Organ Involvement in Systemic Sclerosis – From Early Detection and Assessment to Follow-up

Olga Kaloudi, Marco Matucci-Cerinic
European Musculoskeletal Review, 2011;6(1):38-43
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Abstract

Abstract
Systemic sclerosis (SSc) is a clinically heterogeneous disease and a variety of assessment methods have been proposed. The slow-moving progression of the disease and its extensive organ involvement have caused great difficulty in the choice of sensible methods to detect small variations of its course. Over the last few years, important progress has been made in diagnosis and therapy and new assessment methods have been proposed, but SSc remains one of the major challenges for clinicians.

Keywords
Systemic sclerosis, organ involvement, assessment, follow-up

Disclosure: The authors have no conflicts of interest to declare.
Received: 27 September 2010 Accepted: 26 January 2011 Citation: European Musculoskeletal Review, 2011;6(1):38–42
Correspondence: Olga Kaloudi, Department of Biomedicine, Division of Rheumatology, AOUC, Villa Monna Tessa, Viale Pieraccini 18, 50139 Firenze, Italy. E: olgakaloudi@hotmail.com

Systemic sclerosis (SSc) affects the microvascular, immune and connective tissue systems.1 In the past, clinical, laboratory and X-ray assessments were the main tools for the clinician to diagnose and evaluate organ involvement in SSc. Over the last few decades, new tools have been developed allowing important progress in the early diagnosis of SSc. However, SSc pathogenesis remains elusive and targeted therapy remains one of the major challenges for the clinician. Because of the extensive involvement of internal organs, e.g. the lung, heart, kidney and gastrointestinal (GI) system, patients require a comprehensive approach to manage the clinical features of the disease. Therefore, an early diagnosis of the disease and its organ involvement is of paramount importance in arriving at a timely and effective treatment, thus optimising the clinical outcome. In this article, we analyse the methods that are available to investigate organ involvement in SSc.

Skin Involvement
Skin involvement is frequently the major clinical feature, extending from mild oedema to extensive skin fibrosis and eventually to atrophy. The degree of skin involvement is an important measure and a predictor of mortality.2 Rodnan pioneered the assessment of cutaneous involvement using a semi-quantitative score to measure skin thickness by palpation, which correlated with the weight of skin punch biopsy scores.3 This score has subsequently been simplified (modified Rodnan skin thickness score [MRSS]) which has been reliable and sensitive to change.4 Therefore, the MRSS represents a surrogate marker of disease activity and severity in patients with SSc and has been validated as a useful clinical trial outcome measure. The limitations of the MRSS are the intra- and interobserver variability (12 and 25%, respectively),5 and the need for standardisation among centres when using skin scoring for clinical trials.6 The MRSS should be performed at least every six months to detect even small variations of skin involvement. Recently, ultrasound has been proposed as a non-invasive and reproducible method for measuring skin involvement in SSc.7 Moore et al.8 developed a 17-point dermal US scoring system that gave reliable results with low inter- and intra-observer variability. Our group of 70 SSc patients,9 divided into three subgroups according to the phase of the disease (oedematous, fibrotic and atrophic), found a significant correlation between dermal thickness and the clinical phase of skin involvement. These results suggest that ultrasound is reliable and reproducible for detecting skin thickness, making it a promising tool that may help the physician to understand not only the phase of the disease, but also the thickness of the dermis as an outcome measure. More recently, a novel technique that can be used to assess tissue elasticity, namely ultrasound elastography, has been proposed and implemented but further investigations are needed to confirm the validity of the criteria.10

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