Zapalenie naczyń związane z krioglobulinemią (ZNK) to układowa choroba tkanki łącznej spowodowana odkładaniem się specyficznych przeciwciał w małych naczyniach. Schorzenie to może stanowić jedną z rzadkich przyczyn trudno gojących się ran na kończynach dolnych.
Przyczyny ZNK
Przyczyną ZNK jest odkładanie się w małych naczyniach krioglobulin, czyli przeciwciał o specyficznych właściwościach. Proces ten zachodzi w przypadku obniżenia temperatury organizmu poniżej 37 st. C. Objawy ZNK występują w wyniku reakcji organizmu na obecność krioglobulin.
Do produkcji krioglobulin dochodzi w odpowiedzi na zakażenie wirusami zapalenia wątroby typu B i C, wirusem HIV, a także w wyniku zakażeń o innej etiologii (bakteryjnej lub pasożytniczej). Przyczyną odkładania krioglobulin może być również choroba o podłożu hematologicznym (np. chłoniak) bądź schorzenie tkanki łącznej (reumatoidalne zapalenie stawów, twardzina układowa, toczeń rumieniowaty układowy, zespół Sjorgena).
Objawy ZNK
Wśród objawów ZNK wymienia się zaburzenia neurologiczne o szerokim spektrum (drętwienie i ból kończyn dolnych, zaburzenia mowy, porażenie mięśni). Ze względu na związek ZNK z zakażeniem wirusem zapalenia wątroby u znacznej grupy chorych rozwija się marskość wątroby, żółtaczka, w rzadkich wypadkach także rak wątrobowokomórkowy. Możliwe są również objawy ze strony nerek (zmniejszone wydalanie moczu, nudności, wymioty).
Zapalenie naczyń związane z krioglobulinemią stanowi również rzadką przyczynę trudno gojących się ran. U chorych na ZNK występują dwa główne mechanizmy uszkodzenia tkanek. Odkładanie się krioglobulin typu II lub III prowadzi do leukocytoklastycznego zapalenia naczyń.
W reakcji na krioglobuliny typu I występuje natomiast waskulopatia, która może objawiać się klinicznie jako martwica w narażonych na zimno obszarach, takich jak dystalne części kończyn (dłonie i stopy), a także usta, uszy i nos. Na kończynach dolnych mogą również występować trudno gojące się owrzodzenia, nadżerki i krwotoczne pęcherze.
Leczenie
Terapia ZNK ze względu na szerokie spektrum objawów wymaga kompleksowego zaangażowania specjalistów z różnych dziedzin, w tym m.in. hematologii, gastroenterologii, hepatologii, nefrologii, reumatologii.
W leczeniu farmakologicznym stosuje się glikokortykosteroidy i leki immunosupresyjne. W części przypadków wykorzystywane są również plazmafereza i terapia przeciwciałami monoklonalnymi.
Bardzo istotne jest również leczenie przyczynowe pod kierunkiem specjalisty chorób zakaźnych.
Źródła:
Isoherranen K, Jordan O’Brien J, Barker J et al. EWMA document; Atypical wounds. Best clinical practice and challenges.
Przeczytaj także: Co to jest rana i jak powinien przebiegać proces gojenia?
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