Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO) and chronic recurrent multifocal osteomyelitis (CRMO): Role of imaging in diagnosis

Thakur, Uma; Blacksin, Marcia F.; Beebe, Kathleen S.; Neilson, John C.; Dashefsky, Barry; Tagoylo, Gino

doi:10.1016/j.radi.2011.11.001

Cited by 10 publications

(15 citation statements)

References 14 publications

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“…Therefore, it is no wonder that other studies refute these data and showed that there are no relations between SAPHO and HLA-B27. According to some authors, there is a positive connection with HLA 39 and HLA 61 [ 4 , 17 , 18 ]. Due to some similarities between SAPHO and other autoinflammatory syndromes with a genetic basis and due to familial clustering, several other genes are being studied.…”

Section: Etiologymentioning

confidence: 99%

“…It is seen as bone marrow edema, which shows up as hypointense on T1 and hyperintense on T2 signals in the affected metaphysis. As the disease progresses, hypointense T1 and T2 signals in the medullary space and cortex represent medullary sclerosis and cortical thickening [ 17 ]. Lesions are usually multiple and often symmetrical.…”

Section: Radiological Featuresmentioning

confidence: 99%

“…Lesions are usually multiple and often symmetrical. Involvement of the adjacent epiphysis and altered bone growth are rare [ 17 , 35 ].…”

Section: Radiological Featuresmentioning

confidence: 99%

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SAPHO syndrome: A review

Rukavina

2015

Journal of Children's Orthopaedics

174

167

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IntroductionSynovitis–acne–pustulosis–hyperostosis–osteitis (SAPHO) is an acronym for various osteoarticular and dermatological manifestations that can appear in the same patient. It is a rare syndrome, but since its awareness has increased, there have been more and more such reports in the literature.AimsThe objectives of this review are to summarize the current state of knowledge on pediatric and adult-onset SAPHO syndrome, and to discuss treatment strategies that should be considered.ResultsThe SAPHO syndrome can affect patients of any age, and its etiology is still not known. The syndrome has its cognizable radiological characteristics that are most important in making the diagnosis. There are several diagnostic criteria as well, but they need further validation. No standard treatment protocols are available and current treatment options are not evidenced-based due to the rarity of the syndrome. Therapy is empirical and aimed at easing pain and modifying the inflammatory process. It includes nonsteroidal anti-inflammatory drugs (NSAIDs) as the first-line agents. Antibiotics, corticosteroids, disease-modifying anti-rheumatic drugs, biologicals targeting tumor necrosis factor alpha or interleukin-1, and bisphosphonates have all been used with variable success. Surgery is reserved to treat complications. Even though it is a disease with good long-term prognosis, its treatment remains a challenge and the results are known to be disappointing, especially with the skin component of the disease.ConclusionIt is expected that these patients present at the time of diagnosis and the treatment should be as early, effective, and safe as possible in order to prevent osteoarticular progression and to limit the adverse events associated with pharmacological drugs.

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Section: Etiologymentioning

confidence: 99%

Section: Radiological Featuresmentioning

confidence: 99%

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SAPHO syndrome: A review

Rukavina

2015

Journal of Children's Orthopaedics

174

167

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“…En el periodo temprano de cicatrización se observa esclerosis en el mismo sitio o una mezcla de lesiones osteolíticas y escleróticas, pero, generalmente, en el transcurso de dos años, el hueso recupera su apariencia radiológica normal. Las radiografías también pueden ser normales en estadios tempranos de la enfermedad, por lo que se debe mantener un alto índice de sospecha [1][2][3][4][5]7,10,[29][30][31][32][33] .…”

Section: Estudios Con Imágenesunclassified

Osteomielitis crónica multifocal recurrente: estado del arte

Eusse

Velásquez-Franco

Navas

2016

MedUPB

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RESUMENLa osteomielitis crónica multifocal recurrente es una enfermedad autoinflamatoria que se caracteriza por focos estériles de inflamación ósea que producen dolor recurrente. Es una entidad poco frecuente que tiene como manifestaciones cardinales la presencia de lesiones óseas inflamatorias en las regiones metaepifisiarias de los huesos largos que predomina en miembros inferiores. Esta enfermedad, con frecuencia asociada con sínto-mas sistémicos y lesiones en piel, hace parte del grupo de enfermedades que conforma el síndrome: artritis-dermatitis. Los hallazgos radiológicos más comunes son las áreas de osteolisis rodeadas de un halo esclerótico delgado, sin reacción perióstica asociada o la formación de secuestros óseos. La resonancia nuclear magnética permite hacer una detección precoz de la entidad y facilita su diagnóstico diferencial, principalmente, con infecciones y tumores óseos. El tratamiento está basado en la información obtenida mediante reportes de caso y series de pacientes y consiste en el uso de antiinflamatorios no esteroideos y en casos refractarios, bisfosfonatos y antagonistas del factor de necrosis tumoral alfa. El pronóstico de esta enfermedad, en general, es bueno, con un curso clínico benigno y autolimitado, en la mayoría de los casos. Palabras clave: osteomielitis; inflamación; hiperostosis. ABSTRACTChronic recurrent multifocal osteomyelitis is a sterile autoinflammatory disease characterized by foci of sterile inflammation in the bone that generate recurrent pain. It is a rare entity in which the cardinal clinical manifestation is the presence of inflammatory bone lesions in metaepiphyseal regions of long bones, predominating in lower limbs. The disease is often associated with systemic symptoms and skin involvement making it part of the arthritis-dermatitis syndrome. The most common radiographic finding is osteolysis surrounded by a thin sclerotic halo, without periosteal reaction associated with bone formation or bone sequestrum. Magnetic resonance imaging allows early detection and facilitates the differential diagnosis between osteomyelitis and infections or bone tumors. Treatment is supported mainly by case reports and series of patients and consists of the use of nonsteroidal anti-inflammatory drugs and the use of bisphosphonates or tumor necrosis factor alpha antagonists in refractory cases. The prognosis of this disease is generally good, with a benign and self-limited clinical course in most cases.

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“…[2][3][4]. Uzrok ovog sindroma je nepoznat, ali postoji genetska predispozicija i u nekoliko studija nađena je veća učestalost HLA B27, dok je u drugim studijama nađena veća učestalost HLA39 i 61 [3,[5][6][7], zbog čega je ovaj sindrom svrstan u grupu seronegativnih artropatija. Incidenca i prevalenca ovog sindroma još uvek nije poznata [3].…”

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SAPHO syndrome the therapeutical challenge

Dejanović¹,

Ristić²,

Perić³

et al. 2016

Praxis Med

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Uvod: SAPHO sindrom se karakteriše košanozglobnim i kožnim simptomima, Akronim "SAPHO" je skraćenica za promene na koštanozglobnom sistemu i koži: S-sinovitis; A-akne; P-pustulosis; H-hiperostosis; O-osteitis. SAPHO sindrom sreće se pod 50 različitih imena koja se koriste u literaturi. On je za sada nepoznate etiologije, spada u grupu seronegativnih spondilartopatija. U terapiji SAPHO sindroma koriste se NSAIL, glukokortikoidi, bolest modifikujući lekovi (BML), antibiotici, bisfosfonati i anti TNF-alfa lekovi sa promenljivim uspehom. Prikaz slučaja: Pacijent P.Z. životne dobi 47.g. ženskog pola, dolazi zbog bolova u predelu grudne kosti, propagacije bola u desno rame i jutarnje ukočenost u krstima kraćeg trajanja. Recidivantne kožne promene na dlanovima obostrano u vidu gnojnih pustula, kojima prethodi svrab. Urađena je scintigrafija kostiju: Uočava se jasno pojačano vezivanje radiofarmaka u projekciji desnog sternoklavikulanog zgloba, diskretno jače u oba ramena i oba kolena. PH nalaz: Pustulosis palmoplantaris. U terapiji uveden azithromycin u dozi od 500mg 2x1 nedeljno u sledećih 16 nedelja u dva navrata. Pacijentkinja je nakon jedne godine bez recidiva artritisa i kožnih lezija. Zaključak: Ovaj rad ima za cilj da se proširi znanje o ovom retkom entitetu i da se on lakše prepoznaje. Da se o ovom entitetu pored reumatologa, dermatologa, pedijatara upoznaju i ostale komplementarne grane.

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Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO) and chronic recurrent multifocal osteomyelitis (CRMO): Role of imaging in diagnosis

Cited by 10 publications

References 14 publications

SAPHO syndrome: A review

SAPHO syndrome: A review

Osteomielitis crónica multifocal recurrente: estado del arte

SAPHO syndrome the therapeutical challenge

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