A Rare Case of Primary Sjogren’s Syndrome Coexisting With Gilbert Syndrome

Gilbert syndrome (GS) is an autosomal recessive inherited bilirubin metabolism disorder characterized by chronic unconjugated hyperbilirubinemia in the absence of hemolysis and liver disease. Primary Sjogren's (pSS), mainly occurring women, a common connective tissue disease (CTD) wherein levels are generally reduced. We report rare case pSS coexisting with GS. A 35-year-old female patient presented to our hospital hyperbilirubinemia, for which low-dose methylprednisolone was ineffective. The patient’s function test results were normal, serological tests hepatitis virus negative, abdominal ultrasound did not indicate abnormal morphology. Bone mineral density determination showed that Z scores left femoral neck lumbar spine -1.9 -2.6, respectively, T -2.1 -2.8, respectively. Full-exon sequencing revealed homozygous TA insertion TATA box (A(TA)7TAA) heterozygous base substitution from C at nucleotide position 686 exon 1 (c.686C>A) uridine glucuronosyltransferase 1A1 (UGT1A1) gene. Therefore, diagnosed pSS, GS, osteoporosis. dose then reduced gradually stopped, treatment osteoporosis strengthened. To knowledge, this first It important clarify cause patients CTD, including affects formulation correct plans.