A rare clinical case of overlapping the sjogren’s syndrome, autoimmune hepatitis, and primary sclerosing cholangitis
- VernacularTitle:Шегрен өвчин болон аутоиммун гепатит, анхдагч хатууралт холангит хавсарсан эмнэлзүйн ховор тохиолдол
- Author:
Misheel B
1
,
2
;
Duulim B
1
,
2
;
Tsolmon D
1
,
3
;
Zulgerel D
1
,
3
Author Information
1. School of Medicine, MNUMS
2. Intermed Hospital
3. Mongolian-Japanese Hospital, MNUMS
- Publication Type:Case Reports
- Keywords:
Biopsy, liver dysfunction, liver cirrhosis, symptoms, chronic disease
- From:
Mongolian Journal of Health Sciences
2025;87(3):244-247
- CountryMongolia
- Language:Mongolian
-
Abstract:
Background:Sjögren's syndrome is a chronic autoimmune inflammatory disease
characterized by lymphocytic infiltration of exocrine glands, typically presenting with
symptoms such as xerostomia (dry mouth) and xerophthalmia (dry eyes). This disease
can appear as an isolated condition or in association with other diseases. It is
most commonly associated with rheumatologic disorders such as rheumatoid arthritis
and systemic lupus erythematosus, but in rare cases, it may also be associated with
other autoimmune diseases involving various organ systems. Autoimmune hepatitis
is a chronic liver inflammation characterized by elevated serum globulin and antibody
levels. The coexistence of Sjögren's syndrome and autoimmune hepatitis is very rare,
with some sources indicating an incidence of only 1.7%. In our clinical case report, a
rare occurrence was observed in a 52-year-old female who had been diagnosed with
Sjögren's syndrome in 2022 and later developed symptoms of hepatitis, leading to a
diagnosis of autoimmune hepatitis. When autoimmune hepatitis coexists with other
autoimmune diseases, it often presents with mild clinical symptoms, which may delay
the diagnosis.
Case report:A 52-year-old female patient presented in 2020 with complaints
of dry eyes and mouth, blurred vision, decreased saliva production, fatigue,
and occasional swelling of the lymph nodes, as reported during her medical history.
In December 2022, she was seen by a rheumatologist at the Mongolia-Japan Medical
Center. Laboratory tests revealed positive results for anti-SSA52, CENP-B, ANA IgG,
and RF, with altered liver function (see Table 1). A Shirmer test was positive, and saliva
production was ≤ 0.1 mL/min. According to the ACR-EULAR 2016 diagnostic criteria,
she scored 5 points, confirming the diagnosis of Sjögren's syndrome. Treatment was
initiated with hydroxychloroquine and corticosteroids, along with medications for gastric
protection, liver protection, and prevention of complications. In March 2023, during a
follow-up visit, laboratory tests showed altered liver function (see Table 1). Hepatitis
B and C antibodies were negative, and Liver-9-line results were normal. Due to the
positive clinical dynamics of Sjögren's syndrome, the dose of hydroxychloroquine was
reduced, and other treatments were continued. The patient was also advised to see a
gastroenterologist for further evaluation. In August 2024, she presented to the gastroenterology
department at Intermed Hospital with complaints of left abdominal pain and
jaundice. Upon examination, abdominal ultrasound was normal, but laboratory results
showed elevated IgG (132 H), ANA (>400 U/L), ALT (119.2 U/L), and AST (132.8 U/L),
which raised suspicion of autoimmune hepatitis. Consequently, a liver biopsy was performed.
Liver Biopsy (August 2024): The liver tissue shows a portal triad with 13 portal
trios, where there is mild infiltration of lymphocytes, eosinophils, and a few neutrophils
around the portal triads. Focal macrosteatosis of hepatocytes and bile stasis are
observed. In Masson’s trichrome stain: There is fibrosis around the portal triads with
connective tissue proliferation (ISHAK-1). In PAS staining: Focal positive staining is
observed within the hepatocytes. According to the international autoimmune hepatitis
diagnostic criteria, the diagnosis of autoimmune hepatitis was confirmed with a score of
8 (ANA +2, IgG +2, Biopsy +2, HBV HCV negative +2). Liver biopsy confirmed the diagnosis
of primary sclerosing cholangitis. Treatment with corticosteroids and choleretic
therapy was initiated, and a follow-up visit is planned in one month. In September 2024,
upon follow-up, liver function had improved compared to previous tests, and treatment
was continued. The patient is now under ongoing monitoring by both a gastroenterologist
and a rheumatologist.
Conclusion:The coexistence of Sjögren's syndrome and
autoimmune hepatitis is a rare clinical occurrence, with foreign studies reporting an
incidence of less than 1%. However, if autoimmune hepatitis goes undiagnosed, it can
lead to complications such as liver cirrhosis and hepatocellular carcinoma. Therefore,
it is of critical importance to perform antibody tests and tissue biopsy for early detection
and differential diagnosis in patients diagnosed with Sjögren's syndrome who present
with liver and biliary symptoms or laboratory findings indicating liver dysfunction. This
clinical case emphasizes the need for careful monitoring and early intervention.
- Full text:2025052423171872793244-247.pdf
