System manifestations of primary biliary cirrhosis

• Published in: Klinicheskaia meditsina Vol. 84; no. 12; p. 42
• Main Authors: Burnevich, E Z, Lopatkina, T N
• Format: Journal Article
• Language: Russian
• Published: Russia (Federation) 2006
• Subjects: Adult; Aged; Biomarkers - blood; Biopsy; Bronchoscopy; Diagnosis, Differential; Endoscopy, Gastrointestinal; Female; Follow-Up Studies; Humans; Incidence; Liver Cirrhosis, Biliary - blood; Liver Cirrhosis, Biliary - complications; Liver Cirrhosis, Biliary - diagnosis; Male; Middle Aged; Nephritis, Interstitial - complications; Nephritis, Interstitial - diagnosis; Nephritis, Interstitial - epidemiology; Prognosis; Pulmonary Fibrosis - complications; Pulmonary Fibrosis - diagnosis; Pulmonary Fibrosis - epidemiology; Rheumatoid Factor - blood; Risk Factors; Severity of Illness Index; Sjogren's Syndrome - complications; Sjogren's Syndrome - diagnosis; Sjogren's Syndrome - epidemiology; Thyroiditis, Autoimmune - complications; Thyroiditis, Autoimmune - diagnosis; Thyroiditis, Autoimmune - epidemiology; Tomography, X-Ray Computed
• ISSN: 0023-2149

Primary biliary cirrhosis (PBC) is characterized by high frequency of systemic extrahepatic manifestations (EHM), which often precede the development of full clinical picture of PBC and play the leading part in the clinical course of the disease, sometimes determining its prognosis. The examination of 145 PBC patients (including two men) found a frequency of EHM of 72. 4%. The majority of EHM were caused by delayed-type hypersensitivity reactions, such as Sjogren syndrome, fibrosing alveolitis, autoimmune thyroiditis, tubulointerstitial nephritis, pulmonary granulomatosis/sarcoidosis, systemic scleroderma, rheumatoid arthritis, and ulcerative colitis; immunocomplex pathology including vasculites involving blood vessels of different caliber and localization and polyneuropathy was rarer. In 24.1% of PBC patients, system EHM manifestations were the first clinical signs of the disease. In 62.8% of patients with EHM their different combinations were registered. The most frequent one was a combination of Sjogren syndrome, fibrosing alveolitis, and tubulointerstitial nephritis, which was found in 16.2% of the patients. 6 7% of the patients had a combination of four EHM - Sjogren syndrome, fibrosing alveolitis, tubulointerstitial nephritis, and autoimmune thyroiditis. A long duration of PBC (more than five years from the debut), stage IV of the disease, and the presence of the rheumatoid factor in blood serum, were risk factors of the development of system PBC manifestations. In 20% of EHM patients their symptoms prevailed in the clinical picture, thus determining the severity of the condition. Three patients died of system EHM of PBC (systemic scleroderma, pulmonary granulomatosis). Thus, PBC diagnostics must be performed with taking into account system EHM found in this category of patients.