The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research group (EUSTAR) community. It is time to cut the Gordian knot and develop a prevention or rescue strategy

Abstract

Early diagnosis of systemic sclerosis (SSc) may allow the start of treatment that could slow disease progression. For this reason early diagnosis of the disease is of pivotal importance. However, the lack of diagnostic criteria and valid predictors significantly limit patient evaluation and the use of potentially effective drugs in the earliest phase of SSc. Early SSc may be suspected on the basis of Raynaud's phenomenon, puffy fingers, autoantibodies and SSc capillaroscopic pattern. In practice, the aim is to have criteria for the diagnosis of very early SSc. The criteria that are proposed are obviously provisional and need to be validated: (a) initially through a Delphi technique; (b) thereafter perhaps using already available datasets; but (c) of critical importance, through prospective studies. Only after prospective studies can these potential criteria be considered validated. The consensus on criteria for the classification of very early SSc might be part of the evolving EULAR/ACR project of reclassification of SSc.

Determinants of Morbidity and Mortality of Systemic Sclerosis in Canada

“…Чтобы установить диагноз ССД и определить адекватные терапевтические подходы на этом начальном этапе бо-лезни, существующих критериев АCR недостаточно. Иногда поражение внутренних органов развивается ра-П р о г р а м м а н е п р е р ы в н о г о п о с т д и п л о м н о г о о б р а з о в а н и я в р а ч е й [17,18].…”

Systemic sclerosis – diagnostic and therapeutic recommendations of the Polish Dermatological Society. Part 1: diagnosis and monitoring

“…obrzęknięte palce (puffy fingers) w połączeniu z ww. cechami nazwano "czerwonymi flagami" i zaliczono do objawów wczesnej SSc (very early diagnosis of SSc -VEDOSS) [8,9]. Wstępne wyniki prospektywnego badania VEDOSS wykazały, że dodatnia wartość predykcyjna dla SSc u chorych [16,17].…”

“…In 2001, LeRoy and Medsger proposed diagnostic criteria for early SSc which included Raynaud's phenomenon, nailfold capillaroscopy changes and presence of SSc-specific autoantibodies [7]. In 2014, the so-called puffy fingers -in combination with the above -were indicated as "red flags" and included as symptoms of very early diagnosis of SSc (VEDOSS) [8,9]. Preliminary results of the prospective trial VEDOSS show that the positive predictive value for systemic sclerosis in patients presenting with Raynaud's phenomenon, puffy fingers turnmi z 1980 r. [4].…”

Early systemic scleroderma: a current diagnostic algorithm (a lecture)

“…Сегодня развитие учения об эволюции ССД позволи-ло сформулировать представление об очень раннем сис-темном склерозе -состоянии, характеризующемся разви-тием феномена Рейно и/или отечности кистей с элемента-ми склеродактилии, типичными структурными микровас-кулярными нарушениями и характерными аутоиммунны-ми нарушениями [31]. Для диагностики очень ранней ССД авторами предложен приведенный ниже набор признаков, которые рекомендовано валидировать как критерии в про-спективных исследованиях.…”