Antisintetaza sindrom – prikaz bolesnice

Višnja Prus, Dražen Bedeković, Jasminka Milas-Ahić, Roberta Višević, Branko Šegec, Zlatica Jukić, Ljiljana Perić

Twenty-eight year-old woman with predominant signs of polymyositis, pulmonary interstitium involvement and with positive anti-Jo1 antibodies was suspected for antisynthetase syndrome. Over the next three months sores and ulcerations have appeared at the fingertips. In the later course of the disease clinical picture of mixed connective tissue disease associated with interstitial lung disease, with a dominant picture of systemic sclerosis have emerged. She was treated with glucocorticoides and immunosuppressive therapy. Patient condition was mostly stable, without significant progression of lung lesions. Early diagnosis and treatment antisynthetase syndrome significantly contributes to more favorable course and outcome of disease. A prerequisite for that are well-defined diagnostic criteria and an appropriate choice of treatment.

U dvadesetosmogodišnje bolesnice s dominirajućim znacima polimiozitisa i zahvaćanjem plućnog intersticija s pozitivnim nalazom anti-Jo1 protutijela postavljena je sumnja na antisintetaza sindrom. Tijekom sljedeća tri mjeseca pojavile su se ragade i ulceracije na vršcima prstiju. U kasnijem tijeku bolesti javila se slika miješane bolesti vezivnog tkiva udružene s intersticijskom plućnom bolesti uz dominirajuću sliku sistemske skleroze. Liječena je glukokortikoidima uz imunosupresivnu terapiju na koju je stanje uglavnom bilo stabilno, bez značajnije progresije plućnih promjena. Rana dijagnoza i liječenje antisintetaza sindroma utječu na povoljniji tijek i ishod bolesti. Preduvjet za navedeno su dobro definirani dijagnostički kriteriji uz procjenu stanja i adekvatan izbor terapije.