Latuśkiewicz-Potemska Joanna, Zygmunt Agnieszka, Biernacka-Zielińska Małgorzata, Stańczyk Jerzy, Smolewska Elżbieta
Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD.
Postepy Dermatol Alergol. 2013 Oct;30(5):329-36. doi: 10.5114/pdia.2013.38365. Epub 2013 Oct 30.
Mixed connective tissue disease (MCTD) is a systemic inflammatory disease affecting connective tissue with the underlying autoimmunological mechanism. The core of MCTD is an appearance of symptoms of several other inflammatory diseases of connective tissue - systemic lupus erythematosus, systemic scleroderma, poly- or dermatomyositis, rheumatoid arthritis at the same time, accompanied by a high level of anti-ribonucleoprotein antibodies (anti-U1RNP). The disease was described more than 40 years ago by Sharp et al. During recent years, many efforts to better understand clinical and serological features of MCTD have been made. Diagnosis of MCTD can be difficult. Obligatory international diagnostic criteria are required to be fulfilled. Several versions of such criteria have been proposed, but the most widely used one was described by Kasukawa. There is no consensus about treatment - a choice of drugs depends on symptoms. We present a case of a 10-year-old girl with sclerodactyly and trophic damages of fingers accompanied by symptoms of Raynaud's phenomenon. After an almost 2-year course of the disease, a diagnosis of MCTD has been established.
混合性结缔组织病（MCTD）是一种影响结缔组织的系统性炎症性疾病，其潜在机制为自身免疫。MCTD的核心是同时出现其他几种结缔组织炎症性疾病——系统性红斑狼疮、系统性硬化症、多肌炎或皮肌炎、类风湿关节炎的症状，并伴有高水平的抗核糖核蛋白抗体（抗U1RNP）。该疾病于40多年前由夏普等人首次描述。近年来，人们为更好地了解MCTD的临床和血清学特征付出了诸多努力。MCTD的诊断可能具有挑战性。必须满足强制性的国际诊断标准。已经提出了多个版本的此类标准，但应用最广泛的是由加川描述的标准。关于治疗尚无共识——药物的选择取决于症状。我们报告一例10岁女孩，患有手指硬皮病和营养性损害，并伴有雷诺现象的症状。在病程近2年后，确诊为MCTD。
