BRIEF REPORT Liver autoantibodies in patients with scleroderma Thelma L. Skare & Renato M. Nisihara & Osvaldo Haider & Pedro M. Azevedo & Shirley R. R. Utiyama Received: 5 January 2010 / Revised: 13 August 2010 / Accepted: 26 September 2010 # Clinical Rheumatology 2010 Abstract Association between autoimmune liver diseases and scleroderma has been described. The purpose of this study was to study the prevalence of antimithocondrial antibody (AMA), antismooth muscle antibodies (SMA), and liver- kidney-microsomal (LKM-1) autoantibody in a cohort of 63 scleroderma patients and 100 healthy controls. The autoanti- bodies AMA, SMA, and LKM were determined by indirect immunofluorescence. Patients' charts were reviewed for demographic data, scleroderma form, and clinical and anti- nuclear antibody profile, aiming a comparison between patients with and without liver autoantibodies. Nine patients (14.3%) were positive for at least one of the liver autoanti- bodies; only one patient had both AMA and SMA positive. Antibody SMA was positive in 6.4% (4/63) patients; AMA was present in 9.52% (6/63) of them; none were positive to LKM-1. In the control group just one patient (1%) was SMA positive; the other autoantibodies were negative. There is an increased prevalence of liver autoantibodies in patients with scleroderma than in control population. These patients should be carefully followed for liver dysfunction. Keywords Anticentromere antibody . Antimitochondrial antibody . Antismooth muscle antibody . Scleroderma . Systemic sclerosis Introduction Systemic scleroderma (SSc) is a chronic autoimmune disease that has been classified in two forms: localized (l-SSc) and diffuse (d-SSc), according to the distribution of skin involvement [1]. Although the cardinal feature of this disease is skin sclerosis, there is a potential for involvement of others organs [1]. Gastrointestinal symptoms are common in systemic sclerosis, most of them due to motility impairment. Liver disease is considered rare in SSc [1] however some authors have studied this association. Abu Shakra et al. [2] described that four of their 264 patients (1.5%) with SSc had chronic liver disease. In a postmortem study, D'Angelo et al. [3] found histological damage in the liver of 8.8% of their 57 SSc cases. Nevertheless, it is not uncommon that patients with SSc develop associated autoimmune liver disease such as primary biliary cirrhosis (PBC) or autoimmune hepatitis (AIH) [1, 4–6]. Other possible causes of liver damage in sclerodermic patients are nodular regenerative hyperplasia [1, 7], portal hyperten- sion [8], spontaneous liver rupture, liver infarction, and hepatic duct obstruction related to vasculitis [1]. Amongst all these forms of hepatic involvement, PBC is the commonest. Pope et al. studying 61 sleroderma patients found that 13% had antimitocondrial antibodies (AMA), two of them with PBC [9]. Hudson et al., studying 719 Colombian and Canadian patients with SSc found a prevalence of 15.4% of PBC [10]. T. L. Skare : R. M. Nisihara : O. Haider : P. M. Azevedo Department of Medicine, Evangelical University, Curitiba, Paraná, Brazil R. M. Nisihara : S. R. R. Utiyama Laboratory of Immunopathology, Clinical Hospital, Federal University of Paraná, Curitiba, Paraná, Brazil S. R. R. Utiyama (*) Laboratório de Imunopatologia, Setor de Ciências da Saúde, R. Padre Camargo, 280, 80060-240 Curitiba, Paraná, Brazil e-mail: shirley@ufpr.br Clin Rheumatol DOI 10.1007/s10067-010-1586-0