Session Information
Title: Systemic Lupus Erythematosus - Clinical Aspects and Treatment: Treatment and Management Studies
Session Type: Abstract Submissions (ACR)
Background/Purpose: Autoimmune Hepatitis (AIH) is a chronic progressive liver disease of unknown cause, characterized by circulating autoantibodies and hyperglobulinemia. Patients with AIH often have other autoimmune diseases such as autoimmune thyroiditis and ulcerative colitis. However, AIH accompanied by SLE (AIH-SLE overlap) is relatively rare condition. The aims of our study were to identify the distinct features of AIH-SLE overlap compared with primary AIH (PAIH) and to evaluate the factors related with outcome of AIH-SLE overlap.
Methods: From May. 1995 to Feb. 2014, the clinical data of 164 patients with PAIH and 23 patients with AIH-SLE overlap in a tertiary referral center were reviewed retrospectively. AlH was diagnosed if pretreatment or posttreatment score was above 9 or 11, according to AIH diagnostic scoring system of American Association for the Study of Liver Disease in 2002. Liver biopsy was performed in all AIH patients. SLE patients fulfilled at least 4 of the 1997 revised American College of Rheumatology criteria. Progression was defined as occurrence of liver cirrhosis (LC), hepatocellular carcinoma (HCC), liver transplantation (LT) or death from hepatic failure.
Results: The mean follow-up duration of AIH-SLE overlap and PAIH were 7.62 ± 4.13 years (range: 1.5 – 16) and 6.23 ± 4.21 (0.5 -17.5), respectively (Table 1). The age at AIH diagnosis was younger and initial serum IgG level was higher in AIH-SLE overlap (P < 0.005). There were no significant differences of histological findings and treatment strategy. Although proportion of overall progression was not different, severe progression such as HCC, LT or death only happened in PAIH patients. Among 23 patients of AIH-SLE overlap, 8 patients with progression showed higher serum IgG level (4077.38 ± 1641.02 mg/dl) compared to 15 patients without progression (2560.71 ± 932.24) (p=0.017). Furthermore, progression in AIH-SLE overlap was associated with serum IgG level of above 2 folds upper limit of normal (OR = 11.00, 95% CI = 1.420 – 85.201, P = 0.026).
Conclusion: The clinical course of AIH might be expected less aggressively in AIH-SLE overlap than PAIH. In addition, we could suggest that initial high level of serum IgG is a poor prognostic factor in patients with AIH-SLE overlap.
Table 1. Characteristics of AIH-SLE overlap and PAIH
|
Characteristic |
AIH-SLE overlap (n= 23) |
PAIH (n= 164) |
|
Age at AIH diagnosis, years a |
37.35 ± 12.55 |
49.98 ± 12.365 |
|
Sex, female (%) |
23 (100) |
149 (90.9) |
|
Follow up, year |
7.62 ± 4.13 (1.5 – 16) |
6.23 ± 4.21 (0.5 – 17.5) |
|
Other autoimmune disease (%) |
4 (17.4) |
21 (12.8) |
|
Arthritis (%) a |
9 (39.1) |
27 (16.6) |
|
Leukopenia (%) |
3 (13.1) |
25 (15.2) |
|
Thrombocytopenia (%) |
11 (47.8) |
44 (26.8) |
|
Protein, g/dl |
8.40 ± 1.10 |
8.02 ± 4.07 |
|
Albumin, g/dl |
3.42 ± 0.70 |
3.53 ± 0.2 |
|
AST, IU/l |
402.43 ± 444.87 |
432.40 ± 552.15 |
|
ALT, IU/l |
372.70 ± 514.39 |
355.34 ± 468.92 |
|
Alkaline phosphatase, IU/l |
235.39 ± 176.12 |
165.20 ± 116.71 |
|
GGT, IU/l |
168.24 ± 190.10 |
162.44 ± 250.23 |
|
Total bilirubin, mg/dl |
5.99 ± 10.60 |
5.57 ± 6.83 |
|
Serum IgG, mg/dl a |
3112.23 ± 1411.84 |
2419.23 ± 899.64 |
|
Autoantibody anti nuclear antibody (%) anti smooth muscle antibody (%) anti LKM1 (%) anti mitochondrial antibody (%) |
23/23 (100) 8/21 (38.1) 1/17 (4.3) 0/22 (0) |
141/162 (87) 42/161 (26.1) 0/136 (0) 6/160 (3.8) |
|
Biopsy interface hepatitis (%) plasma cell infiltration (%) rosette (%) biliary change (%) |
16 (69.6) 5 (21.7) 2 (8.7) 6 (26.1) |
93 (56.7) 35 (21.3) 3 (1.8) 23 (14) |
|
Progression at AIH diagnosis (%) |
4/23 (17.4) |
37/164 (22.5) |
|
Progression after AIH diagnosis (%) liver cirrhosis hepatocellular carcinoma liver transplatation death from hepatic failure |
4/19 (21.1) 4 (21.1) 0 (0) 0 (0) 0 (0) |
45/127 (35.4) 38 (29.9) 1 (0.8) 3 (2.4) 3 (2.4) |
a: p < 0.05
AST, Aspartate aminotransferase; ALT, Alanine transaminase; GGT, Gamma glutamyltransferse;
anti LKM1, anti Liver-Kidney-Microsomes antibody
Disclosure:
D. H. Lim,
None;
S. H. Bae,
None;
S. M. Ahn,
None;
S. Hong,
None;
Y. G. Kim,
None;
C. K. Lee,
None;
B. Yoo,
None.
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