Early systemic sclerosis: marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markersReport as inadecuate




Early systemic sclerosis: marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markers - Download this document for free, or read online. Document in PDF available to download.

Arthritis Research and Therapy

, 15:R63

First Online: 29 May 2013Received: 06 November 2012Revised: 20 February 2013Accepted: 29 May 2013

Abstract

IntroductionEarly systemic sclerosis SSc is characterized by Raynaud-s phenomenon together with scleroderma marker autoantibodies and-or a scleroderma pattern at capillaroscopy and no other distinctive feature of SSc. Patients presenting with marker autoantibodies plus a capillaroscopic scleroderma pattern seem to evolve into definite SSc more frequently than patients with either feature. Whether early SSc patients with only marker autoantibodies or capillaroscopic positivity differ in any aspect at presentation is unclear.

MethodsSeventy-one consecutive early SSc patients were investigated for preclinical cardiopulmonary alterations. Out of these, 44 patients and 25 controls affected by osteoarthritis or primary fibromyalgia syndrome were also investigated for serum markers of fibroblast carboxyterminal propeptide of collagen I, endothelial soluble E-selectin and T-cell soluble IL-2 receptor alpha activation.

ResultsThirty-two of the 71 patients 45.1% had both a marker autoantibody and a capillaroscopic scleroderma pattern subset 1, 16 patients 22.5% had only a marker autoantibody subset 2, and 23 patients 32.4% had only a capillaroscopic scleroderma pattern subset 3. Patients with marker autoantibodies n = 48, 67.6% had a higher prevalence of impaired diffusing lung capacity for carbon monoxide P = 0.0217 and increased serum levels of carboxyterminal propeptide of collagen I P = 0.0037, regardless of capillaroscopic alterations. Patients with a capillaroscopic scleroderma pattern n = 55, 77.5% had a higher prevalence of puffy fingers P = 0.0001 and increased serum levels of soluble E-selectin P = 0.0003 regardless of marker autoantibodies.

ConclusionThese results suggest that the autoantibody and microvascular patterns in early SSc may each be related to different clinical-preclinical features and circulating activation markers at presentation. Longitudinal studies are warranted to investigate whether these subsets undergo a different disease course over time.

KeywordsRaynaud-s phenomenon early systemic sclerosis systemic sclerosis marker autoantibodies nailfold videocapillaroscopy preclinical organ involvement puffy fingerscirculating activation markers carboxyterminal propeptide of collagen I soluble E-selectin soluble IL-2 receptor alpha AbbreviationsACRAmerican College of Rheumatology

DLCOdiffusing lung capacity for carbon monoxide

E-A ratioearly-atrial late ventricular filling velocity ratio

ELISAenzyme-linked immunosorbent assay

EULAREuropean League Against Rheumatism

ICTPcarboxyterminal telopeptide of type I collagen

NVCnailfold videocapillaroscopy

RPRaynaud-s phenomenon

sE-selectinsoluble E-selectin

sIL-2Rasoluble IL-2 receptor alpha

SScsystemic sclerosis.

Electronic supplementary materialThe online version of this article doi:10.1186-ar4236 contains supplementary material, which is available to authorized users.

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Author: Gabriele Valentini - Antonella Marcoccia - Giovanna Cuomo - Serena Vettori - Michele Iudici - Francesco Bondanini - Carlo S

Source: https://link.springer.com/



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