundice, FatigueUCNoneANA, SMANASteroids, AZAYRecovery
Abdo et al[24], 2002CanadaM51Abdominal Pain, Weight Loss, FatigueNNoneANA, SMANASteroids, AZA, UDCAYRecovery
Abdo et al[24], 2002CanadaM54Abdominal Pain, Fatigue, SplenomegalyUCCirrhosis, Alcohol-induced PancreatitisNANASteroids, AZA, UDCA, MSMYRecovery
Abdo et al[24], 2002CanadaF25Jaundice, Itching, Abdominal Pain, Fatigue, HepatomegalyNNoneANA, SMANASteroids, AZA, UDCAYRecovery
Abdo et al[24], 2002CanadaF23FatigueUCNoneANA, SMANASteroids, AZA, UDCA, MSMYRecovery
Abdo et al[24], 2002CanadaM20Jaundice, Abdominal Pain, Weight Loss, Hepatomegaly, SplenomegalyNCirrhosisANA, SMANASteroids, AZA, UDCAYRecoveryLiver transplantation
van Buuren et al[25], 2000NetherlandsM7NAUCNoneANA, SMANASteroids, AZANARecovery
van Buuren et al[25], 2000NetherlandsM14NACDNoneANA, SMANASteroids, AZANARecovery
van Buuren et al[25], 2000NetherlandsF21NAUCNoneANA, pANCANASteroids, AZANARecovery
van Buuren et al[25], 2000NetherlandsF22NACDNoneANA, SMANASteroids, AZAYRecoveryLiver transplantation
van Buuren et al[25], 2000NetherlandsM20NAUCNoneSMANASteroids, AZA, UDCANARecovery
van Buuren et al[25], 2000NetherlandsM23NAUCCirrhosis, Esophageal VaricesANA, pANCANASteroids, AZA, UDCANARecovery
van Buuren et al[25], 2000NetherlandsM37NANNoneANANASteroids, AZA, UDCANARecovery
van Buuren et al[25], 2000NetherlandsM54NACDNoneANA, SMANASteroids, AZA, UDCAYRecoveryLiver transplantation
van Buuren et al[25], 2000NetherlandsF44JaundiceUCHepatic InsufficiencypANCANASteroids, AZA, UDCAYRecoveryLiver transplantation
Li et al[26], 2017ChinaM52Jaundice, ItchingNRheumatoid ArthritisNANASteroidsNRecovery
Gharibpoor et al[27], 2017IranM26Jaundice, Itching, Choluria, Acholia, Abdominal Pain, Weight Loss, HepatomegalyNNoneANA, SMANASteroids, AZA, UDCANRecovery
Sander et al[28], 2007GermanyM24CDNoneANA, SMAB8, DR4Steroids, AZA, UDCAYRecovery
Smolka et al[29], 2016Czech RepublicM16JaundiceNNoneANA, pANCANANANANA
Smolka et al[29], 2016Czech RepublicM17Diarrhea, Abdominal PainUCNonepANCANANANANA
Smolka et al[29], 2016Czech RepublicF15FatigueNNonepANCANANANANA
Smolka et al[29], 2016Czech RepublicM14UCNoneNA, pANCA, SMANANANANA
Smolka et al[29], 2016Czech RepublicF16CDNonepANCANANANANA
Smolka et al[29], 2016Czech RepublicF10Fever, Weight Loss, FatigueNSICNoneLKM1, pANCANANANANA
Smolka et al[29], 2016Czech RepublicM12Abdominal PainUCNonepANCANANANANA
Smolka et al[29], 2016Czech RepublicF9Melena, FatigueUCNoneANA, pANCANANANANA
Smolka et al[29], 2016Czech RepublicM3Diarrhea, Abdominal PainUCNoneANA, pANCANANANANA
Smolka et al[29], 2016Czech RepublicF9NNoneANA, pANCA, SMANANANANA
Smolka et al[29], 2016Czech RepublicF15Itching, Diarrhea, Abdominal PainCDNoneANA, pANCANANANANA
Griga et al[30], 2000United KingdomF24DiarrheaCDNoneANA, pANCANASteroids, MSM, UDCANRecovery
Griga et al[30], 2000United KingdomM28Jaundice, ItchingNNoneANA, pANCAB8, DR4Steroids, UDCANRecovery
Warling et al[31], 2014BelgiumM29Jaundice, FatigueUCMembranous GlomerulonephritispANCADR3Steroids, AZA, UDCA, MSM, 6-MPYRecovery
Hyslop et al[32], 2010United StatesNA40NAUCNoneANANANANANA
Hyslop et al[32], 2010United StatesNA24NAUCNoneANANANANANA
Hyslop et al[32], 2010United StatesNA53NACDCirrhosisANANANANANA
Hyslop et al[32], 2010United StatesNA37NAUCNoneANANANANANA
Hyslop et al[32], 2010United StatesNA32NAUCCirrhosisANANANANANA
Hyslop et al[32], 2010United StatesNA61NACDCirrhosisANANANANANA
Hyslop et al[32], 2010United StatesNA52NACDNoneANANANANANA
Hyslop et al[32], 2010United StatesNA26NACDCirrhosisANANANANANA
Hyslop et al[32], 2010United StatesNA33NAUCNoneANANANANANA
Hyslop et al[32], 2010United StatesNA44NAUCCirrhosisANANANANANA
Fukuda et al[33], 2012JapanM72AscitesNCirrhosis, Hepatocellular CarcinomaANA, AMADRB1*0405, DRB1*0901Steroids, UDCAYDeath
Hatzis et al[34], 2001GreeceF46Fever, Arthralgia, Fatigue, SplenomegalyNNephrectomy for Reflux NephropathyANA, pANCA, SMAA3, A11, B16, B35, Cw4, DR13, DR14, DR52, DQ6Steroids, AZA, UDCA, AntibioticsNRecovery
Thakker et al[35], 2010IndiaF9Fever, Jaundice, Itching, Arthralgia, Fatigue, Hepatomegaly, SplenomegalyNNoneANANASteroids, UDCANRecovery
Koskinas et al[36], 1999GreeceM18Fever, Jaundice, Hematochezia, Fatigue, Hepatomegaly, Splenomegaly, AscitesUCPyoderma GangrenosumNAA2, A32, B7, B21, B49, Bw4, Bw6, DR6, DR10, DR13Steroids, AZA, UDCA, MSM, AntibioticsYRecovery
Lucas et al[37], 2007United StatesM18Fecal incontinence, Abdominal PainUCNANANANANANA
Protzer et al[38], 1996SwitzerlandM22Jaundice, Nausea, Diarrhea, Abdominal Pain, FatigueUCPIGCHSMAA1, A2, B8, B44, Cw5, Cw7, DR3, DR52, DQ2Steroids, UDCAYRecovery
Protzer et al[38], 1996SwitzerlandF32NPIGCHpANCAA2, A28, B55, B67, Cw3, DR4, DR11, DQ2, DQ3Steroids, AZAYRecovery
Protzer et al[38], 1996SwitzerlandM28NCirrhosis, PIGCHANAA1, B8, DR3Steroids, AZAYDeath
Protzer et al[38], 1996SwitzerlandM26NPIGCHANAA1, B8, DR3Steroids, UDCAYRecovery
Hong-Curtis et al[39], 2004United StatesF34Jaundice, Itching, FatigueUCAnemiaANANASteroids, UDCA, AntibioticsYRecovery
Simão et al[40], 2012PortugalM15ItchingNNoneANA, AMANASteroids, AZA, UDCAYRecovery
Larsen et al[41], 2012DenmarkM10Vomiting, Diarrhea, Abdominal Pain, Weight LossCDNonepANCA, SMANASteroids, AZA, UDCANRecovery
Guerra et al[42], 2016PeruF22Jaundice, Choluria, Fatigue, Splenomegaly, AscitesNCirrhosis, Esophageal VaricesANAA2, A11, B35, B60, DR9, DR13Steroids, UDCAYRecoveryLiver transplantation
Ng et al[43], 2011AustraliaF33NANANANANAUDCANANA
Igarashi et al[44], 2017JapanF19NNoneANANASteroids, UDCAYRecovery
Igarashi et al[44], 2017JapanM61NRenal Cell CarcinomaNANASteroids, UDCAYRecovery
Gargouri et al[45], 2013TunisiaM10Jaundice, Abdominal Pain, Fatigue, Hepatomegaly, SplenomegalyNEsophageal VaricespANCANASteroids, AZA, UDCANRecovery
Patrico et al[46], 2013ItalyF7Fever, Acholia, HepatomegalyNNoneLKM1NASteroids, AZYRecovery
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.