Classification of systemic sclerosis in the Japanese population based on rapid progression of skin thickening

Abstract

In general, patients with systemic sclerosis (SSc) have been classified in two clinical subsets (diffuse and limited) based on the extent of skin thickening, and the extent of skin fibrosis is closely related to the severity of organ involvement and mortality rates. In addition, there is a rapid progression of skin thickening in diffuse cutaneous SSc (dcSSc). In this study, we classified a novel subset of SSc based on the rate of skin thickening progression, and evaluated the relationship of the subset to disease severity and laboratory markers. Thirty-three patients with dcSSc were included in the study. Participants who had a modified Rodnan's total skin thickness score higher than 15 points within a year from the first symptoms were defined as having rapidly progressive SSc (RPSSc; group 1), while all the other dcSSc patients were defined as having non-RPSSc (group 2). The frequencies of interstitial lung disease and renal crisis were significantly higher in group 1 than in group 2. The frequencies of anti-topoisomerase I antibody (anti-topo I) and anti-SS-A antibody were significantly higher in group 1 than in group 2. Conversely, the frequency of anticentromere antibody was significantly higher in group 2 than in group 1. This study demonstrates the clinical significance of a new subset of SSc (defined as RPSSc), which is characterized by a markedly rapid progression of skin thickening. This new classification may be useful for predicting the prognosis of SSc at an early stage, so that patients who should receive aggressive therapy with immunosuppressants might be identified.

The degree of skin involvement identifies distinct lung disease outcomes and survival in systemic sclerosis

“…3 Subsequently, numerous proposals for classification systems that divide scleroderma into disease subtypes based on the extent of skin involvement have been published. [4][5][6][7] Studies comparing their clinical utility have generated conflicting results, often due to a relatively low number of patients investigated. [8][9][10][11] For this reason, a dichotomous classification system has prevailed.…”

Malnutrition Is Common in Systemic Sclerosis: Results from the Canadian Scleroderma Research Group Database

“…Gastrointestinal (GI) involvement in patients with systemic sclerosis (SSc) is common, and diffuse bowel involvement may result in bacterial overgrowth and malabsorption [1][2][3][4][5] . Prokinetic agents, antibiotics, and parenteral nutrition may be required [6][7][8][9][10][11] , but although malnutrition might be an expected consequence of these features, a systematic approach to assessment of nutritional status in patients with SSc has been undertaken infrequently, and then only in small numbers of patients 12,13 .…”

Clinical Features and Risk Factors of Rapidly Progressive Systemic Sclerosis in a Single Center in China: Anti-RNA Polymerase III Antibodies as a Predictor

“…Some SSc patients show quick progression and early internal organ involvement [5], what reduces the effectiveness of conventional treatment. Usually, these SSc patients show poor prognosis and a high mortality [5][6][7][8]. It is important to distinguish patients at risk of progression through by discovering specific predictors and biomarkers.…”