RELAPSING POLYCHONDRITIS – CASE REPORT

Autori:

Irzal Hadžibegović, Joško Mitrović, Marija Santini, Dalibor Vukelić, Jadranka Morović-Vergles

Sažetak

Sažetak. Ponavljani polihondritis (PP) rijetka je progresivna sustavna upalna bolest u kojoj recidivirajuće epizode upale hrskavičnih struktura konačno dovode do razaranja hrskavica uha, nosa i dišnog sustava. Bolest može zahvatiti zglobove, oči, slušni i krvožilni sustav. U oko 30% bolesnika s PP-om nalazi se druga reumatska ili autoimunosna odnosno maligna bolest poput karcinoma debelog crijeva, pluća, dojke ili limfoma. Etiologija i patogeneza PP-a su poznate. Nema specifičnoga laboratorijskog parametra za PP. Dijagnoza PP-a temelji se na kliničkim kriterijima. Liječi se glukokortikoidima, a u novije vrijeme i primjenom bioloških lijekova. Tijek bolesti obilježen je povratnim akutnim epizodama hondritisa različitih struktura. Prognoza bolesnika s PP-om posljednjih nekoliko godina je bolja zahvaljujući sve boljem konzervativnom i kirurškom liječenju. U radu smo prikazali bolesnika u kojeg je PP dijagnosticiran unutar četiri tjedna od početka simptoma, a primjena glukokortikoida bila je učinkovita.

Summary

Summary. Relapsing polychondritis (RP) is a rare systemic inflammatory disease in which recurrent episodes of cartilage inflammation result in destruction of ears, nose and tracheobronchal tract. The joints, eyes, audiovestibular system and cardiovascular system can also be involved. About 30% of patients with RP have coexisting autoimmune disease, or malignant disease like colon, breast, and lung carcinoma, or malignant lymphoma. Pathogenesis is still unknown, and there is no consistent laboratory parameter specific for RP, which makes the diagnosis mainly clinical. Glucocorticoids are a mainstay of medical treatment of RP, whereas newer studies show positive effects of biological therapy. The course of RP is characterized by recurrent episodes of cartilage inflammation, and the prognosis has been recently improved because of improved medical and surgical treatment. We present a case of a patient with RP who was diagnosed 1 month after the development of first symptoms and responded well to glucocorticoid therapy.