Author/Editor		Videčnik, Viktor
Title		Sistemske vezivno-tkivne bolezni in akrosindromi
Translated title		Systemic connective-tissue disorders and acrosyndromes
Type		članek
Source		Med Razgl
Vol. and No.		Letnik 35, št. Suppl 2
Publication year		1996
Volume		str. 161-7
Language		slo
Abstract		The distinguishing between primary and secondary Raynaud's phenomena (RP) is important for further treatment of patients with this disease as well as for prognosis. In most cases simple clinical criteria do not suffice and additional test methods are required. In certain cases, patients need to be observed for at least 2 years in order to find out whether the Raynaud's phenomenon is merely a manifestation of another disease, or a primary form. The RP is the most frequent sign of vascular disease in progressive systemic sclerosis and the CREST syndrome, while it appears much more rarely in other forms of systemic connective-tissue diseases. Pathophysiological processes in RP are not yet completely understood, but they are certainly complex, multi-causal and include changes in the response of neuroreceptors and vasodilative neuropeptides, as well as vasodilative and vasoconstrictive products of endothelial cells and products released from thrombocytes. The opinion that endotheline, a vasoconstrictor secreted by endothelial cells, is very important in the pathophysiology of RP is prevailing. In distinction from primary RP, damage to endothelial cells, their degeneration and dysfunction occur in systemic connective-tissue disorders. The cause of this damage is not clear. Damage can be proven in the blood of patients by an increased concentration of von Willebrand's factor, the concentration of immune complexes may be increased and signs of the activation of the complement system may be present. Among non-invasive test methods, vital capillaroscopy of the perionychium remains the best basic test in which characteristic morphological changes are found, especially in secondary Raynaud's syndrome and systemic connective-tissue diseases. These morphological changes can be clearly distinguished from barely indicated or absent changes in the primary form of the RP. Numerous other non-invasive methods are also used. (Abstract truncated at 2000 chars)
Summary		Opredelitev primarnega in sekundarnega Raynaudovega fenomena je pomembna zaradi nadaljnje obravnave bolnikov pa tudi prognoze bolezni. Včasih je treba bolnika opazovati najmanj 2 leti, da lahko ugotovimo, ali gre za primarno obliko Raynaudovega fenomena ali pa je le manifestacija neke druge bolezni, kot so sistemska skleroza, sindrom CREST, redkeje ostale oblike sistemskih vezivno-tkivnih bolezni. Patofiziološka dogajanja pri Taynaudovem fenomenu še niso povsem razjasnjena. Vpletajo se spremenjena odzivnost nevroreceptorjev, vazodilatatorni nevropeptidi, razen tega pa tudi vazodilatatorni in vazokonstriktorni produkti endotelnih celic in produkti, ki se sprostijo iz trombocitov. Prevladuje stališče, da je endotelin - vazijibstrujtirm ki ga izloča endotelna celica - zelo pomemben v patofiziologiji Raynaudovega fenomena. Pri sistemskih vezivno-tkivnih boleznih pride za razliko od primarnega Raynaudovega fenomena do poškodbe endotelnih celic, do njihove degeneracije in motnje delovanja. Vzrok poškodbe ni jasen. Poškodbe lahko dokažemo v krvi bolnikov z zvišano koncentracijo von Willebrandovega faktorja, zvišane pa so lahko še koncentracije imunskih kompleksov in prisotni znaki aktivacije komplementnega sistema. Od neinvazivnih preiskovalnih metod je vitalna kapilaroskopija obnohtja še vedno najboljša osnovna preiskava, pri kateri najdemo značilne morfološke spremembe, zlasti pri sekundarnem Raynaudovem fenomenu ter pri sistemskih vezivno-tkivnih boleznih, ki jih lahko jasno ločimo od komaj nakazanih ali odsotnih sprememb pri primarni obliki. Uporabljajo pa se tudi številne druge neinvazivne preiskavne metode. Princip merjenja je pri vseh enak. Ugotavljamo spremenjen pretok krvi v subpapilarnih pletežih prstov, ki se patološko zmanjša ali celo ustavi med ohladitvenimtestom, nato pa merimo čas do nastopa reaktivne hiperemije v fazi ogrevanja, ki je pri bolnikih z RF izrazito podaljšan. (Izvleček prekinjen pri 2000 znakih.)
Descriptors		CONNECTIVE TISSUE DISEASES RAYNAUD'S DISEASE