SAPHO syndrome mimicking spondylodiscııtis: Case report

Year 2014, Volume: 6 Issue: 3, 1 - 4, 01.12.2014

Nurdan Kotevoğlu Betül Toygar Rahmi Çubuk Hayal Ahmetoğlu Selin Cilli Adil Öncel

Abstract

SAPHO syndome synovitis,acne,pustulosis,hyperostosis, osteitis although involves the skin, it is a disease of muscle, joint and bone. It is more frequent in young or middle age women. The diagnose is difficult in patients without skin involvement.It affects anterior chest wall most frequently,but also involves the spine.The etiopathogenesis is not very clear. Osteitis and hyperostosis are pathognomotic for his disease and show its inflammatory origin taking primarily from bone. Spondylodisciitis in SAPHO syndrome looks like the one in Spondyloartropathy, vertebral end plate erosion encircled with sclerosis, sometimes involves whole vertebral body. Paravertebral ossification reminds DISH with its massive bridging. There is some literature about its possible relation to seronegative spondiloarthropathy. HLAB27 may be positive in 30%.of paitients. At least one of the following critera should be positive for definite diagnosis according to Benhamou: .a- severe acne as skin manifestation b-palmaplantar pustulosis c-hyperostosis with or without dermatosis d- chronic recurrent osteomyelitis like involvement either in spine or peripheral skeleton. with /or without dermatosis . Erosion in vertebral corners diagnosed with MRI is important for diagnosis.In early period low signal intensity on T1 and high signal intensity on T2 reveals inflammation, in advanced cases non-marginal syndesmophytes more than marginal, similar to osteophyetes are more frequent.Vertebral height loss may cause kphosis, absence of abcess or secestr, preservation of paravertebral soft tissue help differantial diagnosis for SAPHO from pyogenic disciitis. The disease has a favourable prognosis, considering it in the differential diagnosis will prevent unnesseary tests.

Keywords

SAPHO, spondylitis, spondylodisciitis

Spondilodiskiti taklit eden SAPHO sendromu: Olgu sunumu

Abstract

SAPHO sinovit,akne,pustulozis,hiperostozis,osteitis sendromu cildi de tutmakla birlikte kas, eklem ve kemik hastalığıdır. Genç, orta yaş grubunda ve kadınlarda daha sık olarak görülür.Cilt bulgularının olmadığı hastalarda tanı güçtür.Göğüs ön duvarını en sık olmakla birlikte, omurgayı da tutabilir. Etyopatogenezi çok net değildir. Osteitik ve hiperostotik lezyonlar bu hastalık için belirleyici özellikte olup, hastalığın temel olarak kemikten kaynaklanan inflamatuar oluşumunu işaret eder. SAPHO sendromunda spondilodiskit Spondiloartropati’de görülene benzer, skleroz ile çevrelenen vertebral plate erozyonu, bazen tüm vertebra gövdesini tutabilir. Paravetebral ossifikasyon masif köprüleşmelerle seyrederek DISH’u hatırlatır. Seronegatif spondiloartropatiler ile muhtemel bağlantısı olduğu şeklinde literatür bildirimleri vardır. HLAB27 %30 hastada bulunabilir. Benhamou kriterlerine göre SAPHO sendromu tanısı için aşağıdaki kriterlerden bir tanesinin olması yeterlidir: a-cilt bulgusu olarak ciddi akne, b-palmoplantar püstülozis, c- dermatozla birlikte veya tek başına hiperostoz, d-kronik rekürrren osteomiyelite benzer tutulum, omurga veya periferik iskelette olabilir dermatoz olsun/ olmasın .MRI da vertebra köşelerinde erozyon da tanı için önemlidir. Erken dönemde T1 de düşük, T2 de artmış sinyal yoğunluğu ile enflamasyon tanınır, ileri evrelerde marjinal sindesmofitlerden çok non-marjinal olanlar, osteofite benzer olan yapılar görülür. Vertebra yüksekliğinin kaybı kifoz oluşturabilir, abse veya sekestr formasyonunun olmaması, paravertebral yumuşak dokunun tutulmaması SAPHO’yu piyojenik spondilitten ayırmaya yardım eder İyi bir prognoza sahip olan hastalığı, ayırıcı tanıda akılda tutmak gereksiz tetkiklerin yapılmasını da önler.

Keywords

Maltepe Üniversitesi, Tıp Fakültesi, Fiziksel Tıp ve Rehabilitasyon A.D.

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