The liver is a common non-exocrine target in primary Sjögren's syndrome: a retrospective review

Abstract

Background: The autoimmune destruction of exocrine glands that defines primary Sjögren's syndrome (1 degrees SS) often extends to non-exocrine organs including the liver. We aimed to determine the prevalence of liver disease in patients with 1 degrees SS and to evaluate the association of this complication with other non-exocrine features and serologic markers of autoimmunity and systemic inflammation.

Methods: We reviewed 115 charts of patients with 1 degrees SS and further analyzed the 73 cases that fulfilled the European Epidemiology Center Criteria, seeking evidence for clinical and subclinical liver disease.

Results: Liver function tests had been determined in 59 of the 73 patients. Of those, 29 patients (49.1%) had abnormal liver function tests including 20.3% with clinically overt hepatic disease. Liver disease was the most common non-exocrine feature in this cohort. Risk factors for abnormal liver function tests were distributed similarly between the patients with and without liver disease. In 60% of patients with abnormal liver function tests no explanation for this complication was found except for 1 degrees SS. Liver involvement was significantly more common in 1 degrees SS patients who also had evidence of lung, kidney and hematological abnormalities. Patients with abnormal liver function tests were also more likely to have an elevated sedimentation rate and a positive anti-ENA during the course of their disease.

Conclusion: Liver involvement is a common complication in 1 degrees SS. Its presence correlates with systemic disease. We consider that this complication should be routinely sought in patients with 1 degrees SS, especially when a positive anti-ENA or evidence of systemic inflammation is found.

Figure 1

Liver function test values in Sjögren's patients with and without liver involvement. AP = alkaline phosphatase, AST = aspartate amino transferase, ALT = alanine amino transferase. Results represent mean ± range, ^* p < 0.05, n = 59 patients, with liver disfunction n = 29; without liver diysfunction n = 30

Figure 2

Association of liver disease with other extraglandular manifestations in patients with Sjögren's syndrome. Figure represents percentage of patients with and without liver disease who presented with other non-exocrine features of 1°SS. Patients with liver disease = 29; patients without liver disease = 30, ^* p < 0.05.

Figure 3

Association of liver disease with laboratory abnormalities in patients with Sjögren's syndrome. Figure represents percentage of patients with and without liver disease who presented with serological evidence of autoimmunity or inflammation. Patients with liver disease = 29; patients without liver disease = 30, ^* p < 0.05.