Table 3.
Baseline fibrosis score in patients with different types of lipodystrophy
| Fibrosis Score | AGL (N=13) (Age 9–68 years) |
APL (N=5) (Age 10– 30 years) |
CGL; AGPAT2 (N=8) (Age 14 47 years) |
CGL; BSCL2 (N=10) (Age 8–18 years) |
FPL; LMNA (N=12) (Age 18–64 years) |
FPL; PPARY (N=2) (Age 32 and 35 years) |
|---|---|---|---|---|---|---|
| 0 (None) | 2 | 0 | 0 | 0 | 4 | 1 |
|
1A (Mild perisinusoidal only) |
0 | 0 | 3 | 0 | 3 | 0 |
|
1B (Moderate perisinuosoidal) |
1 | 1 | 1 | 0 | 0 | 0 |
| 1C (Periportal only) | 1 | 0 | 1 | 0 | 1 | 0 |
|
2 (Periportal and perisinusoidal) |
2 | 2 | 2 | 1 | 2 | 1 |
| 3 (Bridging fibrosis) | 41 | 2 | 12 | 4 | 2 | 0 |
| 4 (Cirrhosis) | 3 | 0 | 0 | 5 | 0 | 0 |
AGL, Acquired Generalized Lipodystrophy; CGL, Congenital Generalized Lipodystrophy; FPL, Familial Partial Lipodystrophy; APL, Acquired Partial Lipodystrophy.
1
Three out 4 patients in the AGL group with bridging fibrosis also had autoimmune hepatitis
2
The patient with AGPAT2 mutation in the CGL group who had bridging fibrosis also had concomitant hepatitis B.