Co-development of autoimmune hepatitis and Sjögren's syndrome triggered by the administration of herbal medicines
The patient was a 53-year-old woman who presented to our hospital with a 10-day history of yellowish skin coloration. She took herbal medicines of unknown origin as health supplements for 4 weeks until 4 weeks before the development of jaundice. She was healthy, and her medical history was unremarkable. She was not prescribed of any medications and rarely consumed alcohol.\nHer vital signs were unremarkable she denied travel history and past illness. Family history (including autoimmune disease) was not specific.\nLaboratory tests revealed the following results: aspartate aminotransferase (AST) level, 1,136 U/L (normal range, 5-34 U/L); alanine aminotransferase (ALT) level, 1,415 U/L (normal range, 0-55 U/L); alkaline phosphatase (ALP) level, 203 U/L (normal range, 40-150 U/L); and total bilirubin, 8.5 mg/dL (normal range, 0.2-1.4 mg/dL). The findings of serologic tests for hepatitis A, B, and C viruses and tests for detecting other viruses, including Epstein-Barr virus, cytomegalovirus, and herpes simplex virus, were negative. The titer of antinuclear antibody was 1:320, with the antigen-antibody reaction showing a speckled pattern. The results of studies for autoimmune markers, including anti-smooth muscle, antimitochondrial, liver-kidney microsomal, anti-ds DNA, anticentromere, and anti-Scl-70 antibodies, were negative. The levels of anti-Ro and anti-La antibodies were 258 and 429 U/mL (normal range, <150 U/mL), respectively, and immunoglobulin G (Ig G) level was 2,347 mg/dL (normal range, 700-1,400 mg/dL).\nAbdominal ultrasonography revealed slightly coarse echotexture of the liver parenchyma, indicating diffuse parenchymal disease. After admission, the patient complained of dryness in her mouth and foreign body sensation in her eye, which she had not experienced previously. Therefore, we performed a salivary gland scan and Schirmer's test. The salivary gland scan showed hot uptakes in both the salivary glands at 10 and 40 min and markedly diminished excretory function of both salivary glands. The Schirmer's test showed wetting of the paper by 2 mm in 5 min. We suspected primary Sjögren's syndrome (pSS) and administered conservative treatment. The results of liver function tests (LFTs) performed 21 days after admission revealed the following improved values: AST/ALT levels, 207/61 U/L; and total bilirubin level, 3.72 mg/dL. We first diagnosed the condition as drug-induced liver injury and performed follow-up evaluations in the outpatient department. The serum bilirubin and IgG levels obtained 10 days after this diagnosis repeatedly increased to 6.2 mg/dL and 3,039 mg/dL, respectively. We performed the liver biopsy for abnormal LFTs of unknown origin and biopsy findings showed interface hepatitis with moderate porto-periportal lymphoplasmacytic infiltration, periportal fibrosis, hepatic rosette formation and piecemeal necrosis (). There was no evidence of centrilobular zone 3 necrosis, suggestive of toxic hepatitis.\nOn the basis of the scoring system proposed by the International Autoimmune Hepatitis Group and the Simplified Criteria for the Diagnosis of AIH, the scores for our patient's condition were 16 and 8, respectively, and she was diagnosed with AIH. Initially, she was administered 30 mg prednisolone and 50 mg azathioprine. Prednisolone dosage was tapered down to 10 mg for 2 weeks. Thereafter, her condition improved, and within 3 months after the treatment, the results of the LFTs were within the normal range. She has been receiving maintenance therapy with 10 mg prednisolone and 50 mg azathioprine for 1 year, and no complications have been observed during this period ().