Erkin ÖZTAŞ, Erkan PARLAK, Selçuk DİŞİBEYAZ, Bülent ÖDEMİŞ, İsmail Hakki KALKAN, Diğdem Özer ETİK, Fatih Oğuz ÖNDER, Sabite KACAR, Nurgül ŞAŞMAZ

Primer sklerozan kolanjit benzeri kolanjiyograma sahip hastalarin değerlendirilmesi

Primer sklerozan kolanjit benzeri kolanjiyograma neden olan durumlar; safra yollarinda obstrüktif, toksik, iskemik patolojilere neden olan durumlar ve neoplastik hastaliklar olarak sayilabilir. Biz burada primer sklerozan kolanjit benzeri kolanjiyograma sahip 14 olguya ait verileri sunmayi amaçladik. Gereç ve Yöntem: Ocak 2006 Kasim 2010 yillari arasinda kliniğimizce takip edilen veya endoskopik retrograd kolanjiyopankreatografi ünitemize değerlendirme amaçli gönderilen primer sklerozan kolanjit benzeri kolanjiyograma sahip 14 hastaya ait bulgular ve seyir değerlendirildi. Bulgular: Hastalarin ortalama yaşi 56.36±15.93 idi ve 5'i (%36) kadindi. 12 hastada (%86) altta yatan malignite söz konusu iken (karaciğere metastaz yapmiş diğer organlara ait malignitesi olan 9 hasta ve primer hepatobiliyer sistem malignitesi olan 3 hasta) diğer 2 hastanin birinde tüberküloza bağli granülomatöz hepatit, diğerinde ise biliyer sistem tutulumunun da eşlik ettiği sistemik Exophiala dermatitidis enfeksiyonu söz konusuydu. Tüm hastalarin 11'inde (%78.5) temel bulgu ikter iken, bunlarin 5'inde iktere ağri ve/veya kaşinti eşlik ediyordu. İki hastada ise malignite söz konusu olup bunlarda yalnizca kan biyokimyasinda ve ultrasonografik değerlendirmede kolestaz lehine bulgular saptanmişti. Kolanjit ile başvuran yalnizca bir hasta mevcuttu ve bu hastaya sistemik mantar enfeksiyonu tanisi konulmuştu. Primer sklerozan kolanjit benzeri kolanjiyogram saptanan tüm hastalara endoskopik retrograd kolanjiyografi ile endoskopik biliyer drenaj tedavileri denenmişti. Malignitesi olan hastalarin 7'si (%58.3) endoskopik girişim sonrasi kolanjit ve buna sekonder gelişen septik komplikasyonlarla kaybedilirken, tüberküloz ve Exophiala dermatitidis enfeksiyonu olan hastalarda altta yatan hastaliğin medikal tedavisi ile kontrol kolanjiogramlarinda belirgin düzelme gözlendi. Sonuç: Sonuçlarimiza göre primer sklerozan kolanjit benzeri kolanjiyogramli hastalarin büyük çoğunluğunu, karaciğere metastaz yapmiş diğer organ malignitelerine sahip hastalar oluşturmaktadir. Karaciğere, primer sklerozan kolanjit benzeri kolanjiograma neden olacak şekilde metastaz yapmiş veya primer hepatobiliyer maligniteli olgularda, endoskopik retrograd kolanjiyografi araciliğiyla yapilacak endoskopik drenaj tedavileri tabloyu daha da kötüleştirebilir. Öncelikle drenaja en uygun safra kanalinin saptanmasi açisindan manyetik rezonans kolanjio- pankreatografik inceleme uygun olabilir.

Anahtar Kelimeler:

Primer sklerozan kolanjit benzeri kolanjiyogram, endoskopik tedavi, seyir

The evaluation of patients with primary sclerosing cholangitis-like cholangiogram

The conditions causing primary sclerosing cholangitis-like cholangiogram are pathologies of the biliary tract due to obstructive, ischemic or toxic events and neoplastic diseases. We aimed to discuss the data of 14 patients with primary sclerosing cholangitis-like cholangiogram in this report. Materials and Methods: In this report, the demographic, clinical and follow-up data of the patients with primary sclerosing cholangitis-like cholangiogram who were either followed in our clinic or were referred to our endoscopic retrograde cholangiopancreatography unit for further investigation between January 2006 and November 2010 are evaluated. Results: The mean age of the 14 patients was 56.36±15.93 years, and 5 (36%) were female. In 12 patients (86%), the underlying causes of the pathology were malignancies with liver metastases; in the remaining 2 patients, the causes were granulomatous hepatitis secondary to tuberculosis (n: 1) and systemic Exophiala dermatitidis infection with biliary involvement (n: 1). In 11 (78.5%) of the patients, the main symptom was icterus, and in 5 of these patients, pruritus and/or pain was accompanying. In 2 patients with malignancy, only biochemical analysis and ultrasonographic findings revealed cholestasis. One patient referred with cholangitis was diagnosed as systemic fungal infection. In all patients with primary sclerosing cholangitis-like cholangiogram, endoscopic biliary drainage was attempted by endoscopic retrograde cholangiography. After endoscopic approach, 7 (58.3%) of the patients with underlying malignancy died due to cholangitis and secondary septic complications, but in the patients with tuberculosis and Exophiala dermatitidis infection, follow-up cholangiograms revealed improvement with medical therapy of the underlying diseases. Conclusions: In patients with liver metastases causing primary sclerosing cholangitis-like cholangiogram, endoscopic drainage by endoscopic retrograde cholangiography worsens the clinical condition. In order to determine the optimal biliary duct suitable for drainage, magnetic resonance cholangiopancreatographic evaluation should be performed. The underlying disease should be treated whatever the cause.

Keywords:

Primary sclerosing cholangitis-like cholangiogram, endoscopic treatment, course,

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Lee YM, Kaplan MM. Primary sclerosing cholangitis. N Engl J Med 1995; 332: 924-33.
Talwalkar JA, Lindor KD. Primary sclerosing cholangitis. Inflamm Bowel Dis 2005; 11: 62-72.
Silveira MG, Lindor KD. Clinical features and management of primary sclerosing cholangitis. World J Gastroenterol 2008; 14: 3338-49.
Vitellas KM, El-Dieb A, Vaswani KK, et al. MR cholangiopancreatog- raphy in patients with primary sclerosing cholangitis: interobserver variability and comparison with endoscopic retrograde cholangi- opancreatography. AJR Am J Roentgenol 2002; 179: 399-407.
Ross AS, Kowdley KV. Sclerosing Cholangitis and Recurrent Pyoge- nic Cholangitis In: Feldman M, Friedman LS, Brant LJ (eds). Gastro- intestinal and liver Disease. Sleisenger and Fordtran’s. 9th Edition. Canada; 1153-70.
Terada T, Nakanuma Y. Intrahepatic cholangiographic appearance simulating primary sclerosing cholangitis in several hepatobiliary di- seases: a postmortem cholangiographic and histopathological study in 154 livers at autopsy. Hepatology 1995; 22: 75-81.
Altraif I, Lewall D. Primary sclerosing cholangitis-like cholangiog- raphic features in autopsied cirrhotic livers are not reproduced in clinical practice Hepatology 1997; 25: 780-1.
Haratake J, Kasai T, Makino H. Diffuse mucosal carcinoma of intra- hepatic and extrahepatic bile ducts including gallbladder. Pathol Int 2002; 52: 784-8.
Kirchner GI, Scherer MN, Obed A, et al. Outcome of patients with ischemic-like cholangiopathy with secondary sclerosing cholangitis after liver transplantation. Scand J Gastroenterol 2011; 46: 471-8. Epub 2010 Nov 30.
Deltenre P, Valla DC. Ischemic cholangiopathy Semin Liver Dis 2008; 28: 235-46.
Tombazzi C, Waters B, Ismail MK, et al. Sarcoidosis mimicking pri- mary sclerosing cholangitis requiring liver transplantation. Ann He- patol 2008; 7: 83-6.
Alazmi WM, McHenry L, Watkins JL, et al. Chemotherapy-induced sclerosing cholangitis: long-term response to endoscopic therapy. J Clin Gastroenterol 2006; 40: 353-7.
Özin Y, Parlak E, Kılıç ZM, et al. Sclerosing cholangitis-like changes in hepatobiliary tuberculosis. Turk J Gastroenterol 2010; 21: 50-3.
Oztas E, Odemis B, Kekilli M, et al. Systemic phaeohyphomycosis resembling primary sclerosing cholangitis caused by Exophiala der- matitidis. J Med Microbiol 2009; 58: 1243-6.
Vilgrain V, Erlinger S, Belghiti J, et al. Cholangiographic appearan- ce simulating sclerosing cholangitis in metastatic adenocarcinoma of the liver. Gastroenterology 1990; 99: 850-3.
Edmond HA, Craig JR. Neoplasms of the liver. In Schiff ER, Schiff L, (eds). Disease of the liver. 6th ed. Philedelphia: Lippincott; 1987: 1109-58.
Engel JJ, Trujillo Y, Spellberg M. Metastatic carcinoma of the bre- ast; a cause of obstructive jaundice. Gastroenterology 1980; 78; 132-5.
Gerber MA, Thung SN, Bodenheimer HC, et al. Characteristic his- tologic triad in liver adjacent to metastatic neoplasm. Liver 1986; 6: 85-8.