Programmed cell death-1 inhibitor-related sclerosing cholangitis: A systematic review

Table 1 Demographic characteristics of patients with programmed cell death-1 inhibitor-related sclerosing cholangitis

Patient characteristics	Value
Age, median (range, yr)	67.0 (43–89)
Sex, male/female	21/10
Primary disease
NSCLC	20
Melanoma	4
GC	3
BC	2
SCLC	1
Epithelioid mesothelioma	1
Drugs
Nivolumab	19
Pembrolizumab	10
Avelumab	1
Durvalumab	1
Atezolizumab	0
Treatment cycles until onset	5.5 (1–27)
Symptoms
Abdominal pain or discomfort	11
Fever	6
Jaundice	4
Vomiting	2
Appetite loss	2
Diarrhea or soft stool	2
Skin disorder	2
General fatigue	1
Backache	1
None (liver dysfunction)	8
Liver functional test
T-Bil, median (range, mg/dL)	0.75 (0.3–15.9)
AST, median (range, U/L)	129.0 (49–961)
ALT, median (range, U/L)	125.0 (31–1536)
ALP, median (range, U/L)	1543.0 (237–5066)
GGT, median (range, U/L)	452.0 (114–2094)
Serological test
IgG, median (range, U/L)	1230.0 (1050-1789)
IgA, median (range, U/L)	297.5 (199-474.4)
IgM, median (range, U/L)	64.0 (38-94)
IgG4, ≥ 135 U/L / < 135 U/L	1/12
Antinuclear antibody, ≥ 40 / < 40	7/12
Imaging findings
Biliary stenosis	8
Intrahepatic bile duct	3
Extrahepatic bile duct	1
Multiple	4
Absence	15
Biliary dilation
Presence/Absence	20 / 6
Hypertrophy of the biliary tract	20
Diffuse	19
Gallbladder	1
Absence	1
Pathological findings
Liver	15
Inflammation	15
Biliary or peribiliary tract	14
-CD 8+ T cells dominant	8
Lobular hepatitis	2
Bile duct	8
Inflammation	8
-CD 8+ T cells dominant	2
Gallbladder	2
Inflammation	2
-CD 8+ T cells dominant	1
Therapy
Corticosteroid	26
UDCA	13
MMF	6
Tacrolimus	1
Bezafibrate	1
Response to steroid therapy
Good	3
Moderate	15
Poor	8

NSCLC: Non-small cell lung cancer; SCLC: Small cell lung cancer; GC: Gastric cancer; BC: Bladder cancer; MCC: Merkel cell carcinoma; RPC: Renal pelvis cancer; T-Bil: Total bilirubin; AST: Aspartate aminotransferase; ALT: Alanine aminotransferase; ALP: Alkaline phosphatase; GGT: Gamma-glutamyl transpeptidase; IgG: Immunoglobulin G; IgA: Immunoglobulin A; IgM: Immunoglobulin M; IgG4: Immunoglobulin G4; Mpsl: Methylprednisolone; PSL: Prednisolone; UDCA: Ursodeoxycholic acid; MMF: Mycophenolate mofetil.