A case of primary biliary cirrhosis, CREST and Sjögren's syndrome overlap presented with severe esophageal variceal bleeding

Özet

SüleymanDemirel Üniversitesi TIP FAKÜLTESİ DERGİSİ: 2007 Haziran; 14(2) A case of primary biliary cirrhosis, CREST and Sjögren's syndrome overlap presented with severe esophageal variceal bleeding Mehmet Sahin*, Ulku Sarıtas**, Nurhayat Ozkan***, Sevket Ercan Tunc*, Nermin Karahan**** *Department of Internal Medicine, Division of Rheumatology, Suleyman Demirel University, Faculty of Medicine, Isparta, Turkey **Department of Internal Medicine, Division of Gastroenterology, Suleyman Demirel University, Faculty of Medicine, Isparta, Turkey ***Department of Internal Medicine, Suleyman Demirel University, Faculty of Medicine, Isparta, Turkey ****Department of Pathology, Suleyman Demirel University, Faculty of Medicine, Isparta, Turkey Özet Ciddi Özafagus Varis Kanaması İle Başvuran, CREST Ve Sjögren Sendromu İle Çakışma Yapan Bir Primer Biliyer Siroz Vakası Primer biliyer sirozda karaciğer hasarının mekanizması otoimmüniteye ait deliller olmasına ve çeşitli otoimmün hastalıklarla çakışma yapabilmesine rağmen tam olarak bilinmemektedir. Burada masif hematemez yakınması ile başvuran 52 yaşındaki bir kadın hastayı takdim ediyoruz. Hastaya yapılan üst gastrointestinal sistem endoskopisinde rüptüre özafageal varisler tespit edildi. İki kez başarılı bir şekilde hastaya skleroterapi yapıldı. Sonrasında hastada belirgin asit ortaya çıktı. Buna rağmen hasta tedaviden sonraki 3.günde kendisini daha iyi hissediyordu. Hastaya klinik bulgular, yüksek düzeydeki alkalen fosfataz ve immünglobülin M düzeyleri, antimitokondrial antikor ve antisentromer antikor pozitifliği ve minor tükrük bezi biyopsisindeki pozitif sonuçlar ışığı altında sınırlı tipte sistemik skleroz ve Sjögren sendromu ile çakışma yapan primer biliyer siroz tanısı konuldu. Diğer bir bulgu ise mitral stenoz idi. Hastaya klinik bulguların semptomatik tedavisi için propranolol ve primer biliyer siroz tedavisine yönelik ursodeoksikolik asit verildi. Anahtar kelimeler: özafageal varis kanaması, sınırlı tipte sistemik sklerozis, primer biliyer siroz, Raynaud fenomeni, Sjögren sendromu. Abstract Despite the evidences indicate that it can be autoimmune in origin the exact mechanism of liver damage in primary biliary cirrhosis is unknown, and may overlap with a variety of autoimmune-mediated diseases. Here, we report a 52-year-old female patient presented with massive hematemesis. Upper gastrointestinal endoscopy showed ruptured esophageal varices. Sclerotherapy was applied two times successfully. The patient developed manifest ascite afterwards. However, she felt better three days after the treatment. She was diagnosed as overlapping of limited cutaneous systemic sclerosis, Sjögren's syndrome and primary biliary cirrhosis based on clinical features, high levels of alkalen phosphatase, positive anti-mitochondrial and anticentromer antibodies, high titers of serum immunoglobulin M and positive minor salivary gland biopsy findings. Another finding was mitral stenosis. The patient was adequately managed symptomatically by propranolol treatment for clinical findings and also ursodeoxycholic acid for primary biliary cirrhosis. Key words: esophageal variceal bleeding, limited systemic sclerosis, primary biliary cirrhosis, Raynaud's phenomenon, Sjögren's syndrome