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Owrzodzenia i zmiany martwicze palców powikłaniami zespołu Raynauda. Jakie są objawy i na czym polega leczenie?

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W przebiegu zespołu Raynauda dochodzi do powtarzających się nagłych skurczów naczyń obwodowych palców. Skutkuje to kolejno zblednięciem, zasinieniem i zaczerwienieniem skóry. U osób cierpiących na to schorzenie na opuszkach palców mogą rozwinąć się owrzodzenia i zgorzel.

Objaw Raynauda dotyka około 3-5 proc. populacji. Częściej występuje u kobiet niż u mężczyzn. Wyróżnia się dwa rodzaje tego schorzenia. Odmiana pierwotna, inaczej choroba Raynauda, stanowi około 80 proc. wszystkich przypadków. Jej etiologia nie jest znana. Postać wtórna, inaczej zespół Raynauda, towarzyszy schorzeniom, w przebiegu których dochodzi do zaburzeń ukrwienia. Charakteryzuje ją także większa bolesność i nasilenie objawów.

Przyczyny zespołu Raynauda

Do najczęstszych przyczyn zespołu Raynauda należą układowe choroby tkanki łącznej, w tym przede wszystkim twardzina układowa. W przebiegu tego schorzenia dochodzi do włóknienia skóry i zajęcia drobnych naczyń krwionośnych, które ulegają zwężeniu. W konsekwencji dochodzi do ograniczenia przepływu krwi.

Zespół Raynauda występuje również w u osób cierpiących na choroby tętnic, np. miażdżycę, w przebiegu której dochodzi do zmian patologicznych w obrębie mikrokrążenia. Może też towarzyszyć chorobie Buergera, która objawia się stopniowym zwężeniem i zamknięciem naczyń krwionośnych.

Objaw Raynauda towarzyszy również chorobom układu nerwowego, np. zespołowi cieśni nadgarstka. U osób cierpiących na tę neuropatię dochodzi do zaburzeń ukrwienia nerwu pośrodkowego. Zespół Raynauda pojawia się także w u osób cierpiących na schorzenia przebiegające z nadmierną lepkością krwi, np. chłoniaka czy chorobę zakrzepowo-zatorową.

Na wystąpienie zespołu Raynauda szczególnie narażone są także osoby przyjmujące takie leki jak m.in. chemioterapeutyki, estrogeny i nieselektywne beta-adrenolityki.

Czynnikiem zwiększającym ryzyko wystąpienia zespołu Raynauda jest również palenie papierosów. Nikotyna powoduje zwężenie naczyń krwionośnych, co skutkuje zmniejszonym przepływem krwi do skóry.

Objawy zespołu Raynauda

Zespół Raynauda w przeważającej liczbie przypadków dotyczy palców dłoni. Tylko u ok. 5 proc. pacjentów zmiany obejmują również okolice palców stóp. W bardzo rzadkich przypadkach zmiany chorobowe występują także na nosie, wargach, małżowinach usznych, języku i brodawkach sutkowych.

Do napadu zespołu Raynauda może dojść pod wpływem m.in. zimna lub stresu. Niekiedy czynnik prowokujący wystąpienie epizodu nie jest znany.

U chorego wskutek nagłego skurczu naczyń krwionośnych dochodzi do zblednięcia palców. Pacjent doświadcza uczucia mrowienia. Następnie w wyniku skumulowania się odtlenowanej krwi palce stają się sine, czemu towarzyszą objawy takie jak drętwienie i ból. Później dochodzi do gwałtownego rozszerzenia naczyń krwionośnych. W konsekwencji skóra staje się zaczerwieniona i ucieplona. Pojawia się także uczucie pieczenia.

U niektórych pacjentów epizod ogranicza się jedynie do zblednięcia i zasinienia albo zasinienia i zaczerwienienia.

Zmiany skórne są wyraźnie odgraniczone od obszaru nieobjętego procesem chorobowym. Mogą być zarówno asymetryczne, jak i symetryczne.

Zazwyczaj objawy ustępują po upływie od kilku do 20 minut. U niektórych pacjentów epizody trwają jednak nawet ponad godzinę. Napady mogą wystąpić kilka razy w ciągu doby, w tym także w nocy podczas snu.

Powikłania zespołu Raynauda

Wskutek powtarzających się napadów, podczas których dochodzi do niedokrwienia palców, tkanki nie otrzymują wystarczającej ilości tlenu i składników odżywczych. Następstwem tego jest zwiększona podatność na uszkodzenia.

Na opuszkach palców mogą powstać rany, które u wielu pacjentów rozwijają się w owrzodzenia. Ze względu na umiejscowienie ran na obszarze ciała szczególnie narażonym na kontakt z drobnoustrojami często dochodzi do zakażeń.  Jednocześnie ze względu na występujące zaburzenia ukrwienia gojenie ran jest utrudnione. Na palcach może dojść do zmian martwiczych i zgorzeli.

Na opuszkach palców obserwuje się również blizny naparstkowe. W niektórych przypadkach dochodzi do samoistnej amputacji paliczków dystalnych.

U chorych na zespół Raynauda w obrębie dłoni pojawiają także się zmiany skórne, m.in. stwardnienie skóry czy wykwity. Charakterystyczne są także zniekształcenia paznokci i ich nasilona łamliwość. U pacjentów odnotowuje się również obrzęk stawów.

Leczenie zespołu Raynauda

Terapia zespołu Raynauda obejmuje przede wszystkim leczenie choroby podstawowej. Wdraża się także działania mające na celu m.in. ograniczenie nasilenia i częstości epizodów oraz wyleczenie zmian skórnych.

Farmakologia

W celu zmniejszenia nasilenia i częstości napadów chorym na zespół Raynauda zaleca się przyjmowanie blokerów kanału wapniowego. Odnotowano także wysoką skuteczność stosowania pentoksyfiliny. Środek ten zwiększa elastyczność ścian naczyń krwionośnych, a tym samym ułatwia przepływ krwinek przez naczynia włosowate. Ponadto zmniejsza zdolność erytrocytów do adhezji i agregacji.

Leczenie miejscowe

W ramach terapii miejscowej stosuje się m.in. środki z nitrogliceryną w formie maści lub kremu. Preparaty te powodują miejscowy wzrost temperatury i poprawiają obwodowy przepływ krwi, a tym samym zmniejszają ryzyko wystąpienia napadu.

Leczenie ran  

W przypadku rozwoju owrzodzeń wdraża się postępowanie z raną trudno gojącą się zgodne ze strategią TIME. Jeśli zmiany martwicze są rozległe lub doszło do zgorzeli, konieczna może okazać się amputacja.

Pacjentom cierpiącym na twardzinę układową zaleca się także stosowanie selektywnego inhibitoru fosfodiesterazy typu 5, który wspomaga gojenie owrzodzeń.

W przypadku ciężkiego przebiegu zespołu Raynauda, nieskuteczności wymienionych wyżej metod leczenia i wystąpienia owrzodzeń stosuje się prostanoidy. U osób chorujących na twardzinę układową w celu zapobiegania powstawaniu dalszych owrzodzeń zaleca się też bozentan.

Leczenie chirurgiczne

W przypadku opornego na leczenie farmakologiczne i zaawansowanego przebiegu zespołu Raynauda przeprowadza się sympatektomię chirurgiczną. Zabieg ten polega na przerwaniu ciągłości nerwów współczulnych w klatce piersiowej. Powoduje to rozszerzenie naczyń krwionośnych.

Źródła: A. Wojteczek, M. Ziętkiewicz, Z. Zdrojewski, „Objaw Raynauda – obraz kliniczny, diagnostyka i leczenie jednego z najczęściej spotykanych objawów reumatologicznych w praktyce lekarskiej”, 2014.

„Chirurgia”, red. W. Noszczyk, t. 2, Warszawa 2005, str. 582-583.

mp.pl

 

Przeczytaj także:

Twardzina układowa przyczyną trudno gojących się ran na dłoniach

Biblioteka Faktów

Przeczytaj bezpłatnie pokrewny artykuł w czasopiśmie „Pielęgniarstwo w Anestezjologii i Intensywnej Opiece”:

Zakażenie ran i owrzodzeń – postępowanie w praktyce pielęgniarskiej

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