SAPHO (sinovitis, akne, pustoloza, hiperostoza i osteitis) sindrom

master's thesis

• Title (english): SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome
• Author: Korina Krmpotić
• Mentor: Porin Perić (mentor)
• Committee member: Iva Žagar (član povjerenstva)
• Committee member: Marija Bakula (član povjerenstva)
• Committee member: Porin Perić (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Physical Medicine and General Rehabilitation); Zagreb
• Defense date and country: 2024-07-12, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Physical Medicine and Rehabilitation

Abstract

SAPHO-sindrom, kratica za sinovitis, akne, pustulozu, hiperostozu i osteitis, predstavlja izazovan kronični upalni poremećaj koji prvenstveno zahvaća kosti, zglobove i kožu. Ovo stanje karakteriziraju različite kliničke manifestacije, uključujući bolne, otečene zglobove i različita kožna izbijanja. Složenost SAPHO-sindroma leži u njegovim različitim simptomima i preklapanju s drugim bolestima, što dijagnozu i liječenje čini posebno izazovnim. Točan uzrok SAPHO-sindroma ostaje nepoznat, ali vjerojatno uključuje genetske čimbenike i imunološki odgovor koji pokrene Cutibacterium acnes. SAPHO-sindrom je često neprepoznat, jer njegove kožne manifestacije mogu biti blage ili potpuno odsutne. Zbog složene anatomije često zahvaćenih područja, kao što je prednji zid prsnog koša, točna dijagnoza zahtijeva integrirani pristup korištenjem različitih modaliteta radiološkog prikazivanja. Iako ne postoji standardni protokol liječenja za SAPHO-sindrom zbog njegove rijetkosti, koristi se nekoliko terapijskih opcija temeljenih na simptomatskom olakšanju i mišljenju stručnjaka; NSAIDi i analgetici su prva linija liječenja SAPHO-sindroma, iako su često potrebni dodatni lijekovi. Kortikosteroidi nude brzo olakšanje, ali se preporučuju samo za kratkotrajnu upotrebu kako bi se izbjegle nuspojave. Za dugotrajne i uporne simptome koriste se DMARD lijekovi. U rezistentnim slučajevima učinkoviti su TNF-inhibitori poput infliksimaba i drugih bioloških lijekova koji ciljaju IL-1 i IL-17. Bisfosfonati pomažu u upravljanju osteitisom zbog svojih protuupalnih učinaka na kosti. Liječenje često zahtijeva prilagodbu kako bi se učinkovito riješili i koštano-zglobni i kožni simptomi, što potencijalno uključuje i kombiniranje sistemnih lijekova za kožu i imunosupresiva za zglobove. Zbog kronične prirode SAPHO-sindroma, potrebna je kontinuirana prilagodba liječenja temeljena na odgovoru pacijenta i aktivnosti bolesti. Interdisciplinarna suradnja neophodna je za optimizaciju ishoda i poboljšanje kvalitete života pacijenata.

Abstract (english)

SAPHO syndrome, an abbreviation for synovitis, acne, pustulosis, hyperostosis and osteitis, represents a challenging chronic inflammatory disorder that primarily affects the bones, joints, and skin. This condition is characterized by diverse clinical manifestations, including painful, swollen joints, and varying skin eruptions. The complexity of SAPHO syndrome lies in its varied symptoms and the overlap it shares with other diseases, making diagnosis and treatment particularly challenging. The exact cause of SAPHO syndrome remains unknown, but may involve genetic factors and an immune response possibly triggered by Cutibacterium acnes. SAPHO syndrome is often underrecognized, as its skin manifestations can be mild or entirely absent. Due to the complex anatomy of commonly affected areas like the anterior chest wall, accurate diagnosis requires an integrated approach using multiple imaging modalities. While there is no standard treatment protocol for SAPHO syndrome due to its rarity, several therapeutic options are utilized based on symptomatic relief and expert opinion; NSAIDs and analgesics are the initial treatments for SAPHO syndrome, though they often need additional therapies. Corticosteroids offer quick relief but are only recommended for short-term use to avoid side effects. For persistent symptoms, DMARDs are used. In resistant cases, TNF-inhibitors like infliximab and other biologics targeting IL-1 and IL-17 are effective. Bisphosphonates help manage osteitis due to their anti-inflammatory effects on bone. Treatment often needs customization to address both osteoarticular and cutaneous symptoms effectively, potentially combining systemic medications for skin and immunosuppressants for joint involvement. Due to its chronic nature, SAPHO syndrome requires ongoing adjustments in treatment based on patient response and disease activity. Interdisciplinary cooperation is essential for optimizing outcomes and improving patient quality of life.

• Keywords: SAPHO-sindrom; kronični upalni poremećaj; imunološki odgovor; dijagnostički izazovi; mogućnosti liječenja
• Keywords (english): SAPHO syndrome; chronic inflammatory disorder; immune response; diagnostic challenges; treatment options
• Language: croatian
• URN:NBN: urn:nbn:hr:105:425610
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2024-06-23 17:31:42