Skin fibrosis, internal organ involvement and auto antibodies in systemic sclerosis: longitudinal development and impact on survival

Research output: ThesisDoctoral Thesis (compilation)


Systemic sclerosis (SSc) is a connective tissue disease with a highly variable prognosis. Since new treatment strategies are emerging, there is a need for prognostic markers in order to help allocate patients to the optimal treatment. When comparison is made to the general population, there is an increased mortality rate in this study, both in the series as a whole and in the subgroups of men and women, and of the diffuse and limited skin involvement variations of the disease. Five- and 10-year survival rates are 86% and 69% respectively. There is a need for early detection, since pulmonary complications, related either to interstitial or vascular lung disease, account for half of the deaths. Treating patients suffering interstitial lung disease with cyclophosphamide did not increase the risk of developing malignancies. Auto antibodies serve as valuable indicators in predicting organ involvement and death. A nucleolar immunofluorescence pattern, anti histone antibodies, male gender, diffuse skin involvement, and the involvement of the kidneys, the heart or the interstitial or vascular involvement of the lungs, are all associated with an increased risk of death. High fibroblast production of versican and biglycan in cell culture are associated with increasing skin echogenicity, when measured by high-frequency (20 MHz) ultrasound, and may predict a more progressive skin involvement. Biglycan production was greater in the earlier stages of the disease and may be involved in the initial steps of the fibrotic process. Pulmonary arterial hypertension, when measured by Doppler echocardiography, is common and progressive in SSc. Age and the presence of interstitial lung disease increase the risk of developing pulmonary arterial hypertension.


Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Rheumatology and Autoimmunity


  • muscle system, rheumatology locomotion, Skelett, muskelsystem, reumatologi, Skeleton, proteoglycan, pulmonary hypertension, organ involvement, auto antibodies, survival, systemic sclerosis, prognosis
Original languageEnglish
Awarding Institution
Supervisors/Assistant supervisor
  • [unknown], [unknown], Supervisor, External person
Award date2003 Dec 6
  • Roger Hesselstrand, Department of Rheumatology, Lund University Hospital,
Print ISBNs91-628-5817-3
Publication statusPublished - 2003
Publication categoryResearch

Bibliographic note

Defence details Date: 2003-12-06 Time: 10:15 Place: the Department of Rheumatology External reviewer(s) Name: Denton, Christopher Title: [unknown] Affiliation: PhD FRCP, Royal Free and University College Medical School, London, UK --- Article: I. Hesselstrand R, Scheja A, Åkesson A. Mortality and causes of death in a Swedish series of systemic sclerosis patients. Ann Rheum Dis 1998; 57:682-686.II. Hesselstrand R, Scheja A, Shen GQ, Wiik A, Åkesson A. The association of antinuclear antibodies with organ involvement and survival in systemic sclerosis. Rheumatology 2003; 42:534-540.III. Hesselstrand R, Westergren-Thorsson G, Scheja A, Wildt M, Åkesson A. The association between changes in skin echogenicity and the fibroblast production of biglycan and versican in systemic sclerosis. Clin Exp Rheum 2002; 20:301-308.IV. Hesselstrand R, Ekman R, Eskilsson J, Isaksson A, Scheja A, Öhlin AK, Åkesson A. The longitudinal development of pulmonary arterial systolic pressure in 227 consecutive patients with systemic sclerosis 1992-2001. Manuscript.