CALCIFIC UREMIC ARTERIOLOPATHY: CLINICAL FEATURES AND TREATMEN
Autori:
Boris Kudumija, Mladen Knotek, DraŠko PavloviĆ, Sonja Dits
Sažetak
Kalcificirajuća uremijska arteriolopatija ili kalcifilaksija zloćudni je oblik kalcificiranja malih arterija i arteriola najčešće u bolesnika s nadomjesnim liječenjem kronične bubrežne bolesti. Uzrokuje visoku smrtnost. Histološka karakterističnost bolesti ogleda se u zahvaćenosti intimalnog sloja arterije gdje su prisutne proliferacija, linearna kalcificiranost unutarnje elastične membrane uz kalcificiranost medijalnoga mišićnog sloja arterije te često u upali i nekrozi potkožnoga masnog tkiva. Bolest započinje bolnim egzantemom, crvenkastolividnim nepravilnim plakovima ili mrežoliko oblikovanim lividnoljubičastim pjegama. Može napredovati prema eshari ili ranama koje se inficiraju i često uzrokuju sepsu. Prva prikazana bolesnica s proksimalnim tipom kalcifilaksije umrla je pod slikom ponovljene sepse. Druga bolesnica s distalnim tipom kalcifilaksije uspješno je liječena. Prijelomni trenutak liječenja nastupio je primjenom kalcimimetika. Liječenje je višestruko. Nužno je normaliziranje metabolizma P i Ca. Izdvaja se učinkovitost kalcimimetika, natrijskog tiosulfata, O2, pažljiva primjena bifosfonata i kirurških postupaka u liječenju rana. Potrebno je obustaviti liječenje varfarinom i razumno je primijeniti vitamin K. Karbonilirani hemoglobin mogao bi potaknuti brže cijeljenje neinficiranih rana.
Summary
Calcific uremic arteriolopathy or calciphylaxis is a malignant form of calcification of small arteries and arterioles, usually present in patients with chronic kidney disease and dialysis therapy. It causes high mortality. Histological distinctive feature are calcium deposits lining vascular intima. Calcification of medial muscle layer, inflammation and necrosis of subcutaneous adipose tissue are frequent. The disease begins with painful violaceous mottling, resembling livedo reticularis. The skin lesion progresses to ulcers and eschars, sometimes it becomes very vulnerable to secondary infection which can often develop into fatal sepsis. Our first patient with the proximal form of calciphylaxis died in repeated sepsis. The second patient with the distal form of calciphylaxis was treated successfully. The decisive moment was the use of calcimimetic. A multiinterventional strategy is likely to be more effective than any single therapy. It is necessary to regulate metabolism of calcium phosphate and secondary hyperparathyroidism. Effectiveness has been demonstrated using calcimimetics, sodium thiosulfate, oxygen therapy, careful application of biphosphonates and surgical procedures. Warfarin withdrawal is urgently recommended and subsequent vitamin K supplementation is appropriate. The control of infection is critically important and the use of carbonylated hemoglobin in the stage without infection could accelerate the wound healing.
