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. Author manuscript; available in PMC: 2022 Jun 6.
Published in final edited form as: Hepatology. 2021 Mar;73(3):1088–1104. doi: 10.1002/hep.31421

TABLE 4.

Histological Characteristics of Liver Biopsies in patients With APECED and APAH

Patient # Age at Liver Biopsy (Years) Sex Histological Activity Index Score
IF Stage
Periportal Necrosis Lobular Inflamm Portal Inflamm Inflamm (Total) Fibrosis (Total) Piecemeal Necrosis Lobular Inflamm Portal Inflamm Confluent Necrosis Inflamm (Total) Fibrosis (Total)  Plasma Cells  Eosinophils Histological Pattern
1 7.7 F 4 3 3 11 1 4 2 3 2 11 2 Increased Increased  Chronic hepatitis
2 2.0 M 3 4 1 8 0 3 3 2 0 8 0 Increased Not increased  Chronic hepatitis
3 31.9 F 6 4 4 14 3 4 3 3 4 14 3 Increased Increased  Chronic hepatitis
4 15.1 F 3 1 1 5 1 3 1 2 0 6 1 Increased Not increased  Chronic hepatitis
5 8.8 F 0 1 0 1 0 0 1 0 0 1 0 Not increased Not increased  Nonspecific changes
6 3.2 F 1 1 1 3 1 1 1 1 0 3 1 Not increased Not increased  Nonspecific changes
7 5.9 F 6 4 3 13 0 4 4 3 5 16 0 Increased Not increased  Acute hepatitis
8 20.9 F 5 3 3 11 1 3 2 2 4 11 2 Increased Not increased  Chronic hepatitis
9 2.7 F 3 3 1 7 1 3 2 2 0 7 2 Not increased Not increased  Chronic hepatitis
10 4.3 F 5 4 1 10 0 2 3 2 5 12 0 Not increased Not increased  Acute hepatitis
11 9.8 M 4 3 4 11 1 3 2 3 0 8 2 Increased increased  Chronic hepatitis
12* 2.1 M N/A N/A N/A N/A N/A N/A N/A N/A N/A N/A N/A N/A N/A  N/A
13 15.0 F 3 4 1 8 1 3 4 2 3 12 1 Increased Not increased  Chronic hepatitis
14 7.0 M 6 4 3 13 1 3 3 3 4 13 1 Increased Not increased  Chronic hepatitis
15 2.1 M 6 4 3 3 0 4 4 3 5 16 0 Not increased Increased  Chronic hepatitis
16 16.6 F 4 4 3 11 1 4 2 3 2 11 1 Increased Not increased  Chronic hepatitis
17 11.7 F 4 4 3 11 1 4 2 2 3 11 1 Increased Increased  Chronic hepatitis
18* 8.0 F N/A N/A N/A N/A N/A N/A N/A N/A N/A N/A N/A N/A N/A  N/A
*

In 2 of the 18 patients with APAH, we could not obtain the prior liver biopsies for review at NIH.

Abbreviations: F, female; Inflamm, inflammation; M, male; N/A, not available.