POLYMYOSITIS AND SYSTEMIC SCLEROSIS OVERLAP – A CASE REPORT AND A REVIEW OF THE LITERATURE

Polimiozitis (PM) autoimunosna je bolest koja primarno zahvaća poprečnoprugastu muskulaturu. U mlađoj životnoj dobi najčešće se radi o idiopatskom obliku povezanom sa specifi čnim autoprotutijelima (anti Jo), dok je u zreloj dobi češće povezana s novotvorinama. PM može biti udružen s drugim autoimunosnim bolestima kao što je sistemska skleroza (SSc), rijetka progresivna bolest kojoj je glavno obilježje patološka fi broza tkiva i organa. 65-godišnji bolesnik, dugogodišnji pušač, primljen je u bolnicu zbog bolnog edema podlaktica i potkoljenica te izražene slabosti proksimalnih mišića udova. Iako mišićni enzimi nisu bili povišeni, dijagnoza PM-a postavljena je na osnovi karakterističnoga patohistološkog nalaza. Na trupu su uočene kožne promjene koje bi mogle odgovarati SSc-u, ali nije bilo pozitivnih autoprotutijela ni drugih dijagnostičkih kriterija SSc-a. Nakon iscrpne obrade zbog moguće maligne bolesti započeta je terapija glukokortikoidima (GK) koja je dovela do jenjavanja edema i jačanja mišićne snage. Već nakon četiri tjedna bolesnik se javlja sa simptomima koji upućuju na SSc: Raynaudov sindrom, disfagija i izrazito zadebljanje kože prethodno otečenih dijelova udova. PM je nerijetko pridružen SSc-u. U ovom slučaju nije jasno je li egzacerbacija latentnog SSc-a potaknuta višim terapijskim dozama GK ili se radi o jednostavnom preklapanju dviju bolesti s različitim početkom. Premda ne postoje terapijske smjernice za liječenje preklapanja PM-a i SSc-a, nužna je oprezna primjena GK već i kod diskretnih naznaka SSc-a zbog poznatog učinka viših doza GK na razvoj i tijek bolesti.Polymyositis (PM) is an autoimmune disease which aff ects skeletal muscles. In young age, it usually occurs as an idiopathic disorder associated with specifi c autoantibodies (anti-Jo), while in older age it is oft en associated with neoplasms. It can present with symptoms of other autoimmune diseases, such as systemic sclerosis (SSc), a rare progresive disease characterized by collagen deposits in various tissues and organs. A 65-year-old patient, long-time smoker, came to the ER because of painful edema in the distal parts of his limbs and proximal muscle weakness of his arms and legs. Although his muscle enzymes were not increased, PM was confi rmed by the characteristic pathohistological fi nding. Th e patient had sclerodermal skin lesions on his back, but he did not have other typical SSc symptoms, and the specifi c autoantibodies were negative. He received glucocorticoid therapy (GC) aft er we had fi nished screening for malignant tumors. He felt better, his muscle strength returned, and the limb edema disappeared. Four weeks later, he developed symptoms which are more typical of SSc, such as dysphagia, Raynaud’s phenomenon, and skin thickening of the limbs that had been swollen. PM is oft en associated with SSc. It is not clear if the exacerbation of latent SSc was stimulated by GC, or if it was just a simple overlap of the two diseases with diff erent onsets. Th ere are no therapy guidelines for the treatment of this combination of diseases. Careful use of GC is necessary even if SSc symptoms are discreet, because of the well-known eff ects of GC in SSc