Mehmet ŞAHİN, Ülkü SARITAŞ, NURHAYAT ÖZKAN SEVENCAN, Şevket Ercan TUNÇ, NERMİN KARAHAN

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

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:

Karaciğer sirozu, safra, Raynaud hastalığı, CREST Sendromu, Kadın, Orta yaşlı, Skleroderma, sistemik, Sjogren sendromu

Ciddi özafagus varis kanaması ile başvuran, CREST ve Sjögren sendromu ile çakışma yapan bir primer biliyer siroz vakası

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.

Keywords:

Liver Cirrhosis, Biliary, Raynaud Disease, CREST Syndrome, Female, Middle Aged, Scleroderma, Systemic, Sjogren's Syndrome,

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