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The Cytoplasmic Dot Staining Pattern Is Detected in a Subgroup of Patients with Primary Biliary Cirrhosis
DONALD B. BLOCH, JIANG HONG YU, WEI-HONG YANG, FIONA GRAEME-COOK, KEITH D. LINDOR, ANJALI VISWANATHAN, KENNETH D. BLOCH, and AYAKO NAKAJIMA
ABSTRACT. Objective. To determine the clinical significance of the cytoplasmic dot anti-"nuclear" antibody (ANA) staining pattern. Methods. We describe a patient with fatigue, arthralgias, elevated serum transaminase, and antibodies staining 5–20 cytoplasmic dots in HEp-2 cells. A liver biopsy revealed the presence of Stage III primary biliary cirrhosis (PBC). Using 2-color immunofluorescence, we determined the relationship between the cytoplasmic dot staining pattern and that produced by antibodies directed against the GW182 component of mRNA processing bodies. To determine the prevalence of the cytoplasmic dot staining pattern in patients with PBC, sera from 493 patients were tested for antibodies producing this staining pattern. Results. Antibodies in our patient's serum colocalized with anti-GW182 antibodies in cytoplasmic dots, but did not react with recombinant GW182, suggesting that they were directed against an additional component(s) of these structures. The cytoplasmic dot staining pattern was observed in 21 of 493 (4.3%) patients with PBC. In comparison, this staining pattern was not produced by serum from 248 patients with other autoimmune diseases. Conclusion. A subset of patients with PBC have autoantibodies that produce the cytoplasmic dot staining pattern. These antibodies react with one or more as yet unidentified components of the mRNA processing body. Appreciation of the clinical significance of the cytoplasmic dot staining pattern may assist in appropriate diagnosis and treatment of patients with PBC. (J Rheumatol 2005; 32:477-83) Key Indexing Terms:
ANTINUCLEAR ANTIBODIES
From the Departments of Medicine and Pathology, Harvard Medical School, Boston, Massachusetts; the Center for Immunology and Inflammatory Diseases and the Cardiovascular Research Center of the General Medical Services, Massachusetts General Hospital, Boston, Massachusetts; and the Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA. Supported by grants from the Arthritis Foundation (DBB), the National Institutes of Health (DK-051179 to DBB, HL-57172 to KDB), and a pilot grant from the Harvard Clinical Nutrition Center (DK-40561). K.D. Bloch was supported by an Established Investigator Award and D.B. Bloch by an Established Investigator Grant from the American Heart Association. D.B. Bloch, MD, Assistant Professor of Medicine; J.H. Yu, MD, Research Fellow; W-H. Yang, MD, Research Fellow; F. Graeme-Cook, MB, BCh, Assistant Professor of Pathology; K.D. Bloch, MD, Associate Professor of Medicine; A. Nakajima, MD, Research Fellow, Harvard Medical School; K.D. Lindor, MD, Professor of Medicine, Mayo Clinic College of Medicine; A. Viswanathan, MD, Clinical Fellow, St. Vincent's Catholic Medical Center, Staten Island, NY. Address reprint requests to Dr. D.B. Bloch, Massachusetts General Hospital-East, CNY 149 13th Street, Charlestown, MA 02129. E-mail: bloch@helix.mgh.harvard.edu Submitted April 26, 2004; revision accepted October 13, 2004. |