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Journal ArticleDOI

Early Diagnostic and Predictive Value of Capillaroscopy in Systemic Sclerosis.

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TLDR
NVC is a safe and reliable tool for the early diagnosis of SSc and the different NVC scleroderma patterns have a predictive value for the clinical complications of the disease.
Abstract
Nailfold microvascular impairment represents an early feature of systemic sclerosis (SSc) and its progression through different patterns of capillary damage and their validated scoring, is evaluable by nailfold videocapillaroscopy (NVC) in a safe and reliable manner. The presence of specific morphological microvascular alterations at the NVC (i.e., presence of giant capillaries) is fundamental and mandatory for the early diagnosis of SSc, together with the presence of the Raynaud's phenomenon. Furthermore, a recent longitudinal study showed a dynamic transition of microvascular damage through different NVC patterns of microangiopathy in almost 50% of SSc patients and clinical symptoms progressed in accordance with the NVC morphologic changes in 60% of the SSc patients. A pilot study was the first demonstrating an association between baseline NVC patterns and future severe, peripheral vascular and lung involvement with stronger odds according to worsening scleroderma patterns. Prognostic indexes for digital trophic lesions, especially for daily use in SSc clinics and simply limited to the mean score of capillary loss are now validated. Very recently, it has been described that efficacious potentially disease modifying therapies in SSc may interfere with progression of nailfold microvascular damage, as assessed by NVC, over long term at least in presence of digital ulcers. NVC is a safe and reliable tool for the early diagnosis of SSc and the different NVC scleroderma patterns have a predictive value for the clinical complications of the disease.

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Journal ArticleDOI

Standardisation of nailfold capillaroscopy for the assessment of patients with Raynaud's phenomenon and systemic sclerosis

TL;DR: Experts in the field of capillaroscopy/microcirculation provide in this very consensus paper their view on image acquisition and analysis, different capillsaroscopic techniques, normal and abnormal capillARoscopic characteristics and their meaning, scoring systems and reliability of image acquisitionand interpretation.
Journal ArticleDOI

Morphea and Eosinophilic Fasciitis: An Update

TL;DR: A state-of-the-art flow chart is presented that guides care providers in the treatment of morphea and EF and shows how to take disease subtype, degree of activity, depth of involvement, and quality of life impairments into account when initiating treatment.
Journal ArticleDOI

Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF).

TL;DR: The detection of specific autoantibodies serves a critical role in the diagnosis of CTD-ILD, but there remains a lack of data to guide clinical practice including which autoantIBodies should be tested on initial assessment and when or in whom serial testing should be performed.
Journal ArticleDOI

Interstitial Pneumonia with Autoimmune Features.

TL;DR: The relevant background that led to the development of IPAF is focused on, the proposed criteria is summarized, and cohort studies of patients with IPAF published to date and what they have taught us about the IPAF phenotype are discussed.
Journal ArticleDOI

Existing and novel biomarkers for precision medicine in systemic sclerosis.

TL;DR: An urgent unmet need exists for validated, non-invasive biomarkers for the diagnosis, assessment of disease activity and response to therapy of patients with systemic sclerosis (SSc).
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