SLE diagnosis was rigorously confirmed by a medical record review according to the revised 1997 American College of Rheumatology classification criteria for SLE. was 4.7 1.0 years. The most common initial presentation was the involvement of the hematological system in 9 patients and then skin and mucosal involvement in 5 patients, arthritis in 4 patients, and nephritis in 3 patients. Patients with cirrhosis had a significantly higher rate of hematological system involvement (thrombocytopenia and leukopenia) and worse liver function; a higher level of immune globulin G had higher mortality ( 0.05) than patients without cirrhosis. Conclusions Cirrhosis is a rare and severe subtype of SLE with a poor prognosis. Those patients with hematological system involvement and impaired liver function should be paid more attention. 1. Introduction The systemic lupus erythematosus (SLE) is an autoantibody-mediated, diffuse connective tissue disease with extremely variable and heterogeneous clinical presentation. A variety of organs can be involved, with the most common organ kidney, followed by the cardiovascular, nervous system, respiratory system, digestive system, blood system, etc., of which digestive damage, especially liver damage, is less common. The liver is not only a lymphoid organ involved in the immune response [1] but also a target of autoimmune reactions. The most common finding is an elevation in liver enzymes. Nevertheless, advanced liver disease with cirrhosis and liver failure is rare in patients with connective tissue diseases. The liver may be involved in 19.4% to 60% of patients with SLE at some point during the diseases, of which cirrhosis only accounts for about 1-2% [2C4]. Relatively, few studies have reported data of cirrhosis in SLE. We conduct this case-control study to explore the clinical characteristics of systemic lupus erythematosus with cirrhosis. 2. Patients and Methods 2.1. Study Population This study was a single-center retrospective study. We utilized the Hospital Information Retrieval System to identify the SLE and cirrhosis patients admitted to the Peking Union Medical College Hospital (PUMCH) from January 2012 to December 2018. The records of each patient’s hospitalization and outpatient visit can be checked by the patient’s identification document (ID) in the medical record system. SLE diagnosis was rigorously confirmed by a medical record review according to the revised 1997 American College of Rheumatology classification criteria for SLE SB-505124 [5]. The diagnosis of liver cirrhosis was based on a combination of clinical, laboratory, and imaging criteria features (e.g., nodular liver, portosystemic collaterals found in abdominal echography, computerized tomography (CT), and/or magnetic resonance imaging (MRI)) [6]. We exclude other reason of cirrhosis such as alcoholic liver cirrhosis, nonalcoholic fatty liver disease, viral infection, drug-induced liver disease, inherited metabolic liver disease, cardiogenic liver disease, and other autoimmune liver disease, through screening medical history and systemic examination. For medical history, we focused on alcoholic abuse, hepatotoxic medication use, heart disease history, and family metabolic history. For systemic examination, we did a laboratory test such as hepatitis B/C virus test, ceruloplasmin test, autoantibody of primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH), transthoracic SB-505124 echocardiograph, and hepatic vascular ultrasound. We present a case-controlled study, matched by age and gender, to discover the clinical characteristics of systemic lupus erythematosus with cirrhosis. The date of entry was the date of the first diagnosis of cirrhosis for the cirrhosis group and as the hospital admission date for the noncirrhosis group. Patients were followed until death, liver transplantation, or end of the study (July 2019). Written informed consent was obtained from each patient. 2.2. Clinical and Laboratory Data Collection Medical records were retrospectively reviewed, and data were collected in a dedicated case report form. The following data were obtained from medical records by well-trained rheumatologists: gender, age at entry, duration of SLE, involved organs, lupus disease activity, and laboratory data. Lupus disease activity was evaluated using the SLE disease activity index 2000 (SLEDAI-2K), stratified to stable ( 5), mild active (5-9), moderate active (10-14), and severe active ( 14). The severity of liver diseases was evaluated with the Child-Pugh score. Clinical ascites, hepatic encephalopathy, and the other complications were classified as absent or present. Routine blood tests, including platelet count, hemoglobin, liver and renal SB-505124 function tests, and prothrombin time, were collected. All parameters used SB-505124 for comparison were those at the time of diagnosis of cirrhosis for the cirrhosis group and the diagnosis of SLE for the noncirrhosis group. 2.3. Antibody IFNA17 Assay The anti-nuclear antibody (ANA) SB-505124 was tested by indirect immunofluorescence (IIF) using HEp-2 cell substrates. The anti-dsDNA antibody was measured by IIF using flagellate protoctista substrates and enzyme-linked immunosorbent assay (ELISA). Anti-ENA antibodies.