Utilidad de la capilaroscopia como factor pronóstico en la esclerodermia sistémica.

Abstract

Systemic sclerosis (SE) is a connective tissue disease characterized by excessive_x000D_ deposit of collagen in tissues and microcirculation vascular abnormalities in the context_x000D_ of an immunologic disturbance. Its main feature is skin induration, although there is_x000D_ frequent internal organ involvement, such as digestive, respiratory, renal and cardiac_x000D_ systems, leading to a wide spectrum of manifestations that may determine both a slow,_x000D_ progressive and indolent evolution, or a rapidly progressive clinical deterioration. The_x000D_ more specific clinical manifestation of the sclerodermic vasculopathy is the Raynaud's_x000D_ phenomenon, the pulmonary arterial hypertension and renal crisis (RC), being this last_x000D_ one the most important in the prognosis of the disease. Since microvascular damage is a_x000D_ typical feature of SE, capillaroscopy is an essential tool for both, the early diagnosis of_x000D_ this disease and the monitoring of its evolution and prognosis._x000D_ The aim of the present work is to correlate the capillaroscopy microcirculation_x000D_ alterations with clinical and immunological features in patients with SE, in order to_x000D_ assess the usefulness of this test as a prognostic tool. We have fullfilled a retrospective_x000D_ study that included 235 patients classified on the basis of the capillaroscopic pattern_x000D_ (active pattern, slow pattern), correlating those data with clinical features of the disease_x000D_ (cutaneous manifestations, respiratory alterations, renal crisis, cardiac complications,_x000D_ vascular ulcers ...) as well as the immunological profile (antinuclear, anticentromere o_x000D_ antitopoisomerase antibodies positivity)._x000D_ The present work demonstrates that the presence of active capillaroscopic_x000D_ pattern is related to diffuse ES subtype, to the antitopoisomerase antibody positivity, to_x000D_ a higher frequency of vascular ulcer development and a higher incidence of CR. At the_x000D_ same time, by the survival study we've been able to define independent factors of poor_x000D_ prognosis: diffuse ES subtype, age at diagnosis 65 years or older, development of_x000D_ pulmonary hypertension, interstitial lung disease and scleroderma renal crisis._x000D_ Therefore, although the active capillaroscopic pattern failed to prove to be a poor_x000D_ prognostic independent factor, is significantly associated with poor prognostic clinical_x000D_ phenotypes (diffuse ES subtype). Similarly, this pattern is associated with one of the_x000D_ main causes of morbidity of the disease: vascular ulcers._x000D_ In conclusion, the determination of the capillaroscopic pattern is essential, not_x000D_ only for the ES diagnosis, but also as a tool to identify patients at risk for bad clinical_x000D_ outcome.

Date of Award	27 Nov 2013
Original language	Spanish
Supervisor	Vicente Fonollosa Pla (Director) & Carmen Pilar Simeon Aznar (Director)