Systemische Sklerose – Diagnose und Klassifikation

Abstract

Systemic sclerosis (SSc) is a polymorphic and heterogenic systemic disorder with inflammation, fibrosis and vascular damage. Early diagnosis and classification may be difficult if disease expression is oligosymptomatic (undifferentiated), presenting with only Raynaud's phenomenon or limited scleroderma. Scleroderma specific antinuclear autoantibodies, which are present early and persistently in about 90% of the patients with SSc, play an important taxonomic role. Scleroderma specific findings in nailfold capillary microscopy are sensitive and predictive for evolving SSc. An algorithm will be presented for the diagnosis and classification of SSc using clinical, capillaroscopic and serologic criteria, which are also useful for mixed or special forms of SSc. The 6th Outcome Measures in Rheumatology Clinical Trials (OMERACT) conference proposed different outcome measurements for clinical studies, however, for daily clinical practice there is as yet no consensus on status indices for disease activity, disease related damage or suitable prognostic criteria.

Systemic sclerosis – dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings

“…Unusual and very rapid courses as well as poor therapy response should prompt considering paraneoplasia and respective diagnostics to search for an underlying malignancy. Based on the diagnostic criteria different classifications have been proposed including that of the Working Group Dermatological Research (Arbeitsgruppe Dermatologische Forschung, ADF) from 1986 [23] as well as the in the meantime generally accepted division into a limited systemic scleroderma and a diffuse systemic scleroderma [24]. Recent data from European and US-American registries confirm the usefulness of this division, as a correlation of immunoserologic parameters and the course as well as type and extent of organ involvement is possible (Table 1).…”

“…Due to sclerosis of the skin the individual physiognomy is lost, so that scleroderma patients appear quite similar at first glance. The involvement of internal organs is typical for systemic scleroderma, but is quite variable with respect to the temporal course, extent and involvement of individual organs [5,24,30,32] (Table 2, Figure 8). This heterogeneity due to the lack of clearly defined biomarkers can be foreseen only to a limited extent.…”

Initial characterization of stiff skin‐like syndrome in West Highland white terriers

“…Stiff skin is a descriptive term for indurated, inelastic skin that is attached firmly to the underlying tissue and is often used interchangeably with stone hard skin in human medicine to describe changes in stiff skin syndrome (SSS), geleophysic dysplasia, systemic sclerosis (SSc) and localized scleroderma or morphea …”

Capillaroscopy

“…Die Kapillarmikroskopie ist eine wich tige Ergänzung der Autoantikörperdia gnostik in der Differenzierung zwischen primärem und sekundärem Raynaud Phänomen [14,19,21]. Das Risiko für ei ne definitive SSc steigt bei Vorliegen von RaynaudPhänomen und gleichzeitigem Vorliegen von SScassoziierten Autoanti körpern bei kapillarmikroskopischen Veränderungen nochmals um den Fak tor 5; 80% dieser Patienten entwickeln in 10 Jahren eine manifeste Erkrankung.…”