Successful maintenance therapy with tocilizumab for severe acute liver failure associated with adult-onset still’s disease

Figures & data

Figure 1. Abdominal CT, skin, and pathological findings of liver in our present case. At the time of acute liver failure, abdominal CT revealed the development of ascites (A), and skin erythema appeared around the same time (B). Hematoxylin-eosin (H&E) staining showed mild infiltration of inflammatory cells into lobules (arrow) (original magnification: x100) (C), and silver staining showed fibrous portal expansion (arrow) (original magnification: x100) (D). Immunostaining of CD4 (E), CD8 (F), and CD68 (G) revealed the dominance of CD8 or CD68-positive cell infiltration (original magnification: x200).

Table 1. Laboratory data of the present case on admission (day 1), day 14, and day 28.

	Day1	Day14	Day28
Blood chemistry
TP (g/dL)	5.9	6.0	6.7
Alb (g/dL)	2.9	1.9	3.3
T-Bil (mg/dL)	3.4	7.1	3.0
D-Bil (mg/dL)	2.4	5.1	0.8
AST (U/L)	1720	528	24
ALT (U/L)	1601	486	85
LDH (U/L)	728	799	235
TG (mg/dL)	135	240	205
NH3 (μmol/L)	64	13	17
CRP (mg/dL)	1.014	12.74	0.03
Ferritin (ng/mL)	7156	55968	3734
sIL-2R (U/mL)	1849	3912	1500
Blood count
WBC (/μL)	10100	10000	5900
Neut	9292	8420	4779
Hb (g/dL)	11.7	10.2	11.2
Plt (x10^4/μL)	20.5	21.6	19.0
Coagulation
PT-INR	1.16	1.5	1.00
APTT (sec)	36	35	22.8
Fib (mg/dL)	288	272	152
D-dimer (μg/mL)	3.6	8.8	0.6
Urinalysis
protein	–	–	–
blood	–	–	–
Bil	2+	3+	–
CAST	–	–	–

Alb: albumin, ALT: alanine aminotransferase, APTT: activated partial thromboplastin time, AST: aspartate aminotransferase, Bil: bilirubin, CRP: C-reactive protein, D-Bil: direct bilirubin, Fib: fibrinogen, Hb: hemoglobin, LDH: lactate dehydrogenase, Neut: neutrophil, Plt: platelets, sIL-2R: soluble il-2 receptor, PT-INR: prothrombin international normalized ratio, T-Bil: total bilirubin, TG: triglyceride, TP: total protein, WBC: white blood cells.

Figure 2. Clinical course of a present case. T-Bil, ALT, ferritin, PT-INR, and CRP improved after initiation of high-dose PSL and CyA, and his disease status remained stable without relapse for half a year after we switched CyA to TCZ. Abbreviations: CyA: cyclosporine; mPSL: methylprednisolone; PSL: prednisolone; TCZ: tocilizumab.