Characteristics Of Patients With Primary Sj(?)gren's Syndrome Complicated With Pancreatic Damage

Background:Primary Sjogren syndrome(pSS)is a chronic systemic autoimmune disease characterized by a lymphocytic infiltration and destruction of exocrine glands,involving mainly women of 40?50 years old.The estimated prevalence is 0.33 to 0.77 per 100 persons in China.pSS is mainly characterized by dryness of the mouth and eyes and kerato-conjunctivitis sicca.Besides,it may affect the gastrointestinal system,from the mouth,esophagus,bowel to liver and pancreas.Pancreatic involvement,although rare,includes acute or chronic pancreatitis,pancreatic insufficiency and morphology abnormalities.Objective:To analyze the clinical characteristics of primary Sjogren's syndrome(pSS)complicated with pancreatic damage.Methods:The clinical features,laboratory examinations,radiological findings,treatments and prognosis of twenty-two pSS patients complicated with pancreatic damage at Peking Union Medical College Hospital from January 2005 to January 2018 were retrospectively analyzed.We summarized 1:2 matched pSS patients without pancreatic damage by age and gender during the same period.Relevant factors were analyzed by t-test between two groups and multiple factors logistic regression analysis was conducted.Results:Among 1627 patients with pSS,twenty-two patients had pancreas involvement.There were twenty-one females and one male.The mean age of disease onset was 53.59 years old.Six patients(6/22)presented pancreatic abnormities as the initial manifestations.pSS with pancreatic involvement manifested as nausea(10/22),vomiting(8/22),abdominal pain(10/22),abdominal distension(5/22)and diarrhea(3/22).IgG increased in ten(10/18)patients;complement decreased in four(4/14)patients;ESR elevated in fourteen(14/19)patients and hsCRP elevated in ten(10/20)patients.There were ten(10/17)patients presenting with elevated serum amylase and nine(9/17)patients with elevated blood lipase.Abnormal abdominal ultrasound results were found in eight(8/15)patients and abnormal computerized tomography were found in sixteen patients(16/19).pSS patients with pancreas involvement had longer course of disease and higher triglyceride level than pSS patients without pancreas involvement(P<0.05).The course of disease(OR=0.870,95%CI:0.776-0.976,P=0.018)and triglyceride level(OR=0.396,95%CI:0.181-0.870,P=0.021)are the independent predictors of pSS with pancreatic involvement.Six(6/11)patients achieved clinical improvements,who were mainly treated with high-dose steroids and immunosuppressive agents.Conclusion:Pancreas involvement is a relatively rare complication of pSS.Pancreatic dysfunction and abnormal abdominal imaging manifestations were found in most of the patients.Some patients with pancreatic damage didn't present with gastrointestinal symptoms,which might cause missed diagnosis.