Autoimmune hepatitis in a patient with pulmonary arterial hypertension treated with endothelin receptor antagonists.
A 48-year-old woman was diagnosed with idiopathic pulmonary arterial hypertension (PAH) and administered PAH-specific therapies, including bosentan. Four years after the initiation of treatment with bosentan, liver dysfunction appeared, and ambrisentan was substituted for bosentan. One-and-a half years later, a second episode of liver dysfunction occurred. The pathological findings of a liver biopsy specimen were not definitive, although drug-induced hepatotoxicity caused by ambrisentan was considered. However, the patient's liver dysfunction did not improve even after the discontinuation of ambrisentan. Finally, we diagnosed her with autoimmune hepatitis (AIH). Providing careful observation with a suspicion of AIH is important when treating PAH patients with autoantibodies.
Department of Respirology, Graduate School of Medicine, Chiba University, Japan.
Akira
A
Naito
Jiro
J
Terada
Nobuhiro
N
Tanabe
Toshihiko
T
Sugiura
Seiichiro
S
Sakao
Tatsuo
T
Kanda
Osamu
O
Yokosuka
Koichiro
K
Tatsumi
D001706:Biopsy; D000077300:Bosentan; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use*; D065627:Familial Primary Pulmonary Hypertension / Q000150:complications* / Q000188:drug therapy; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D008875:Middle Aged; D010666:Phenylpropionates / Q000627:therapeutic use; D011724:Pyridazines / Q000627:therapeutic use; D013449:Sulfonamides / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D018616:Ultrasonography, Doppler, Duplex
D065128:Endothelin Receptor Antagonists; D010666:Phenylpropionates; D011724:Pyridazines; D013449:Sulfonamides; C467894:ambrisentan; D000077300:Bosentan