title: Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl.
abstract: 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.
authors.affiliation: Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD.
authors.forename: Joanna
authors.initials: J
authors.lastname: Latuśkiewicz-Potemska
authors.affiliation: Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD.
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Zygmunt
authors.affiliation: Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Biernacka-Zielińska
authors.affiliation: Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD.
authors.forename: Jerzy
authors.initials: J
authors.lastname: Stańczyk
authors.affiliation: Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD.
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Smolewska
keywords: Raynaud's phenomenon; mixed connective tissue disease; sclerodactyly; trophic damages of fingers