Prominencia esternoclavicular: hiperostosis u osificación interarticular ¿Un cajón de sastre? / Sterno-clavicular prominence: hyperostosis or interarticular ossifiction. ¿it is a ragbag?

Introducción. En la práctica clínica observamos con cierta frecuencia una prominencia esternoclavicular dolorosa, deformidad que constituye el principal signo de dos entidades con baja incidencia, aunque bien definidas: la hiperostosis esterno-costo-clavicular (HECC) y la osificación inter-esterno-costo-clavicular (OIECC). El diagnóstico diferencial de estas afecciones debe incluir la artrosis esterno-clavicular, la enfermedad de Paget, la osteitis condensante, la osteomielitis, la pustulosis, la enfermedad de Friederich, el síndrome de Tietze y el osteoma osteoide. Esto es especialmente importante en los casos en los que la afectación sea unilateral. Material y método. Presentamos una serie de nueve pacientes cuyo motivo de consulta fue una prominencia esterno-costo-clavicular, compatible con HECC u OIECC. El motivo de consulta en la mayoría de los pacientes fue el descartar la presencia de un tumor en esa localización. Resultados. Los estudios radiológicos mostraron un aumento variable tanto de la densidad como de la masa ósea, así como diferentes intensidades de afectación de la articulación esterno-costo-clavicular. Discusión. Descartado un tumor y con un diagnóstico concreto de la causa de la prominencia, generalmente, se considera suficiente con el tratamiento antiinflamatorio en la mayoría de los pacientes. En la práctica clínica parece irrelevante la diferenciación entre HECC y OIECC, ya que el tratamiento y el pronóstico de ambas afecciones son superponibles. La biopsia de la articulación y los procedimientos diagnósticos invasivos pueden ser innecesarios(AU)

Introduction. In clinical practice, we see quite often painful sterno-clavicular prominence, deformity, which is the hallmark of two entities with a low incidence, although well defined hyperostosis cost sterno-clavicular (HECC) and ossification cost inter-sterno-clavicular (OIECC). The differential diagnosis of these conditions, you must include sterno-clavicular arthritis, Paget's disease, condensing osteitis, osteomyelitis, pustulosis, Friederich's disease, Tietze's syndrome and osteoid osteoma. This is especially important in cases where the involvement is unilateral. Material and methods. We present a series of nine patients complaining of a painful sterno- clavicular prominence, compatible with HECC or OIECC. The reason for consultation in most patients was rule out the presence of a tumor in that location. Results. Image studies showed a variable increase both the density and bone mass as well as different intensities of joint involvement sterno-clavicular cost. Conclusion. Ruled out a tumor and a specific diagnosis of these, you do not need aggressive treatment, is generally considered sufficient anti-inflammatory treatment in most patients. In clinical practice, it is irrelevant and OIECC HECC differentiation, since treatment and prognosis of both conditions are similar. The biopsy of the joint and aggressive diagnostic procedures may be unnecessary (AU)

[Case of SAPHO syndrome with significant sternocostoclavicular hyperostosis].

A 59-year-old man with hypertension developed general fatigue, fever, and precordialgia in early March 2007. An antibacterial agent was intravenously administered; however, no improvement in his symptoms or laboratory findings was observed. He had acne and pustulosis, and radiographs and CT of the chest revealed sternocostoclavicular hyperostosis. As a result, the diagnosis was revised to SAPHO syndrome, and he improved with steroid administration. SAPHO syndrome is a condition that is rarely found at the initial examination in the Department of Internal Medicine. We report a case with a discussion of the literature.

The psychological burden of an initially unexplained illness: patients with sternocostoclavicular hyperostosis before and after delayed diagnosis.

BACKGROUND: Sternocostoclavicular hyperostosis (SCCH) is a rare, debilitating, chronic inflammatory disorder of the anterior chest wall due to a chronic sterile osteomyelitis of unknown origin. SCCH is largely underdiagnosed and often misdiagnosed. In individual cases it can remain unrecognized for years. The purpose of this study is twofold. Firstly, to evaluate the psychological condition of SCCH patients, both in the sometimes quite extended pre-diagnostic period between first manifestations and confirmed diagnosis of the disease, and in the current situation. Secondly, to investigate the relationships between the pre-diagnostic and the current psychological conditions of confirmed SCCH patients. METHODS: Structured interviews were held with 52 confirmed SCCH patients. Questionnaires were included to assess posttraumatic stress symptoms, social support, aspects of pain, illness perceptions, self-reported health status, and quality of life. RESULTS: SCCH patients reported stronger posttraumatic stress symptoms, more unfavorable illness perceptions, lower health status, and poorer quality of life than healthy individuals and patients with other diseases or traumatic experiences. Psychological distress in the pre-diagnostic period was associated with unfavorable conditions in the current situation. CONCLUSION: SCCH is an illness with serious psychological consequences. Psychological monitoring of patients with unexplained complaints is recommended as long as a diagnosis has not been reached.

Diagnostic delay in sternocostoclavicular hyperostosis: impact on various aspects of quality of life.

OBJECTIVE: Sternocostoclavicular hyperostosis (SCCH) is a rare, debilitating, chronic inflammatory disorder of the anterior chest wall due to a chronic sterile osteomyelitis of unknown origin, often associated with characteristic skin lesions of palms and soles: pustulosis palmoplantaris. SCCH goes often unrecognized for years before the diagnosis is established and treatment instituted. The objective of this study was to trace the diagnostic paths of patients with SCCH and to investigate the consequences associated with diagnostic delay. METHODS: Data were collected through structured interviews of 52 patients with a clinically, scintigraphically, and radiologically established diagnosis of SCCH. RESULTS: The majority of patients presented with swelling and/or pain in the sternocostoclavicular region and/or limited movement of the shoulder girdle. Pustulosis palmoplantaris was present in approximately 30% of patients. The disease went unrecognized for a median of 3.5 years. Patients were often seen by at least 3 members of the medical profession before the diagnosis was suspected and eventually established. Lack of recognition of the clinical manifestations of the disease and delay in diagnosis were associated with important physical, psychological, and socioeconomic consequences affecting quality of life. CONCLUSION: SCCH remains an ill-recognized disease despite its characteristic clinical features. A low level of awareness of the disorder leads to a delay in diagnosis, which has a significant impact on various aspects of quality of life. Awareness should be raised for this disorder, enabling timely diagnosis and initiation of treatment to prevent the irreversible physical and psychological sequelae associated with the protracted untreated state.

Asymptomatic enlargement of the clavicle: a review of underlying aetiologies.

An asymptomatic enlargement of the medial clavicle is a condition that is often both missed and misdiagnosed. We review the most common causes of an isolated and asymptomatic enlargement of the medial clavicle. Underlying aetiologies include osteoarthritis of the sternoclavicular joint, condensing osteitis, spontaneous dislocation of the sternoclavicular joint, and sternocostoclavicular hyperostosis. Key points in the history and physical examination as well as characteristic radiographic findings are sufficient for correct diagnoses. Treatment is conservative.

Clinical and radiologic evolution of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a single center study of a cohort of 71 subjects.

OBJECTIVE: To assess the basic features and outcomes of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. METHODS: We identified all patients seen in our unit between 1990 and 2008 diagnosed according to the proposed inclusion criteria with SAPHO syndrome, who had a followup of at least 2 years. RESULTS: Seventy-one patients (48 women, 23 men) with SAPHO syndrome were identified. The median disease duration at the end of followup was 10 years (interquartile range [IQR] 7-15 years), and the median followup duration was 11 years (IQR 6-11.5 years). Six patients were diagnosed with Crohn's disease. Fourteen patients had never had cutaneous involvement, but 8 patients presented >1 skin manifestation. Nine patients (13%) presented a limited (<6 months) monophasic disease course, 25 cases (35%) had a relapsing-remitting course, and 37 patients (52%) had an acute painful phase with a prolonged course lasting >6 months. A total of 4% of the patients were HLA-B27 positive. Female sex (odds ratio [OR] 7.2, 95% confidence interval [95% CI] 2.2-22.9) and the presence at onset of anterior chest wall (ACW) involvement (OR 5.7, 95% CI 1.8-18.1), peripheral synovitis (P = 0.0036), skin involvement (OR 10.3, 95% CI 3.4-31.1), and high values of acute-phase reactants (OR 7.7, 95% CI 2.7-22) were correlated with a chronic disease course and involvement of new osteoarticular sites. CONCLUSION: A chronic course is the more common evolution of SAPHO syndrome. Female sex, elevated erythrocyte sedimentation rate and C-reactive protein values, ACW involvement, peripheral synovitis, and skin involvement at the onset seem to be associated with a chronic course.

SAPHO syndrome: misdiagnosed and operated.

SAPHO is a rare disorder that results in synovitis, acne, pustulosis, hyperostosis and osteitis. Patients with this syndrome typically present with musculoskeletal complaints, frequently localized to the anterior chest wall. However, diagnosis can be difficult in case of involvement of only one symptomatic bone without skin lesions. Awareness of SAPHO syndrome is necessary for accurate diagnosis and to prevent inappropriate and unnecessary treatment.

Síndrome SAPHO: entidade rara ou subdiagnosticada? / SAPHO syndrome: rare or under - diagnosed?

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.

A propósito de un caso de hiperostosis esternocostoclavicular / A case of stenocostoclavicular hyperostosis

Se presentan los estudios clínicos e imagenológicos realizados a un paciente con diagnóstico de hiperostosis esternocostoclavicular(AU)

The sternoclavicular syndrome: experience from a district general hospital and results of a national postal survey.

OBJECTIVE: To report our local experience of the sternoclavicular syndrome and sample the experience of other rheumatologists in the UK. METHODS: We studied case records of 23 patients referred to the Southend rheumatology clinic and data obtained from a postal questionnaire survey of British rheumatologists. RESULTS: We describe 58 cases (20 males and 38 females, mean age 47.2 yr). The disease was unilateral in 40 patients. Shoulder and/or arm pain (38 cases) with limitation of shoulder movements was an important presenting feature; other presenting features were anterior chest wall pain (14 cases) and neck pain (15 cases). Peripheral joint involvement was seen in 12 cases. Skin rash was reported in 12 cases (psoriasis, 6; acne, 2; none had pustulosis). No patients had symptoms or signs of sacroiliitis, and HLA-B27 was negative in 22 out of 23 patients. 99Technetium scintiscanning showed increased uptake in the sternoclavicular region in 31/34 patients (91.1%), but not in the sacroiliac areas. Plain radiographs were abnormal in 18 cases (sclerosis, 9; erosions, 2; soft tissue swelling, 2; bony expansion, 5). CT and/or MRI scans (available in 27 cases) showed erosions in 12 and osteitis in 18. Available histology showed a variable picture, including inflammation, bone erosion, sterile osteomyelitis and fibrosis. The majority of patients (45) were treated with non-steroidal anti-inflammatory drugs: 12 received steroids and 10 received disease-modifying anti-rheumatic drugs (methotrexate, 4; sulphasalazine, 6). Follow-up information was available for 38 patients, of whom 14 became asymptomatic and 24 had chronic disease with intermittent flares. CONCLUSION: Sternoclavicular disease is not uncommon in the UK. It can present with pain in the shoulder, neck or anterior chest wall, and may be underdiagnosed. Our results do not show a link with acne or pustulosis. Features of spondyloarthropathies, such as sacroiliitis and HLA-B27 positivity, were rare in this survey.

Sternocostoclavicular hyperostosis in children: a report of eight cases.

OBJECTIVE: The aim of this paper is to clarify the clinical and radiologic features of sternocostoclavicular hyperostosis by reviewing eight previously unpublished cases in children, identifying its similarities to chronic recurrent multifocal osteomyelitis and the differences between the pediatric and adult population affected with sternocostoclavicular hyperostosis. Appropriate imaging workup will obviate unnecessary diagnostic and therapeutic procedures. MATERIALS AND METHODS: We investigated the clinical and imaging features of sternocostoclavicular hyperostosis in eight children (seven girls and one boy) and compared those features with the characteristic features of chronic recurrent multifocal osteomyelitis and sclerosing Garré's osteomyelitis to determine if sternocostoclavicular hyperostosis can justifiably be classified as a separate entity. All patients underwent one or more bone biopsies to determine the cause of the bone lesion(s). RESULTS: Seven of the eight patients had involvement of the clavicle. Five of the eight patients had associated distant involvement in the pelvis, femur, tibia, fibula, talus, or sacroiliac joints. Except for predominant localization in the anterior chest wall, the symptoms, the clinical and imaging features, and the results of biopsy and histopathologic examination resemble those of chronic recurrent nonspecific sclerosing osteomyelitis. No skin lesion and no causative organism was found in any of the cases. CONCLUSION: Sternocostoclavicular hyperostosis is a descriptive term used to designate a form of chronic sclerosing osteomyelitis. Its only distinctive feature is localization on one or more sites of the anterior chest wall.

Palmoplantar pustulosis associated with sternocostoclavicular hyperostosis.

Palmoplantar pustulosis associated with sternocostoclavicular hyperostosis is characterized by asymmetrical and nonerosive involvement of the sternocostoclavicular joint, spine, and peripheral joints in association with palmoplantar pustulosis. This seronegative arthrosteitis has been most frequently described in the rheumatologic literature. Male and female patients are affect equally, but there tends to be a higher prevalence in Japan and Scandinavian countries. We present a case of 50-year-old man who presented with palmoplantar pustulosis that preceded his sternocostoclavicular hyperostotic symptoms by 1 to 2 years.