151. Autoimmune hepatitis in a patient with pulmonary arterial hypertension treated with endothelin receptor antagonists.
- Author
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Naito A, Terada J, Tanabe N, Sugiura T, Sakao S, Kanda T, Yokosuka O, and Tatsumi K
- Subjects
- Biopsy, Bosentan, Diagnosis, Differential, Drug Therapy, Combination, Familial Primary Pulmonary Hypertension drug therapy, Female, Follow-Up Studies, Hepatitis, Autoimmune complications, Hepatitis, Autoimmune diagnosis, Humans, Middle Aged, Phenylpropionates therapeutic use, Pyridazines therapeutic use, Sulfonamides therapeutic use, Tomography, X-Ray Computed, Ultrasonography, Doppler, Duplex, Endothelin Receptor Antagonists therapeutic use, Familial Primary Pulmonary Hypertension complications, Hepatitis, Autoimmune drug therapy
- Abstract
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.
- Published
- 2014
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