Collagen and elastin degradation products as potential activity markers of scleroderma

Authors: R. Bečvář;  J. Štork 1;  H. Hulejová;  M. Braun;  I. Zatloukalová 2;  P. Zatloukal 3;  P. Jansa 4;  T. Paleček 4
Authors‘ workplace: Revmatologický ústav, Klinika revmatologie 1. lékařská fakulta Univerzity Karlovy, Praha ;  Dermatovenerologická klinika, Všeobecná fakultní nemocnice a 1. lékařská fakulta Univerzity Karlovy, Praha 1;  Nestátní pneumologické zařízení, Poliklinika Kartouzská, Praha 2;  Klinika pneumologie a hrudní chirurgie, Fakultní nemocnice Na Bulovce a 3. lékařská fakulta Univerzity Karlovy a IPVZ, Praha 3;  2. interní klinika kardiologie a angiologie Všeobecné fakultní nemocnice a 1. lékařské fakulty Univerzity Karlovy, Praha 4
Published in: Čes. Revmatol., 17, 2009, No. 1, p. 23-29.
Category: Original Papers


Systemic (SSc) and localized scleroderma (LSc) do not have reliable activity markers. In both conditions an excessive deposition of collagen and elastin fibrils occurs in the skin and subcutis, and in SSc additionally in vessel walls and in majority of parenchymatous organs.

The aim of this study was to assess the degradation of collagen type I, elastin excretion and proinflammatory cytokines in SSc and LSc compared with patients with psoriasis vulgaris (PsV) and healthy controls (HC).

Patients and methods.
Total 91 individuals were examined – 24 with SSc, 16 with LSc, and two control groups - 37 patients with PsV and 14 blood donors. Urinary excretion of pyridinoline (U-PD) and deoxypyridinoline (U-DPD) were measured using sensitive isocratic HPLC method. Urinary excretion of soluble elastin (U-SE) ) was evaluated by quantitative immunoprecipitation method, serum levels of interleukin-6 (IL-6) and soluble interleukin-2 receptor (IL-2R) were assayed using commercial ELISA kits. All measurements were performed at entry and after one year.

U-PD levels were the highest at entry and after one year in SSc group. At entry U-PD concentrations in SSc and LSc groups were increased compared with HC (p < 0.0001, and p < 0.0001 respectively). SSc patients had also a higher levels compared with PsV group (p < 0.001). At entry U-DPD levels in SSc group were increased compared with HC and LSc (p = 0.006, and p < 0.001 respectively). U-SE was the highest at entry in PsV and one year later in SSc group. At entry U-SE in PsV group were increased compared with HC (p < 0.001). After a year U-SE in SSc and PsV patients were higher compared with HC (for both p = 0.001). IL-6 serum levels were increased at entry in SSc, LSc and PsV groups compared with HC (p < 0.0001, p < 0.001, and p = 0.004 respectively), after one year only SSc had increased levels compared with HC (p < 0.001). IL-2R serum levels did not differ among the studied groups at any time.

The increased markers of collagen and elastin turnover in SSc and LSc reflect the active fibrotic process in the diseases and are accordance with the published data. High elastin levels in PsV group are surprising, and we have so far no explanation for this result.

Key words:
systemic sclerosis, morphea, psoriasis, collagen, elastin, activity


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