Abstract
Aim: Primary biliary cholangitis is a chronic cholestatic liver disease mainly affects middle age women. Persistently elevated cholestatic liver enzymes, normal biliary imaging and the presence of antimitochondrial antibodies are key elements of the diagnosis. It was demonstrated that disease can progress under ursodeoxycholic acid (UDCA) treatment. Our aim is to demonstrate response to treatment of PBC patients. Also we aim to determine accuracy rate of Paris-I, Paris-II, Toronto and Barcelona criteria for predict UDCA treatment in PBC.
Material and Method: 82 PBC patients diagnosed between 2000-2010 were investigated retrospectively. Only patients who were treated UDCA with a daily 13-15 mg/kg dose at least two years were included into the study. The diagnosis of PBC was made by two of the following criteria: The presence of AMA, elevated cholestatic liver enzymes and biopsy.
Results: Patients biochemical parameters at 3., 6., 12., 24., 36., 48. and 60. months were compared between Paris-I, Paris-II, Toronto and Barcelona criteria. Paris-I was found the best test to predict treatment response (%71,7). Other tests are less successful to predict treatment response (Toronto:%54,6, Barcelona: %50,6 and Paris-II:%47,4). Beyond the second year of the treatment, successful ratio of tests for predicting response to treatment were increased.29.09
Conclusion: Although liver biochemical tests are getting better in the third month of UDCA treatment, accuracy rate of Paris-I, Paris-II, Toronto and Barcelona increases after the second year of the treatment. In our study we found low levels of the accuracy rate of these tests.
References
- 1- Gershwin ME, Mackay IR. The causes of primary biliary cirrhosis: convenient and inconvenient truths. Hepatology 2008;47:737-45. 2- Hirschfield GM, Invernizzi P. Progress in the genetics of primary biliary cirrhosis. Semin Liver Dis 2011;31:147-56. 3- Rubin E, Schaffner F, Popper H. Primary biliary cirrhosis. Chronic non-suppurative destructive cholangitis. Am J Pathol 1965;46:387-407. 4- Lindor KD, Gershwin ME, Poupon R, et al. Primary biliary cirrhosis. Hepatology 2009;50:291-308. 5- Poupon RE, Balkau B, Eschwege E, et al. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-PBC Study Group. N Engl J Med 1991;324:1548-54. 6- Poupon R. Primary biliary cirrhosis: a 2010 update. J Hepatol 2010;52:745-58. 7- Corpechot C, Carrat F, Bahr A, et al. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 2005;128:297-303. 8- Bonnand AM, Heathcote EJ, Lindor KD, et al. Clinical significance of serum bilirubin levels under ursodeoxycholic acid therapy in patients with primary biliary cirrhosis. Hepatology 1999;29:39-43. 9- Corpechot C, Carrat F, Poupon R, et al. Primary biliary cirrhosis: incidence and predictive factors of cirrhosis development in ursodiol treated patients. Gastroenterology 2002;122:652-8. 10- Pares A, Caballeria L, Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid. Gastroenterology 2006;130:715-20. 11- Kuiper EM, Hansen BE, de Vries RA, et al. Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology 2009;136:1281-7. 12- Kumagi T, Guindi M, Fischer SE, et al. Baseline ductopenia and treatment response predict long-term histological progression in primary biliary cirrhosis. Am J Gastroenterol 2010;105:2186-94. 13- Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet 2003;362:53-61. 14- Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med. 2005;353:1261-73. 15- Poupon RE, Lindor KD, Cauch-Dudek K, et al. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology 1997;113:884-90. 16- Silveira MG, Talwalkar JA, Angulo P, et al. Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes. Am J Gastroenterol 2007;102:1244-50. 17- Poupon RE, Poupon R, Balkau B. Ursodiol for the long-term treatment of primary biliary cirrhosis. The UDCA-PBC Study Group. N Engl J Med 1994;330:1342-7. 18- Zhang LN, Shi TY, Shi XH, et al. Early biochemical response to ursodeoxycholic acid and long-term prognosis of primary biliary cirrhosis: Results of a 14-year cohort study. Hepatology 2013;58:264-72. 19- Silveira MG, Brunt EM, Heathcote J, et al. American Association for the Study of Liver Diseases endpoints conference: design and endpoints for clinical trials in primary biliary cirrhosis. Hepatology 2010;52:349-59. 20- Lammert C, Juran BD, Schlicht E, et al. Biochemical response to ursodeoxycholic acid predicts survival in a North American cohort of primary biliary cirrhosis patients. J Gastroenterol 2014;49:1414-20. 21- Papastergiou V, Tsochatzis EA, Rodriquez-Peralvarez M, et al. Biochemical criteria at 1 year are not robust indicators of response to ursodeoxycholic acid in early primary biliary cirrhosis: results from a 29-year cohort study. Aliment Pharmacol Ther 2013;38:1354-64.
Abstract
Giriş ve Amaç: Primer
biliyer kolanjit çoğunlukla orta yaş kadınların etkilendiği, kronik kolestatik
bir karaciğer hastalığıdır. Persistan kolestatik karaciğer enzim yüksekliği,
normal biliyer görüntüleme ve antimitokondrial antikor varlığı primer biliyer
kolanjit teşhisi için gereklidir. Ursodeoksikolik asit tedavisi altındaki bazı hasta
gruplarında hastalığın ilerlediği gösterilmiştir. Biz kliniğimizde primer
biliyer kolanjit tanısı ile izlenen hastaların tedavi sonuçlarını vermeyi ve
Paris-I ve Paris-II, Toronto ve Barselona kriterlerine göre ursodeoksikolik
asit yanıtını ön görmede testlerin doğruluk oranlarını göstermeyi amaçladık
Gereç ve
Yöntem: Yüksek İhtisas Hastanesi Hepatoloji
Bölümü’nde 2000-2010 yılları arasında primer biliyer kolanjit tanısı alan 82 hasta retrospektif
olarak incelendi. Primer biliyer kolanjit tanısı antimitokondrial
antikor
pozitifiliği, alkalen fosfataz enzim yüksekliği ve/veya biyopsi ile konuldu. En
az 2 yıl boyunca ursodeoksikolik asit 13-15 mg/kg/g dozunda alan
hastalar çalışmaya alındı
Sonuçlar: Hastalar 3. ay, 6 ay,
1. yıl, 2. yıl, 3. yıl, 4. yıl ve 5. yıl biyokimyasal yanıt açısından Barselona,
Paris I-II ve Toronto kriterleri açısından değerlendirildi. Tedaviye yanıtı ön
görmede en başarılı test doğruluk oranına göre Paris I (%71.7) olarak
hesaplandı. Hastalarımızda tedavi başarısını ön görmekte en başarılı diğer
testler sırası ile Toronto (%54.6), Barselona (%50.6) ve Paris-II (%47.4) olarak
saptandı. Tedavi yanıtını ön görmedeki başarı tüm testlerde 2 yıldı ve
sonrasında başarı oranları artmakta idi.
Sonuç: Ursodeoksikolik
asit tedavisi
başlanan hastalarda 3. ayda karaciğer biyokimya testlerinde iyileşme
görülmesine rağmen, ursodeoksikolik asit tedavi yanıtını ön görmede
kullanılan testlerin doğruluk oranları 2. yılda artmakta idi. Çalışmamızda tanı
testlerinin doğruluk oranı düşük idi. Bu nedenle daha geniş hasta gruplu
çalışmalar yapılıp sonuçlarımızın doğrulanması gerekmektedir.
Abstract
Aim: Primary biliary cholangitis is a
chronic cholestatic liver disease mainly affects middle age women. Persistently
elevated cholestatic liver enzymes, normal biliary imaging and the presence of
antimitochondrial antibodies are key elements of the diagnosis. It was
demonstrated that disease can progress under ursodeoxycholic acid (UDCA)
treatment. Our aim is to demonstrate response to treatment of PBC patients.
Also we aim to determine accuracy rate of Paris-I, Paris-II, Toronto and
Barcelona criteria for predict UDCA treatment in PBC.
Material and Method: 82 PBC patients
diagnosed between 2000-2010 were investigated retrospectively. Only patients
who were treated UDCA with a daily 13-15 mg/kg dose at least two years were
included into the study. The diagnosis of PBC was made by two of the following
criteria: The presence of AMA, elevated cholestatic liver enzymes and biopsy.
Results: Patients biochemical
parameters at 3., 6., 12., 24., 36., 48. and 60. months were compared between
Paris-I, Paris-II, Toronto and Barcelona criteria. Paris-I was found the best
test to predict treatment response (%71,7). Other tests are less successful to
predict treatment response (Toronto:%54,6, Barcelona: %50,6 and
Paris-II:%47,4). Beyond the second year of the treatment, successful ratio of tests
for predicting response to treatment were increased.
Conclusion: Although liver
biochemical tests are getting better in the third month of UDCA treatment,
accuracy rate of Paris-I, Paris-II, Toronto and Barcelona increases after the
second year of the treatment. In our study we found low levels of the accuracy
rate of these tests.Giriş ve Amaç: Primer
biliyer kolanjit çoğunlukla orta yaş kadınların etkilendiği, kronik kolestatik
bir karaciğer hastalığıdır. Persistan kolestatik karaciğer enzim yüksekliği,
normal biliyer görüntüleme ve antimitokondrial antikor varlığı primer biliyer
kolanjit teşhisi için gereklidir. Ursodeoksikolik asit tedavisi altındaki bazı hasta
gruplarında hastalığın ilerlediği gösterilmiştir. Biz kliniğimizde primer
biliyer kolanjit tanısı ile izlenen hastaların tedavi sonuçlarını vermeyi ve
Paris-I ve Paris-II, Toronto ve Barselona kriterlerine göre ursodeoksikolik
asit yanıtını ön görmede testlerin doğruluk oranlarını göstermeyi amaçladık
Gereç ve
Yöntem: Yüksek İhtisas Hastanesi Hepatoloji
Bölümü’nde 2000-2010 yılları arasında primer biliyer kolanjit tanısı alan 82 hasta retrospektif
olarak incelendi. Primer biliyer kolanjit tanısı antimitokondrial
antikor
pozitifiliği, alkalen fosfataz enzim yüksekliği ve/veya biyopsi ile konuldu. En
az 2 yıl boyunca ursodeoksikolik asit 13-15 mg/kg/g dozunda alan
hastalar çalışmaya alındı
Sonuçlar: Hastalar 3. ay, 6 ay,
1. yıl, 2. yıl, 3. yıl, 4. yıl ve 5. yıl biyokimyasal yanıt açısından Barselona,
Paris I-II ve Toronto kriterleri açısından değerlendirildi. Tedaviye yanıtı ön
görmede en başarılı test doğruluk oranına göre Paris I (%71.7) olarak
hesaplandı. Hastalarımızda tedavi başarısını ön görmekte en başarılı diğer
testler sırası ile Toronto (%54.6), Barselona (%50.6) ve Paris-II (%47.4) olarak
saptandı. Tedavi yanıtını ön görmedeki başarı tüm testlerde 2 yıldı ve
sonrasında başarı oranları artmakta idi.
Sonuç: Ursodeoksikolik
asit tedavisi
başlanan hastalarda 3. ayda karaciğer biyokimya testlerinde iyileşme
görülmesine rağmen, ursodeoksikolik asit tedavi yanıtını ön görmede
kullanılan testlerin doğruluk oranları 2. yılda artmakta idi. Çalışmamızda tanı
testlerinin doğruluk oranı düşük idi. Bu nedenle daha geniş hasta gruplu
çalışmalar yapılıp sonuçlarımızın doğrulanması gerekmektedir.
References
- 1- Gershwin ME, Mackay IR. The causes of primary biliary cirrhosis: convenient and inconvenient truths. Hepatology 2008;47:737-45. 2- Hirschfield GM, Invernizzi P. Progress in the genetics of primary biliary cirrhosis. Semin Liver Dis 2011;31:147-56. 3- Rubin E, Schaffner F, Popper H. Primary biliary cirrhosis. Chronic non-suppurative destructive cholangitis. Am J Pathol 1965;46:387-407. 4- Lindor KD, Gershwin ME, Poupon R, et al. Primary biliary cirrhosis. Hepatology 2009;50:291-308. 5- Poupon RE, Balkau B, Eschwege E, et al. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-PBC Study Group. N Engl J Med 1991;324:1548-54. 6- Poupon R. Primary biliary cirrhosis: a 2010 update. J Hepatol 2010;52:745-58. 7- Corpechot C, Carrat F, Bahr A, et al. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 2005;128:297-303. 8- Bonnand AM, Heathcote EJ, Lindor KD, et al. Clinical significance of serum bilirubin levels under ursodeoxycholic acid therapy in patients with primary biliary cirrhosis. Hepatology 1999;29:39-43. 9- Corpechot C, Carrat F, Poupon R, et al. Primary biliary cirrhosis: incidence and predictive factors of cirrhosis development in ursodiol treated patients. Gastroenterology 2002;122:652-8. 10- Pares A, Caballeria L, Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid. Gastroenterology 2006;130:715-20. 11- Kuiper EM, Hansen BE, de Vries RA, et al. Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology 2009;136:1281-7. 12- Kumagi T, Guindi M, Fischer SE, et al. Baseline ductopenia and treatment response predict long-term histological progression in primary biliary cirrhosis. Am J Gastroenterol 2010;105:2186-94. 13- Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet 2003;362:53-61. 14- Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med. 2005;353:1261-73. 15- Poupon RE, Lindor KD, Cauch-Dudek K, et al. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology 1997;113:884-90. 16- Silveira MG, Talwalkar JA, Angulo P, et al. Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes. Am J Gastroenterol 2007;102:1244-50. 17- Poupon RE, Poupon R, Balkau B. Ursodiol for the long-term treatment of primary biliary cirrhosis. The UDCA-PBC Study Group. N Engl J Med 1994;330:1342-7. 18- Zhang LN, Shi TY, Shi XH, et al. Early biochemical response to ursodeoxycholic acid and long-term prognosis of primary biliary cirrhosis: Results of a 14-year cohort study. Hepatology 2013;58:264-72. 19- Silveira MG, Brunt EM, Heathcote J, et al. American Association for the Study of Liver Diseases endpoints conference: design and endpoints for clinical trials in primary biliary cirrhosis. Hepatology 2010;52:349-59. 20- Lammert C, Juran BD, Schlicht E, et al. Biochemical response to ursodeoxycholic acid predicts survival in a North American cohort of primary biliary cirrhosis patients. J Gastroenterol 2014;49:1414-20. 21- Papastergiou V, Tsochatzis EA, Rodriquez-Peralvarez M, et al. Biochemical criteria at 1 year are not robust indicators of response to ursodeoxycholic acid in early primary biliary cirrhosis: results from a 29-year cohort study. Aliment Pharmacol Ther 2013;38:1354-64.
Details
| Subjects | Health Care Administration |
|---|---|
| Journal Section | Articles |
| Authors | |
| Publication Date | September 29, 2017 |
| Published in Issue | Year 2017 Volume: 16 Issue: 2 |
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