Table 3.
Patients/ diagnosis
|
Gender
|
Age at diagnosis (yr)
|
Type of presentation
|
IBD
|
Splenomegaly1
|
IgG > ULN
|
Auto-antibodies
|
Histology
|
Medications
|
|||
Interface hepatitis
|
Fibrosis
|
Cirrhosis
|
Biliopathy3
|
|||||||||
AIH | F | 4.2 | Asymptomatic | IC | Not | Yes | SMA 1:40; p-ANCA | No | Yes | No | Yes | Pred/MMF/UDCA/Mesa |
AIH | F | 10.9 | Asymptomatic | UC | Not | Yes | ANA 1:320; SMA 1:160; p-ANCA positive | Yes | Yes | No | Yes | Pred/UDCA/Mesa |
ASC | F | 16 | Insidious | None | Yes | Not | ANA 1:160; p-ANCA positive | Yes | Yes | Yes | No | Pred/MMF/UDCA |
ASC | M | 4.3 | Asymptomatic | CD | Not | Yes | ANA 1:160; SMA 1:160; p-ANCA +++ | Yes | Yes | No | Yes | Pred/UDCA/Mesa |
ASC | F | 8.6 | Insidious | UC | Not | Not | SMA 1:40; p-ANCA positive | No | No | No | Yes | Pred/AZA/UDCA/Mesa |
ASC | F | 12.1 | Insidious | UC2 | Not | Not | SMA 1:40; p-ANCA positive | No | No | No | Yes | Pred/AZA/UDCA |
ASC | M | 14.1 | Insidious | None | Not | Not | SMA 1:40; p-ANCA positive | Yes | Yes | No | Yes | Pred/AZA/UDCA |
ASC | M | 14.3 | Acute | UC | Not | Yes | ANA 1:320; SMA 1:320 | Yes | No | No | Yes | Pred/UDCA/Mesa |
ASC | F | 13.8 | Asymptomatic | UC | Yes | Yes | ANA 1:640; p-ANCA positive | No | Yes | Yes | Yes | Pred/MMF/UDCA/Mesa |
ASC | F | 5.1 | Acute | IC | Not | Not | ANA 1:160; p-ANCA positive | No | Yes | No | Yes | Pred/MMF/UDCA/Mesa |
ASC | F | 13.1 | Acute | CD | Yes | Yes | ANA 1:80; p-ANCA positive | No | Yes | No | Yes | Pred/MMF/UDCA/Mesa |
ASC | F | 3.6 | Asymptomatic | UC | Yes | Not | ANA 1:160; SMA 1:80; p-ANCA positive | Yes | Yes | No | Yes | Pred/MMF/UDCA/Mesa |
Spleen size detected on liver scan o magnetic resonance cholangiopancreatography.
Patient underwent colectomy at age of 14 years.
It includes at least one of the following: Inflammatory injury of the bile duct, ductular reaction, periductular fibrosis, biliary metaplasia, granulomatous cholangitis[16].
AIH: Autoimmune hepatitis; ASC: Autoimmune sclerosing cholangitis; IBD: Inflammatory bowel disease; UC: Ulcerative colitis, CD: Crohn disease; IC: Indeterminate colitis; ULN: Upper limit of normal; F: Female; M: Male; ANA: Anti-nuclear antibody; SMA: Smooth muscle antibody; ANCA: Anti-neutrophil cytoplasmic antibodies; Pred: Prednisone; UDCA: Ursodeoxycholic acid; MMF: Mycophenolate mofetil; Mesa: Mesalazyne.