Title Autoinflamatorne bolesti kostiju Title (english) Autoinflammatory bone disorders Author Nastasia Čekada Mentor Marija Jelušić (mentor)Committee member Ivan Malčić (predsjednik povjerenstva)Committee member Jurica Vuković (član povjerenstva)Committee member Marija Jelušić (član povjerenstva)Granter University of Zagreb Defense date and country 2016-07-15, Croatia Scientific / art field, BIOMEDICINE AND HEALTHCARE Abstract Uvod: Autoinflamatorne bolesti kostiju karakterizirane su sterilnom upalom kosti praćene kožnim manifestacijama. Ovoj skupini pripadaju sinovitis, akne, pustuloza, hiperostoza, osteitis (SAPHO) sindrom i kronični rekurirajući multifokalni osteomijelitis (CRMO), Majeed sindrom, DIRA, PAPA sindrom i kerubizam. CRMO i SAPHO su dva najčešća entiteta. Obzirom na niz sličnosti, dio autora smatra da je CRMO, koji se dominantno javlja u dječjoj dobi, podtip SAPHO sindroma, koji se najčešće klinički prezentira u adolescenciji ili odrasloj dobi.
Cilj: Analizirati i usporediti karakteristike bolesnika s CRMO i SAPHO sindromom.
Metode i ispitanici: Retrospektivna analiza i usporedba podataka iz medicinske dokumentacije bolesnika s CRMO i SAPHO sindromom liječenih u Zavodu za imunologiju i reumatologiju Klinike za pedijatriju KBC Zagreb, Referetnom centru za pedijatrijsku i adolescentnu reumatologiju RH u razdoblju od 2002. – 2015. godine.
Rezultati: U razdoblju od 2002. – 2015. godine liječene su 3 djevojčice s CRMO prosječne dobi od 5 godina i 3 mladića sa SAPHO sindromom, prosječne dobi od 16 godina u vrijeme postavljanja dijagnoze. Svi bolesnici su inicijalno imali umjereno povišene upalne parametre. U svih bolesnica s CRMO obradom su nađene multifokalne upalne promjene različitih dijelova aksijalnog i perifernog skeleta, u samo jedne bolesnice promjene su bile simetrične. Niti jedna nije imala kožnih promjena. Liječene su nesteroidnim protuupalnim lijekovima, u jedne je navedenom terapijom postignuta remisija, dok je u dvije bolesnice inidicirana i kraća sistemska primjena kortikosteroida s istim, povoljnim ishodom.
U samo jednog bolesnika sa SAPHO sindromom nađen je tipični scintigrafski nalaz upalnog zahvaćanja sternoklavikularnih zglobova i sternuma, dok su u ostalih bolesnika promjene detektirane u različitim dijelovima skeleta, uključujući maksilu i mandibulu. Kožne promjene u vidu opsežnih akni imala su sva 3 bolesnika, u 2 su akne predstavljale inicijalni simptom bolesti, dok su se u jednog razvile 2 godine nakon početka upalnih promjena skeleta. U dva bolesnika zadovoljavajuća kontrola bolesti uspostavljena je primjenom indometacina ili metotreksata, u jednog bolesnika anti TNF pripravkom.
Zaključak: CRMO i SAPHO sindrom imaju niz zajedničkih karakteristika te se prema dijelu recentne literature CRMO smatra dječjom formom SAPHO sindroma, koji se dominantno javlja u adolescenata i odraslih. Rezultati usporedbe naših bolesnika s navedenim bolestima sukladni su literaturnim podacima. Tek će otkrivanje još uvijek nepoznate etiopatogeneze definitivno razjasniti odnos dviju najčešćih autoinflamatornih bolesti kostiju.
Abstract (english) Introduction: The main characteristic of autoinflammatory bone disorders is a sterile inflammation of the bone followed by skin pathology. These disorders are synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome, chronic recurrent multifocal osteomyelitis (CRMO), Majeed syndrome, DIRA, PAPA syndrome and cherubism. CRMO and SAPHO are the most common entities. Considering the similarities, a group of authors consider CRMO, which predominately affects children, to be a subtype of the SAPHO syndrome, which most often presents in adolescents or adults.
Aim: To analyze and compare characteristics of patients with CRMO and SAPHO syndrome.
Methods: A retrospective analysis and comparison of information from the medical documentation of patients with CRMO and SAPHO syndrome treated in the Department of immunology and rheumatology of the Pediatrics clinic KBC Zagreb, the Reference center for pediatrics and adolescent rheumatology RH in the period of 2002 – 2015.
Results: In the period of 2002 – 2015 we treated three girls with CRMO, average age of 5 years and three boys with SAPHO syndrome, average age of 16 years at the moment of diagnose. All patients initially had moderately elevated parameters of inflammation. All patients with CRMO had multifocal inflammatory changes of the axial and peripheral skeleton, only in one patient were these changes symmetrical. No skin pathology was noted. They were treated with nonsteroidal anti-inflammatory drugs which led to remission in one, while the other two patients underwent a shorter systemic usage of corticosteroids with the same positive outcome. Only one of the patients had typical scintigraphic findings of affected sternoclavicular joints and sternum while others had inflammatory changes in different locations, including maxilla and mandible. All three patients had extensive acne, two of them as an initial symptom, whereas the third patient had acne two years after initial inflammatory bone changes. Satisfactory control of the disease was accomplished in two patients due to indomethacin or methotrexate, whereas in one patient with TNF antagonists.
Conclusion: CRMO and SAPHO syndrome share a series of characteristics, regarding to a section of recent literature CRMO is considered a children’s form of the SAPHO syndrome which affects mostly adults and adolescents. The results of the comparison of our patiens with said diseases are in compliance with the information from literature. Only the revelation of the jet unknown etiopathogenesis will clarify the relationship between the two most common autoinflammatory bone diserders.
Keywords
autinflamatorne bolesti kostiju
SAPHO sindrom
kronični rekurirajući multifokalni osteomijelitis
CRMO
Keywords (english)
autoinflammatory bone disorders
SAPHO syndrome
chronic recurrent multifocal osteomyelitis
CRMO
Language croatian URN:NBN urn:nbn:hr:105:354036 Study programme Title: Medicine Type of resource Text File origin Born digital Access conditions Open access Terms of use Created on 2017-07-07 07:47:51
