Síndrome SAPHO: entidade rara ou subdiagnosticada?

Abstract

Guerra JG et al. Radiol Bras 2005;38(4):265271265 correlação entre cinco manifestações clínicas, radiológicas e patológicas freqüentemente combinadas, das quais deriva o acrônimo que a denomina: sinovite, acne, pustulose, hiperostose e osteíte (1-5) .Os componentes fundamentais da síndrome, que pode ser entendida como um conjunto de diversas desordens idiopáticas, são a hiperostose e a osteíte inflamatória. Na presença desses achados, o clínico e o radiologista devem considerar a possibilidade diagnóstica da síndrome (2) .As manifestações cutâneas incluem pustulose e formas graves de acne, tais como acne conglobata. As lesões pustulares usualmente se manifestam ou por psoríase ou por pustulose palmoplantar. No entanto, a ausência de alterações cutâneas não exclui o diagnóstico, pois essas lesões OBJETIVO: Descrever os achados clínicos, radiológicos e patológicos da síndrome SAPHO e sugerir que, apesar de ser considerada rara, esta síndrome deve estar sendo subdiagnosticada por clínicos e radiologistas, provavelmente em função do desconhecimento das suas características. MATERIAIS E MÉTODOS: Foi realizado estudo retrospectivo de seis casos confirmados desta síndrome, dando-se ênfase aos achados clínicos (idade, sexo e sintomas) e de imagem (cintilografia óssea, radiografia convencional, tomografia computadorizada e ressonância magnética). RESULTADOS: A manifestação clínica inicial de todos os pacientes foi dor na parede torácica ântero-superior há pelo menos quatro meses. Todos apresentavam achados de imagem de processo inflamatório e/ou osteíte e hiperostose nas articulações da parede torácica ântero-superior. As alterações cutâneas da síndrome, tipo pustulose palmoplantar, estiveram presentes em cinco dos seis pacientes. Em nenhum dos seis casos o diagnóstico foi sugerido na consulta clínica inicial ou na primeira interpretação das imagens feita por radiologistas não especialistas em sistema músculo-esquelético. CONCLUSÃO: Os nossos achados estão de acordo com os descritos na literatura, devendo ser considerado este diagnóstico em todo paciente que apresente quadro doloroso de parede torácica acompanhado de manifestações dermatológicas e/ou osteíte. Unitermos: Síndrome de hiperostose adquirida; Hiperostose esternocostoclavicular; Psoríase; Osteíte.SAPHO syndrome: rare or under-diagnosed? OBJECTIVE: To describe the clinical, imaging and pathological findings of SAPHO syndrome and to point out that although considered rare this syndrome may be under-diagnosed by clinicians and radiologists, probably due to unfamiliarity with its features. MATERIALS AND METHODS: A retrospective study of six cases diagnosed in the last six years, with emphasis on clinical findings (age, sex and symptoms) and imaging findings (bone scintigraphy, conventional x-ray, computed tomography and magnetic resonance imaging) was conducted. RESULTS: The initial clinical presentation in all patients was pain in the upper anterior chest wall for at least four months. Imaging findings of all these patients indicated inflammatory process and/or hy...

Clinical manifestation and diagnostic challenges of SAPHO Syndrome: a case report

“…Some authors propose different diagnostic criteria for SAPHO syndrome (Table 1), with those from 2003 being considered more accurate [1,5]. Diagnosis can be challenging when there are atypical osteoarticular lesions in non-characteristic sites, particularly with no associated skin lesion [7]. Due to its complexity and varied clinical presentation, differential diagnoses may involve several inflammatory, neoplastic, and infectious diseases, like rheumatoid or psoriatic arthritis, Behçet's disease, chronic bacterial osteomyelitis, osteosarcoma, and metastatic tumors [1].…”

“…It mainly affects young and middle-aged adults, predominantly in the average age of 30 and 50, and is more common in women [5,6]. The pathogenesis remains uncertain and includes a multifactorial condition that associates genetic susceptibility, autoimmunity, and infectious components [5,7]. Clinical and imaging findings guide the diagnosis, and with no specific serological marker, pose a challenge in clinical practice [1].…”

Chronic recurrent multifocal osteomyelitis exhibiting predominance of periosteal reaction

“…1 Some studies suggest that CRMO and other diseases such as SAPHO syndrome and Garré's osteomyelitis are variants of the same pathology, with their respective sites, characteristics and prevalent age groups. [1][2][3] It represents up to 2-5% of the cases of osteomyelitis, 2 with an approximate incidence of up to 4/1,000,000 individuals, 4 and average age of disease onset estimated between 8-11 years, predominantly in females (64-83%). 1,[4][5][6] Signs and symptoms include local inflammation with pain and edema, episodes of mild fever, which do not profoundly affect the general condition of the patient, and common asymptomatic lesions (17-56%).…”

“…Histological findings are nonspecific and often similar to those found in septic osteomyelitis, but with no evidence of infection by any microorganism. 1,[2][3][4][5][6][7][8][9][10] Imaging tests such as radiography, CT, magnetic resonance imaging (MRI) and scintigraphy are some of the diagnostic tools. The latter two are most commonly used to rule out other diseases and to detect asymptomatic sites employing whole body screening techniques.…”