Increased risks of spontaneous bacterial peritonitis and interstitial lung disease in primary biliary cirrhosis patients with concomitant Sjögren syndrome

Chun-Ting Chen, Yu-Chen Tseng, Chih-Wei Yang, Hsuan-Hwai Lin, Peng-Jen Chen, Tien-Yu Huang, Yu-Lueng Shih, Wei-Kuo Chang, Tsai-Yuan Hsieh, Heng-Cheng Chu

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of longterm outcomes between them. A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the remaining 125 patients, 77 (61.6%) were PBC-only and 48 (38.4%) were PBC-SS patients. The mean follow-up duration was 8.76 years. During the observation period, the incidence of interstitial lung disease was higher in the PBC-SS group than in the PBC-only group (P = 0.005). The occurrence of spontaneous bacterial peritonitis was significantly different in PBC-SS patients than in PBC-only patients (P = 0.002). The overall survival was lower in PBC-SS patients than in PBC-only patients (P = 0.033). Although the incidence of hepatocellular carcinoma, end-stage renal disease, variceal bleeding, and hypothyroidism were all higher in the PBC-SS group than in the PBC-only group, the differences were not significant. Our study suggests that PBC-SS patients have a higher risk of developing interstitial lung disease and spontaneous bacterial peritonitis and have a poor prognosis. Aggressive surveillance of thyroid and pulmonary functions should therefore be performed in these patients. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.
Original languageEnglish
JournalMedicine (United States)
Volume95
Issue number2
DOIs
Publication statusPublished - 2016

Fingerprint

Biliary Liver Cirrhosis
Interstitial Lung Diseases
Peritonitis
Incidence
Sclerosing Cholangitis
Alcoholic Liver Diseases

Cite this

Increased risks of spontaneous bacterial peritonitis and interstitial lung disease in primary biliary cirrhosis patients with concomitant Sjögren syndrome. / Chen, Chun-Ting; Tseng, Yu-Chen; Yang, Chih-Wei; Lin, Hsuan-Hwai; Chen, Peng-Jen; Huang, Tien-Yu; Shih, Yu-Lueng; Chang, Wei-Kuo; Hsieh, Tsai-Yuan; Chu, Heng-Cheng.

In: Medicine (United States), Vol. 95, No. 2, 2016.

Research output: Contribution to journalArticle

Chen, Chun-Ting ; Tseng, Yu-Chen ; Yang, Chih-Wei ; Lin, Hsuan-Hwai ; Chen, Peng-Jen ; Huang, Tien-Yu ; Shih, Yu-Lueng ; Chang, Wei-Kuo ; Hsieh, Tsai-Yuan ; Chu, Heng-Cheng. / Increased risks of spontaneous bacterial peritonitis and interstitial lung disease in primary biliary cirrhosis patients with concomitant Sjögren syndrome. In: Medicine (United States). 2016 ; Vol. 95, No. 2.
@article{88a3156e00a04834bb696eb45ffd3b7a,
title = "Increased risks of spontaneous bacterial peritonitis and interstitial lung disease in primary biliary cirrhosis patients with concomitant Sj{\"o}gren syndrome",
abstract = "The incidence of Sj{\"o}gren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of longterm outcomes between them. A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the remaining 125 patients, 77 (61.6{\%}) were PBC-only and 48 (38.4{\%}) were PBC-SS patients. The mean follow-up duration was 8.76 years. During the observation period, the incidence of interstitial lung disease was higher in the PBC-SS group than in the PBC-only group (P = 0.005). The occurrence of spontaneous bacterial peritonitis was significantly different in PBC-SS patients than in PBC-only patients (P = 0.002). The overall survival was lower in PBC-SS patients than in PBC-only patients (P = 0.033). Although the incidence of hepatocellular carcinoma, end-stage renal disease, variceal bleeding, and hypothyroidism were all higher in the PBC-SS group than in the PBC-only group, the differences were not significant. Our study suggests that PBC-SS patients have a higher risk of developing interstitial lung disease and spontaneous bacterial peritonitis and have a poor prognosis. Aggressive surveillance of thyroid and pulmonary functions should therefore be performed in these patients. Copyright {\circledC} 2016 Wolters Kluwer Health, Inc. All rights reserved.",
author = "Chun-Ting Chen and Yu-Chen Tseng and Chih-Wei Yang and Hsuan-Hwai Lin and Peng-Jen Chen and Tien-Yu Huang and Yu-Lueng Shih and Wei-Kuo Chang and Tsai-Yuan Hsieh and Heng-Cheng Chu",
note = "Export Date: 22 March 2016 CODEN: MEDIA 通訊地址: Chu, H.-C.No. 252, Wu-Xing Street, Xinyi District 110, Taiwan; 電子郵件: chu5583@ms55.hinet.net 參考文獻: Jansen, P.L., Primary biliary cirrhosis. Overview (1999) Eur J Gastroenterol Hepatol, 11, pp. 583-586; Ali, A.H., Carey, E.J., Lindor, K.D., Diagnosis and management of primary biliary cirrhosis (2014) Expert Rev Clin Immunol, 10, pp. 1667-1678; Liu, B., Zhang, F.C., Zhang, Z.L., Interstitial lung disease and Sjogren's syndrome in primary biliary cirrhosis: A causal or casual association? (2008) Clin Rheumatol, 27, pp. 1299-1306; Kang, J.H., Lin, H.C., Comorbidities in patients with primary Sjogren's syndrome: A registry-based case-control study (2010) J Rheumatol, 37, pp. 1188-1194; Perez, B., Kraus, A., Lopez, G., Autoimmune thyroid disease in primary Sjogren's syndrome (1995) Am J Med, 99, pp. 480-484; Tunc, R., Gonen, M.S., Acbay, O., Autoimmune thyroiditis and anti-thyroid antibodies in primary Sjogren's syndrome: A case-control study (2004) Ann Rheum Dis, 63, pp. 575-577; Bossini, N., Savoldi, S., Franceschini, F., Clinical and morphological features of kidney involvement in primary Sjogren's syndrome (2001) Nephrol Dial Transplant, 16, pp. 2328-2336; Shimoda, S., Harada, K., Niiro, H., Biliary epithelial cells and primary biliary cirrhosis: The role of liver-infiltrating mononuclear cells (2008) Hepatology (Baltimore, Md), 47, pp. 958-965; Heathcote, E.J., Management of primary biliary cirrhosis. The American Association for the Study of Liver Diseases practice guidelines (2000) Hepatology (Baltimore, Md), 31, pp. 1005-1013; Kaplan, M.M., Gershwin, M.E., Primary biliary cirrhosis (2005) N Engl J Med, 353, pp. 1261-1273; Cho, H.C., Jung, H.Y., Sinn, D.H., Mortality after surgery in patients with liver cirrhosis: Comparison of Child-Turcotte-Pugh, MELD and MELDNa score (2011) Eur J Gastroenterol Hepatol, 23, pp. 51-59; Langegger, C., Wenger, M., Duftner, C., Use of the European preliminary criteria, the Breiman-classification tree and the American-European criteria for diagnosis of primary Sjogren's Syndrome in daily practice: A retrospective analysis (2007) Rheumatol Int, 27, pp. 699-702; Befeler, A.S., Di Bisceglie, A.M., Hepatocellular carcinoma: Diagnosis and treatment (2002) Gastroenterology, 122, pp. 1609-1619; Bruix, J., Sherman, M., Management of hepatocellular carcinoma: An update (2011) Hepatology (Baltimore, Md), 53, pp. 1020-1022; Shen, M., Zhang, F., Zhang, X., Primary biliary cirrhosis complicated with interstitial lung disease: A prospective study in 178 patients (2009) J Clin Gastroenterol, 43, pp. 676-679; Plantinga, L.C., Tuot, D.S., Powe, N.R., Awareness of chronic kidney disease among patients and providers (2010) Adv Chronic Kidney Dis, 17, pp. 225-236; Bhuva, M., Ganger, D., Jensen, D., Spontaneous bacterial peritonitis: An update on evaluation, management, and prevention (1994) Am J Med, 97, pp. 169-175; Chang, C.J., Hou, M.C., Liao, W.C., Management of acute gastric varices bleeding (2013) J Chin Med Assoc, 76, pp. 539-546; Garber, J.R., Cobin, R.H., Gharib, H., Clinical practice guidelines for hypothyroidism in adults: Cosponsored by the American Association of Clinical Endocrinologists and the American Thyroid Association (2012) Endocr Pract, 18, pp. 988-1028; Loaeza-Del-Castillo, A., Paz-Pineda, F., Oviedo-Cardenas, E., AST to platelet ratio index (APRI) for the noninvasive evaluation of liver fibrosis (2008) Ann Hepatol, 7, pp. 350-357; Kruger, F.C., Daniels, C.R., Kidd, M., APRI: A simple bedside marker for advanced fibrosis that can avoid liver biopsy in patients with NAFLD/NASH (2011) S Afr Med J, 101, pp. 477-480; Gershwin, M.E., Selmi, C., Worman, H.J., Risk factors and comorbidities in primary biliary cirrhosis: A controlled interview-based study of 1032 patients (2005) Hepatology (Baltimore, Md), 42, pp. 1194-1202; Mavragani, C.P., Fragoulis, G.E., Moutsopoulos, H.M., Endocrine alterations in primary Sjogren's syndrome: An overview (2012) JAutoimmun, 39, pp. 354-358; Jara, L.J., Navarro, C., Brito-Zeron, M.D.P., Thyroid disease in Sjogren's syndrome (2007) Clin Rheumatol, 26, pp. 1601-1606; Kelly, C.A., Foster, H., Pal, B., Primary Sjogren's syndrome in north east England: A longitudinal study (1991) Brit J Rheumatol, 30, pp. 437-442; Bouanani, M., Bataille, R., Piechaczyk, M., Autoimmunity to human thyroglobulin. Respective epitopic specificity patterns of antihuman thyroglobulin autoantibodies in patients with Sjogren's syndrome and patients with Hashimoto's thyroiditis (1991) Arthritis Rheum, 34, pp. 1585-1593; Aasarod, K., Haga, H.J., Berg, K.J., Renal involvement in primary Sjogren's syndrome (2000) Clin J Am Soc Nephrol, 93, pp. 297-304; Vitali, C., Tavoni, A., Sciuto, M., Renal involvement in primary Sjogren's syndrome: A retrospective-prospective study (1991) Scand J Rheumatol, 20, pp. 132-136; Matsumura, R., Kondo, Y., Sugiyama, T., Immunohistochemical identification of infiltrating mononuclear cells in tubulointerstitial nephritis associated with Sjogren's syndrome (1988) Clin Nephrol, 30, pp. 335-340; Salaffi, F., Manganelli, P., Carotti, M., A longitudinal study of pulmonary involvement in primary Sjogren's syndrome: Relationship between alveolitis and subsequent lung changes on high-resolution computed tomography (1998) Bri J Rheumatol, 37, pp. 263-269; Davidson, B.K., Kelly, C.A., Griffiths, I.D., Ten year follow up of pulmonary function in patients with primary Sjogren's syndrome (2000) Ann Rheum Dis, 59, pp. 709-712; Wallace, J.G., Jr., Tong, M.J., Ueki, B.H., Pulmonary involvement in primary biliary cirrhosis (1987) J Clin Gastroenterol, 9, pp. 431-435; Guarner, C., Runyon, B.A., Young, S., Intestinal bacterial overgrowth and bacterial translocation in cirrhotic rats with ascites (1997) J Hepatol, 26, pp. 1372-1378; Runyon, B.A., Morrissey, R.L., Hoefs, J.C., Opsonic activity of human ascitic fluid: A potentially important protective mechanism against spontaneous bacterial peritonitis (1985) Hepatology (Baltimore, Md), 5, pp. 634-637; Runyon, B.A., Low-protein concentration ascitic fluid is predisposed to spontaneous bacterial peritonitis (1986) Gastroenterology, 91, pp. 1343-1346; Llach, J., Rimola, A., Navasa, M., Incidence and predictive factors of first episode of spontaneous bacterial peritonitis in cirrhosis with ascites: Relevance of ascitic fluid protein concentration (1992) Hepatology (Baltimore, Md), 16, pp. 724-727; Tito, L., Rimola, A., Gines, P., Recurrence of spontaneous bacterial peritonitis in cirrhosis: Frequency and predictive factors (1988) Hepatology (Baltimore, Md), 8, pp. 27-31; Runyon, B.A., Patients with deficient ascitic fluid opsonic activity are predisposed to spontaneous bacterial peritonitis (1988) Hepatology (Baltimore, Md), 8, pp. 632-635; Laffi, G., Carloni, V., Baldi, E., Impaired superoxide anion, platelet-activating factor, and leukotriene B4 synthesis by neutrophils in cirrhosis (1993) Gastroenterology, 105, pp. 170-177; Rimola, A., Soto, R., Bory, F., Reticuloendothelial system phagocytic activity in cirrhosis and its relation to bacterial infections and prognosis (1984) Hepatology (Baltimore, Md), 4, pp. 53-58",
year = "2016",
doi = "10.1097/MD.0000000000002537",
language = "English",
volume = "95",
journal = "Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries",
issn = "0025-7974",
publisher = "Lippincott Williams and Wilkins",
number = "2",

}

TY - JOUR

T1 - Increased risks of spontaneous bacterial peritonitis and interstitial lung disease in primary biliary cirrhosis patients with concomitant Sjögren syndrome

AU - Chen, Chun-Ting

AU - Tseng, Yu-Chen

AU - Yang, Chih-Wei

AU - Lin, Hsuan-Hwai

AU - Chen, Peng-Jen

AU - Huang, Tien-Yu

AU - Shih, Yu-Lueng

AU - Chang, Wei-Kuo

AU - Hsieh, Tsai-Yuan

AU - Chu, Heng-Cheng

N1 - Export Date: 22 March 2016 CODEN: MEDIA 通訊地址: Chu, H.-C.No. 252, Wu-Xing Street, Xinyi District 110, Taiwan; 電子郵件: chu5583@ms55.hinet.net 參考文獻: Jansen, P.L., Primary biliary cirrhosis. Overview (1999) Eur J Gastroenterol Hepatol, 11, pp. 583-586; Ali, A.H., Carey, E.J., Lindor, K.D., Diagnosis and management of primary biliary cirrhosis (2014) Expert Rev Clin Immunol, 10, pp. 1667-1678; Liu, B., Zhang, F.C., Zhang, Z.L., Interstitial lung disease and Sjogren's syndrome in primary biliary cirrhosis: A causal or casual association? (2008) Clin Rheumatol, 27, pp. 1299-1306; Kang, J.H., Lin, H.C., Comorbidities in patients with primary Sjogren's syndrome: A registry-based case-control study (2010) J Rheumatol, 37, pp. 1188-1194; Perez, B., Kraus, A., Lopez, G., Autoimmune thyroid disease in primary Sjogren's syndrome (1995) Am J Med, 99, pp. 480-484; Tunc, R., Gonen, M.S., Acbay, O., Autoimmune thyroiditis and anti-thyroid antibodies in primary Sjogren's syndrome: A case-control study (2004) Ann Rheum Dis, 63, pp. 575-577; Bossini, N., Savoldi, S., Franceschini, F., Clinical and morphological features of kidney involvement in primary Sjogren's syndrome (2001) Nephrol Dial Transplant, 16, pp. 2328-2336; Shimoda, S., Harada, K., Niiro, H., Biliary epithelial cells and primary biliary cirrhosis: The role of liver-infiltrating mononuclear cells (2008) Hepatology (Baltimore, Md), 47, pp. 958-965; Heathcote, E.J., Management of primary biliary cirrhosis. The American Association for the Study of Liver Diseases practice guidelines (2000) Hepatology (Baltimore, Md), 31, pp. 1005-1013; Kaplan, M.M., Gershwin, M.E., Primary biliary cirrhosis (2005) N Engl J Med, 353, pp. 1261-1273; Cho, H.C., Jung, H.Y., Sinn, D.H., Mortality after surgery in patients with liver cirrhosis: Comparison of Child-Turcotte-Pugh, MELD and MELDNa score (2011) Eur J Gastroenterol Hepatol, 23, pp. 51-59; Langegger, C., Wenger, M., Duftner, C., Use of the European preliminary criteria, the Breiman-classification tree and the American-European criteria for diagnosis of primary Sjogren's Syndrome in daily practice: A retrospective analysis (2007) Rheumatol Int, 27, pp. 699-702; Befeler, A.S., Di Bisceglie, A.M., Hepatocellular carcinoma: Diagnosis and treatment (2002) Gastroenterology, 122, pp. 1609-1619; Bruix, J., Sherman, M., Management of hepatocellular carcinoma: An update (2011) Hepatology (Baltimore, Md), 53, pp. 1020-1022; Shen, M., Zhang, F., Zhang, X., Primary biliary cirrhosis complicated with interstitial lung disease: A prospective study in 178 patients (2009) J Clin Gastroenterol, 43, pp. 676-679; Plantinga, L.C., Tuot, D.S., Powe, N.R., Awareness of chronic kidney disease among patients and providers (2010) Adv Chronic Kidney Dis, 17, pp. 225-236; Bhuva, M., Ganger, D., Jensen, D., Spontaneous bacterial peritonitis: An update on evaluation, management, and prevention (1994) Am J Med, 97, pp. 169-175; Chang, C.J., Hou, M.C., Liao, W.C., Management of acute gastric varices bleeding (2013) J Chin Med Assoc, 76, pp. 539-546; Garber, J.R., Cobin, R.H., Gharib, H., Clinical practice guidelines for hypothyroidism in adults: Cosponsored by the American Association of Clinical Endocrinologists and the American Thyroid Association (2012) Endocr Pract, 18, pp. 988-1028; Loaeza-Del-Castillo, A., Paz-Pineda, F., Oviedo-Cardenas, E., AST to platelet ratio index (APRI) for the noninvasive evaluation of liver fibrosis (2008) Ann Hepatol, 7, pp. 350-357; Kruger, F.C., Daniels, C.R., Kidd, M., APRI: A simple bedside marker for advanced fibrosis that can avoid liver biopsy in patients with NAFLD/NASH (2011) S Afr Med J, 101, pp. 477-480; Gershwin, M.E., Selmi, C., Worman, H.J., Risk factors and comorbidities in primary biliary cirrhosis: A controlled interview-based study of 1032 patients (2005) Hepatology (Baltimore, Md), 42, pp. 1194-1202; Mavragani, C.P., Fragoulis, G.E., Moutsopoulos, H.M., Endocrine alterations in primary Sjogren's syndrome: An overview (2012) JAutoimmun, 39, pp. 354-358; Jara, L.J., Navarro, C., Brito-Zeron, M.D.P., Thyroid disease in Sjogren's syndrome (2007) Clin Rheumatol, 26, pp. 1601-1606; Kelly, C.A., Foster, H., Pal, B., Primary Sjogren's syndrome in north east England: A longitudinal study (1991) Brit J Rheumatol, 30, pp. 437-442; Bouanani, M., Bataille, R., Piechaczyk, M., Autoimmunity to human thyroglobulin. Respective epitopic specificity patterns of antihuman thyroglobulin autoantibodies in patients with Sjogren's syndrome and patients with Hashimoto's thyroiditis (1991) Arthritis Rheum, 34, pp. 1585-1593; Aasarod, K., Haga, H.J., Berg, K.J., Renal involvement in primary Sjogren's syndrome (2000) Clin J Am Soc Nephrol, 93, pp. 297-304; Vitali, C., Tavoni, A., Sciuto, M., Renal involvement in primary Sjogren's syndrome: A retrospective-prospective study (1991) Scand J Rheumatol, 20, pp. 132-136; Matsumura, R., Kondo, Y., Sugiyama, T., Immunohistochemical identification of infiltrating mononuclear cells in tubulointerstitial nephritis associated with Sjogren's syndrome (1988) Clin Nephrol, 30, pp. 335-340; Salaffi, F., Manganelli, P., Carotti, M., A longitudinal study of pulmonary involvement in primary Sjogren's syndrome: Relationship between alveolitis and subsequent lung changes on high-resolution computed tomography (1998) Bri J Rheumatol, 37, pp. 263-269; Davidson, B.K., Kelly, C.A., Griffiths, I.D., Ten year follow up of pulmonary function in patients with primary Sjogren's syndrome (2000) Ann Rheum Dis, 59, pp. 709-712; Wallace, J.G., Jr., Tong, M.J., Ueki, B.H., Pulmonary involvement in primary biliary cirrhosis (1987) J Clin Gastroenterol, 9, pp. 431-435; Guarner, C., Runyon, B.A., Young, S., Intestinal bacterial overgrowth and bacterial translocation in cirrhotic rats with ascites (1997) J Hepatol, 26, pp. 1372-1378; Runyon, B.A., Morrissey, R.L., Hoefs, J.C., Opsonic activity of human ascitic fluid: A potentially important protective mechanism against spontaneous bacterial peritonitis (1985) Hepatology (Baltimore, Md), 5, pp. 634-637; Runyon, B.A., Low-protein concentration ascitic fluid is predisposed to spontaneous bacterial peritonitis (1986) Gastroenterology, 91, pp. 1343-1346; Llach, J., Rimola, A., Navasa, M., Incidence and predictive factors of first episode of spontaneous bacterial peritonitis in cirrhosis with ascites: Relevance of ascitic fluid protein concentration (1992) Hepatology (Baltimore, Md), 16, pp. 724-727; Tito, L., Rimola, A., Gines, P., Recurrence of spontaneous bacterial peritonitis in cirrhosis: Frequency and predictive factors (1988) Hepatology (Baltimore, Md), 8, pp. 27-31; Runyon, B.A., Patients with deficient ascitic fluid opsonic activity are predisposed to spontaneous bacterial peritonitis (1988) Hepatology (Baltimore, Md), 8, pp. 632-635; Laffi, G., Carloni, V., Baldi, E., Impaired superoxide anion, platelet-activating factor, and leukotriene B4 synthesis by neutrophils in cirrhosis (1993) Gastroenterology, 105, pp. 170-177; Rimola, A., Soto, R., Bory, F., Reticuloendothelial system phagocytic activity in cirrhosis and its relation to bacterial infections and prognosis (1984) Hepatology (Baltimore, Md), 4, pp. 53-58

PY - 2016

Y1 - 2016

N2 - The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of longterm outcomes between them. A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the remaining 125 patients, 77 (61.6%) were PBC-only and 48 (38.4%) were PBC-SS patients. The mean follow-up duration was 8.76 years. During the observation period, the incidence of interstitial lung disease was higher in the PBC-SS group than in the PBC-only group (P = 0.005). The occurrence of spontaneous bacterial peritonitis was significantly different in PBC-SS patients than in PBC-only patients (P = 0.002). The overall survival was lower in PBC-SS patients than in PBC-only patients (P = 0.033). Although the incidence of hepatocellular carcinoma, end-stage renal disease, variceal bleeding, and hypothyroidism were all higher in the PBC-SS group than in the PBC-only group, the differences were not significant. Our study suggests that PBC-SS patients have a higher risk of developing interstitial lung disease and spontaneous bacterial peritonitis and have a poor prognosis. Aggressive surveillance of thyroid and pulmonary functions should therefore be performed in these patients. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.

AB - The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of longterm outcomes between them. A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the remaining 125 patients, 77 (61.6%) were PBC-only and 48 (38.4%) were PBC-SS patients. The mean follow-up duration was 8.76 years. During the observation period, the incidence of interstitial lung disease was higher in the PBC-SS group than in the PBC-only group (P = 0.005). The occurrence of spontaneous bacterial peritonitis was significantly different in PBC-SS patients than in PBC-only patients (P = 0.002). The overall survival was lower in PBC-SS patients than in PBC-only patients (P = 0.033). Although the incidence of hepatocellular carcinoma, end-stage renal disease, variceal bleeding, and hypothyroidism were all higher in the PBC-SS group than in the PBC-only group, the differences were not significant. Our study suggests that PBC-SS patients have a higher risk of developing interstitial lung disease and spontaneous bacterial peritonitis and have a poor prognosis. Aggressive surveillance of thyroid and pulmonary functions should therefore be performed in these patients. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.

U2 - 10.1097/MD.0000000000002537

DO - 10.1097/MD.0000000000002537

M3 - Article

VL - 95

JO - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries

JF - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries

SN - 0025-7974

IS - 2

ER -