Primer sklerozan kolanjiti taklit eden otoimmün kolanjiyopati: Vaka sunumu

Öz

Otoimmün kolanjiyopati veya immünglobulin G4 ilişkili sklerozan kolanjit immün sistem ilişkili bir kolanjit tipidir. Otoimmün pankreatit sıklıkla bu duruma eşlik eder; bununla beraber hastaların çok az bir yüzdesinde otoimmün kolanjiyopati tek başına da görülebilir. 69 yaşında kadın hasta kliniğimize halsizlik, kaşıntı ve sarılıkla başvurdu. Endoskopik retrograd kolanjiopankreatografi de intrahepatik safra yollarında çok sayıda darlık ve dilatasyonlarla beraber ana koledok distalde darlığa rastlandı. Sklerozan kolanjitin diğer nedenlerini ekarte edebilmek için hastadan immünglobulin G4 çalışıldı ve sonuç 1.200 mg/dl olarak geldi. Bu sonuçla otoimmün kolanjiyopati tanısı kondu. Otoimmün kolanjiyopati intrahepatik ve/veya extrahepatik safra yollarında darlık olan hastalarda ayırıcı tanıda mutlaka akılda tutulmalıdır. Şüphe edilen hastalarda immünglobulin G4 tarama amaçlı kullanılabilir. Otoimmün kolanjiyopatinin tanı ve tedavisi hastaları ilerleyen zamanda siroz veya gereksiz cerrahiden koruyacaktır.

Anahtar Kelimeler

• Otoimmün kolanjit,primer sklerozan kolanjit

Autoimmune cholangiopathy mimicking primary sclerosan cholangitis: A case report

Öz

Autoimmune cholangiopathy or IgG4-related sclerosan cholangitis is an immune- mediated type of cholangitis. Autoimmune pancreatitis is frequently associated with this condition. However, in a minority of patients, autoimmune cholangiopathy exist alone. A 69- year-old female patient was admitted to our hospital with fatigue, pruritus, and jaundice. There were multiple strictures and dilations in the intrahepatic biliary tree with stricture in the distal part of the common bile duct. To exclude other causes of sclerosan cholangitis, IgG4 was studied. The IgG4 titer was 1200 mg/dl; therefore, the diagnosis was autoimmune cholangiopathy. Autoimmune cholangiopathy must be investigated in patients with biliary strictures in either the intrahepatic or extrahepatic bile ducts. IgG4 can be used to screen for autoimmune cholangitis. Diagnosis and treatment of this condition prevents patients from cirrhosis and unnecessary surgery.

Anahtar Kelimeler

• Autoimmune cholangiopathy,primary sclerosan cholangitis

Kaynakça

1. 1-Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732-8. 2- Nakazawa T, Ikeda Y, Kawaguchi Y, et al. Isolated intrapancreatic IgG4-related sclerosing cholangitis. World J Gastroenterol 2015;21:1334-43. 3- Navaneethan U, Gutierrez NG, Jegadeesan R, et al. IgG4 levels in bile for distinguishing IgG4-associated cholangiopathy from other biliary disorders: A single blinded pilot study. Clin Endosc 2014;47:555-9. 4- Stone JH, Khosroshahi A, Deshpande V, et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 2012;64:3061-7. 5-Ohara H, Okazaki K, Tsubouchi H, et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 2012;19:536-42. 6-Tanaka A, Tazuma S, Okazaki K, et al. A nationwide survey for primary sclerosing cholangitis and IgG4-related sclerosing cholangitis in Japan. J Hepatobiliary Pancreat Sci 2014;21:43-50. 7- Zaydfudim VM, Wang AY, de Lange EE, et al. IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Gut Liver 2015;9:556-60. 8- Nakazawa T, Naitoh I, Hayashi K, et al. Diagnosis of IgG4-related sclerosing cholangitis. World J Gastroenterol 2013;19:7661-70.