Primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome associated with inflammatory bowel disease: A case report and systematic review

Table 1 Baseline features in 109 patients with overlap syndrome (primary sclerosing cholangitis/autoimmune hepatitis)

Variable	Patients (n = 109)
Mean age (yr)	25.52
Sex (male)	46 (42.59%)
Race	10 (6.78%)
White	7 (70%)
Black	3 (30%)
IBD	48 (44.44%)
CD	13 (27.65%)
UC	32 (68.08%)
Non-specific	3 (6.38%)
PSC
Small Ducts	4 (3.70%)
AIH (median)
SAH pre-treatment (pts)	17 (13-22)
SAH post-treatment (pts)	19 (13-25)
Clinical presentation
Fever	7 (6.48%)
Dyspnea	1 (0.93%)
Headache	1 (0.93%)
Jaundice	31 (28.70%)
Pruritus	11 (10.19%)
Urine alteration	6 (5.55%)
Choluria	5 (83.33%)
Hematuria	1 (16.66%)
Nausea	4 (3.70%)
Emesis	8 (7.40%)
Without blood	4 (50%)
Hematemesis	4 (50%)
Diarrhea	11 (10.19%)
Stools	18 (16.66%)
Hematochezia	1 (5.55%)
Melena	1 (5.55%)
Incontinence	1 (5.55%)
Acholia	3 (16.66%)
Watery stools	11 (61.11%)
Steatorrhea	1 (5.55%)
Abdominal pain	21 (19.44%)
Joint pain	2 (1.85%)
Weight loss	9 (8.33%)
Fatigue	22 (20.37%)
Family history	4 (3.70%)
Hepatomegaly	15 (13.89%)
Splenomegaly	12 (11.11%)
Ascites	7 (6.48%)
Fecal occult blood	3 (2.78%)
Cirrhosis	17 (15.74%)
Encephalopathy	2 (1.85%)
Comorbidities
Esophageal varices	13 (12.03%)
Hypothyroidism	1 (0.93%)
Anemia	1 (0.93%)
Alcohol-induced pancreatitis	1 (0.93%)
Hepatic insufficiency	1 (0.93%)
Rheumatoid arthritis	1 (0.93%)
Smoker	1 (0.93%)
Membranous glomerulonephritis	1 (0.93%)
Hepatocarcinoma	1 (0.93%)
Pyoderma gangrenosum	1 (0.93%)
Reflux nephropathy	1 (0.93%)
Post-infantile giant cell hepatitis	4 (3.70%)
Renal cell carcinoma	1 (0.93%)
Autoimmune thyroiditis	1 (0.93%)
Biopsy	109 (100%)
Grade (Batts-Ludwig)	29 (26.85%)
None	2 (6.89%)
Minimal	1 (3.44%)
Mild	10 (34.48%)
Moderate	11 (37.93%)
Severe	5 (17.24%)
Stage (Batts-Ludwig)	37 (34.25%)
None	1 (2.70%)
Portal fibrosis	14 (37.83%)
Periportal fibrosis	10 (27.02%)
Septal fibrosis	9 (24.32%)
Cirrhosis	3 (8.10%)
Laboratory tests (mean)
Hb (g/dL)	10.20
Ht (%)	32.8
Leucocytes (mm3) (median)	7600
Platelets (mm3) (median)	185000
Prothrombin time (s)	15.35
INR	1.41
ALT (U/L)	378.2
AST (U/L)	378.2
GGT (U/L)	316.6
ALP (U/L)	693.4
Total bilirubin (mg/dL)	5.14
Direct bilirubin (mg/dL)	4.43
Total protein (g/dL)	17.82
Albumin (g/dL) (median)	3.09
Total globulins (mg/L)	51410
IgG total (mg/dL)	2762
IgA total (mg/dL)	230.3
IgM total (mg/dL)	729.7
Antibodies
LKM1	3 (2.78%)
AMA	3 (2.78%)
ANA	59 (54.63%)
SMA	33 (30.56%)
pANCA	36 (33.33%)
HLA	18 (16.66%)
Medications	63 (58.33%)
Steroids	62 (98.41%)
Azathioprine	48 (76.19%)
6-mercaptopurine	1 (1.58%)
Ursodeoxycholic acid	47 (74.60%)
Mesalazine	7 (11.11%)
Antibiotics	3 (4.76%)
D-penicillamine	1 (1.58%)
Cyclosporine A	1 (1.58%)
Mycophenolate mofetil	2 (3.17%)
Clinical improvement	61 (56.48%)
Relapse	41 (37.96%)
Transplantation	13 (12.87%)
Mean time from diagnosis-transplant (mo), n = 10 (76.92%)	74.90
Transplant medications, n = 4	4 (30.76%)
Steroids	4 (100%)
Basiliximab	1 (25%)
Cyclosporine	2 (50%)
Azathioprine	1 (25%)
Tacrolimus	2 (50%)
Mycophenolate mofetil	2 (50%)
Mean time follow-up (mo)	59.18
Death	4 (3.70%)

IBD: Inflammatory bowel disease; CD: Crohn’s disease; UC: Ulcerative colitis; PSC: Primary sclerosing cholangitis; AIH: Autoimmune hepatitis; SAH: Score for autoimmune hepatitis; INR: International normalized ratio; ALT: Alanine transaminase; AST: Aspartate transaminase; GGT: Gamma-glutamyl transferase; ALP: Alkaline phosphatase; LKM1: Liver kidney microsome type 1 antibody; AMA: Anti-mitochondrial antibodies; ANA: Antinuclear antibody; SMA: Smooth muscle antibodies; pANCA: Perinuclear anti-neutrophil cytoplasmic antibodies.