sdü tıp fak derg Medical Journal of Süleyman Demirel University 2602-2109 Süleyman Demirel Üniversitesi A case of primary biliary cirrhosis, CREST and Sjögren's syndrome overlap presented with severe esophageal variceal bleeding Şahin Mehmet Sarıtaş Ülkü Özkan Nurhayat Tunç Şevket Karahan Nermin 04 30 2009 14 2 38 42 04 30 2009 Copyright © 1994, Süleyman Demirel Üniversitesi Tıp Fakültesi Dergisi 1994 Süleyman Demirel Üniversitesi Tıp Fakültesi Dergisi

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

 özafageal varis kanamasý sýnýrlý tipte sistemik sklerozis primer biliyer siroz Raynaudfenomeni Sjögren sendromu Heseltine L, Turner IB, Fussey SM, et al. Primary biliary cirrhosis. Gastroenterology 1990; 99:1786. [PMID: 2227292] LeRoy EC, Black C, Fleischmajer R et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15:202–205. [PMID: 3361530] Czirják L, Zeher M, Nagy Z et al. Sjögren’s syndrome, primary biliary cirrhosis and overlap with scleroderma symptoms. J Intern Med 1993;233:427–428 (Letter; comment). [PMID: 8487013] Vitali C, Bombardieri S, Moustsopoulos HM, et al. Assessment of the European classification criteria for Sjögren’s. Ann Rheum Dis 1996; 55:116-121. [PMID: 8712861] Akimoto S, Ishikawa O, Muro Y et al. Clinical and immunological characterisation of patients with systemic sclerosis overlapping primary biliary cirrhosis: a comparison with patients with systemic sclerosis alone. J Dermatol 1999; 26:18–22. [PMID: 10063207] Culp KS, Fleming RC, Duffy J, et al. Autoimmune association in primary biliary cirrhosis. Mayo Clin Proc 1982; 57:365–70. [PMID: 6896227] Sasaki H, Inoue K, Higuchi K, et al. Primary biliary cirrhosis in Japan: national survey by the Subcommittees on Autoimmune Hepatitis. Gastroenterol Jpn 1985;2:476-485. [PMID: 4076730] Drosos AA, Andonopoulos AP, Costopoulos JS et al.) Sjögren’s syndrome in progressive systemic sclerosis. J Rheumatol 1988; 5: 965–968. [PMID: 3418646] Nagy G, Kovács J, Zeher M et al. Analysis of the oral manifestations of systemic sclerosis. Oral Surg Oral Med Oral Pathol 1994; 77:141–146. [PMID: 8139831] Murray-Lyon IM, Thompson RPH, Ansell ID, Williams cirrhosis. Br J Med 5448799] Reynolds TB, Dnison EK, Fraukl HD, et al. Primary biliary cirrhosis with scleroderma, Raynaud’s phenomenon and telangiectagia. A New syndrome. Am J Med 1971; 50:302–312. [PMID: 5553949] Kaplan MM. Primary biliary cirrhosis. N Engl J Med 1 9 8 7 ; 3 1 6 : 5 2 1 – 5 2 8 . [ P M I D : 3 5 4 3 6 8 2 ] Skopouli FN, Barbatis C, Moutsopoulos HM. Liver involvement in primary Sjogren’s syndrome. Br J Rheumatol 1994; 33:745-748. [PMID: 8055202] Uddenfeldt P, Danielsson A, Forssell A, et al. Features of Sjögren’s syndrome in patients with primary biliary cirrhosis. J Int Med 1991; 230:443-448. [PMID: 1940780] Hansen BU, Lindgren S, Eriksson S, et al. Clinical and immunologic features of Sjögren’s syndrome in patients with primary biliary cirrhosis with emphasis on focal sialadenitis. Acta Med Scand 1988; 224:611- 619. [PMID: 3207072] Butler P, Valle F, Burroughs AK. Mitochondrial antigens and antibodies in primary biliary cirrhosis. Postgrad Med J 1991; 67:790-797. [PMID: 1946125] Tojo J, Ohira H, Suzuki T, Takeda I, Shoji I, Kojima T, et all. Clinicolaboratory characteristics of patients with primary biliary cirrhosis associated with CREST symptoms. Hepatology Research 2002; 22:187–195. [PMID: 11882415] Remmel T, Piirsoo A, Koiveer A, et al. Clinical significance of different antinuclear ntibodies patterns in the course of primary biliary cirrhosis. Hepatogastroenterology 1996; 43:1135-1140. [PMID: 8908541] Oram S, Stokes W The heart in scleroderma. Br Heart J 1 9 6 1 ; 2 3 : 2 4 3 - 2 5 9 . [ P M I D : 1 3 7 3 1 0 8 7 ] D’Angelo WA, Fries JF, Masi AT, Shulman LE Pathological observations in systemic sclerosis (scleroderma). A study of fifty-eight cases and fifty- eight matched controls. Am J Med 1969; 46:428-440. [PMID: 5780367]