OBJECTIVES: In SSc, diagnosis and classification is based mainly on skin sclerosis. Herein, we investigated in a large multicentre cohort, to what extent skin sclerosis reflects organ involvement and additional clinical symptoms. METHODS: A total of 1200 SSc patients from the register of the German Systemic Sclerosis Network (DNSS), classified as either lcSSc or dcSSc, were analysed for their serological characteristics, clinical symptoms and organ manifestations in relation to skin involvement measured by the modified Rodnan skin score (mRSS). RESULTS: SSc patients with different mRSS did not differ significantly in their disease duration and in most of the clinical symptoms. They showed a similar distribution of most organ manifestations such as pulmonary arterial hypertension as well as cardiac, renal and nervous system involvement. More severe skin thickening was found to be associated with pulmonary fibrosis and gastrointestinal symptoms, as well as with digital ulcers and musculoskeletal involvement. CONCLUSIONS: In patients with SSc, potentially life-threatening complications and clinical symptoms with high impact on the quality of life occur independently from the extent of skin sclerosis. The diagnosis in SSc patients with a low mRSS could be missed or they could be insufficiently treated.
OBJECTIVES: In SSc, diagnosis and classification is based mainly on skin sclerosis. Herein, we investigated in a large multicentre cohort, to what extent skin sclerosis reflects organ involvement and additional clinical symptoms. METHODS: A total of 1200 SSc patients from the register of the German Systemic Sclerosis Network (DNSS), classified as either lcSSc or dcSSc, were analysed for their serological characteristics, clinical symptoms and organ manifestations in relation to skin involvement measured by the modified Rodnan skin score (mRSS). RESULTS: SSc patients with different mRSS did not differ significantly in their disease duration and in most of the clinical symptoms. They showed a similar distribution of most organ manifestations such as pulmonary arterial hypertension as well as cardiac, renal and nervous system involvement. More severe skin thickening was found to be associated with pulmonary fibrosis and gastrointestinal symptoms, as well as with digital ulcers and musculoskeletal involvement. CONCLUSIONS: In patients with SSc, potentially life-threatening complications and clinical symptoms with high impact on the quality of life occur independently from the extent of skin sclerosis. The diagnosis in SSc patients with a low mRSS could be missed or they could be insufficiently treated.
Authors: M Aringer; U Müller-Ladner; H Burkhardt; J H W Distler; O Distler; W B Graninger; C Günther; N Hunzelmann; H Kiener; M Sticherling; C Sunderkötter; U A Walker; G Riemekasten Journal: Z Rheumatol Date: 2015-03 Impact factor: 1.372
Authors: Michael D Roth; Chi-Hong Tseng; Philip J Clements; Daniel E Furst; Donald P Tashkin; Jonathan G Goldin; Dinesh Khanna; Eric C Kleerup; Ning Li; David Elashoff; Robert M Elashoff Journal: Arthritis Rheum Date: 2011-09
Authors: Stefan Schanz; Jörg Henes; Anja Ulmer; Ina Kötter; Gerhard Fierlbeck; C D Claussen; Marius Horger Journal: Eur Radiol Date: 2012-07-29 Impact factor: 5.315
Authors: Karen Au; Maureen D Mayes; Paul Maranian; Philip J Clements; Dinesh Khanna; Virginia D Steen; Donald Tashkin; Michael D Roth; Robert Elashoff; Daniel E Furst Journal: Arthritis Care Res (Hoboken) Date: 2010-12 Impact factor: 4.794