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.