Radiologists need to be aware of secondary central venous stenosis in patients with SAPHO syndrome.

OBJECTIVES: We aimed to define central venous stenosis (CVS) caused by sternocostoclavicular hyperostosis as a feature of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome on routine contrast-enhanced computed tomography (CT) images. The relationship between SAPHO syndrome and CVS without venous thrombosis caused by anterior chest wall compression has not been investigated. Therefore, the present study evaluated CVS in patients with SAPHO syndrome at our hospital. METHODS: We retrospectively reviewed contrast-enhanced CT images of ten patients with suspected or diagnosed SAPHO syndrome between January 2007 and November 2015. The patients were assessed by contrast-enhanced CT using 16-, 64- or 128-detector row scanners. Two radiologists independently assessed the presence of CVS or obstruction and SAPHO syndrome in a retrospective review of CT images. RESULTS: Six of the ten patients had findings of CVS with SAPHO syndrome. The mean diameter and patency rate at the site of CVS were 1.88 mm and 27.2%, respectively. Stenosis was more significant in terms of the mean diameter of CVS sites than of stenotic sites that crossed the anteroposterior vein (p < 0.05). CONCLUSIONS: Radiologists who routinely assess contrast-enhanced CT images should be aware that sternocostoclavicular hyperostosis with SAPHO syndrome could cause secondary CVS. KEY POINTS: • SAPHO syndrome can cause central venous stenosis. • Radiologists should consider central venous stenosis in patients with SAPHO syndrome. • Early diagnosis of central venous stenosis due to SAPHO syndrome is challenging.

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)

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.

Tonsillar focal infectious disease involving IgA nephropathy, pustulosis, and ossification.

We report a rare case of IgA nephropathy (IgAN), that was considered as showing tonsillar focal infection, involving pulmoplantar pustulosis (PPP), and sternocostoclavicular hyperosteosis (SCCH). A 53-year-old man with a 3-year history of PPP had hematuria and proteinuria, and he sometimes had anterior chest pain. He was also diagnosed with IgAN and SCCH. We performed tonsillectomy as a treatment. The tonsillectomy was done with the patient under general anesthesia, and this treatment was followed by steroid therapy. Interestingly, all the symptoms of IgAN, PPP, and SCCH were alleviated 6 months after the tonsillectomy. Thus, tonsillectomy and steroid therapy may be effective and could be considered as treatment for these diseases.

Rapid pain relief and remission of sternocostoclavicular hyperostosis after intravenous ibandronate therapy.

Sternocostoclavicular hyperostosis (SCCH) is an infrequent but painful, localized disturbance of bone metabolism of unknown etiology. The diagnosis of SCCH is generally one of exclusion, and it is therefore frequently missed or delayed, leaving patients with pain that frequently fails to respond to standard analgesic therapy. Consequently, SCCH leads to significantly impaired quality of life. Characteristic increased localized bone turnover and inflammatory osteitis provide a strong rationale for using intravenous bisphosphonates to treat the condition. We report on three patients with long-standing, treatment-refractory SCCH in whom intravenous ibandronate injections (a single administration of 4 mg followed by 2 mg every 3 months for up to a year) produced prompt, dramatic, persistent pain relief and resolution of the other symptoms of the disease. We also review recent evidence suggesting that SCCH is more common than generally believed and that technetium-99 bone scanning can aid in making an accurate diagnosis.

[Isolated bilateral Sternocostoclavicular Hyperostosis (SCCH) with a pathological clavicular fracture--a 10-year follow-up]. / Isolierte, bilaterale sternokostoklavikuläre Hyperostose (SCCH) mit pathologischer Klavikulafraktur--Ein Verlauf über 10 Jahre.

The presented case demonstrates the clinical and radiological course of a woman suffering from bilateral sternocostoclavicular hyperostosis (SCCH) from early changes to a spontaneous clavicula fracture after 10 years. SCCH is characterised by a chronic recurrent painful swelling of the sternoclavicular region due to an aseptic inflammation and hyperostosis of the clavicula, sternum, upper ribs and the adjacent soft tissues. Frequently the picture is accompanied by cutaneous and other skeletal symptoms. The disease can be part of the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. The chronic process is represented by the typical "bull horn sign" in a bone scan. NSAIDS should represent the first line treatment.

Stress fracture of the clavicle associated with sternocostoclavicular hyperostosis.

We report a case of stress fracture of the clavicle associated with sternocostoclavicular hyperostosis. A 60-year-old man sustained a stress fracture of the right clavicle with no history of trauma. On radiography, hyperostosis of the anterior chest wall and ankylosis of the sternoclavicular joint were evident in addition to the fracture. Fracture healing was uneventful after 2.5 months. Ankylosis of the sternoclavicular joint may have caused increased stress at the midshaft of the clavicle by daily activity or minor trauma. Such a fracture is a rare complication of sternocostoclavicular hyperostosis.

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.

[SAPHO syndrome: common denominator for various bone and skin diseases]. / SAPHO-syndroom: gemeenschappelijke noemer voor enkele bot- en huidziekten.

The acronym 'SAPHO' stands for synovitis, acne, pustulosis, hyperostosis and osteitis. Three subtypes of SAPHO can be distinguished which have the following features in common: sternoclavicular hyperostosis and sterile inflammatory lesions in both bone and skin. These subtypes are: sternocostoclavicular hyperostosis, chronic recurring multifocal osteomyelitis, and pustular arthro-osteitis. Hyperostosis and osteitic lesions may be similar to those seen in malignant bone tumours. Synovitis generally does not lead to bone erosions and one-third of the patients develop sacroiliitis. The SAPHO syndrome and seronegative spondyloarthropathy share some common features (a higher prevalence of the HLA-B27 antigen, occurrence of sacroiliitis and a higher incidence of chronic inflammatory bowel disease and psoriasis). Aetiology and pathogenesis of SAPHO are unknown; prognosis is good. The SAPHO syndrome often runs a protracted course, with intermittent relapses and remissions without resulting serious disability. Treatment is aimed only at symptomatic relief and mainly consists of analgetics and nonsteroidal antiinflammatory drugs.

Acquired hyperostosis syndrome--AHYS--(sternocostoclavicular hyperostosis, pustulotic arthro-osteitis, SAPHO-syndrome): bone scintigraphy of the anterior chest wall.

The objective of this report is to provide a description of diagnostically significant scintigraphically recognizable sites and patterns of acquired hyperostosis syndrome (AHYS) on the anterior chest wall (ACW), which is involved in 82% of AHYS patients. In 49/90 of our own AHYS patients, planar bone scans of the ACW were performed with the gamma camera, applying an average of 650 MBq of 99mTc-phosphate complexes. In addition, 53 atraumatic patients with extrathoracic cancer were available for routine whole-body scintigraphy. None of these patients had increased uptake identifiable as metastasis clinically or by imaging modalities in either the ACW or the rest of the skeleton. The scintigraphic involvement of the various morphological ACW structures is described in AHYS. Moreover, attention is called to the diagnostic significance of focal hyperactivities at the anterior end of the 2nd-8th rib of adults, which are in the 5th place with respect to their frequency in AHYS. The diagnostic significance of sternocostal-joint involvement in AHYS can likewise be recognized by bone-scan scintigraphy and will be discussed. Bone scintigraphy is more sensitive than radiomorphological imaging in AHYS. This, however, only applies under three conditions. 1. The increased radiotracer uptake in the upper sternocostoclavicular region must be assessed on both the anterior and the posterior view of the ACW scan. 2. In addition to the anterior view of the routine scintiscan, further anterior scans with reduced scan time of the gamma camera are usually necessary. This ensures better visibility of the involvement of certain morphological structures that are important for AHYS diagnosis. Moreover, a statement can be made about the inflammatory ossifying activity/inactivity of the AHYS on ACW.3. Increased radionuclide uptake in the manubrium sterni and corpus sterni on the anterior scan should be verified by additional lateral or oblique scans of the thorax (sternum).

Osseous manifestations of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome.

The SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a complex group of disorders characterized by peculiar bone lesions, most commonly involving the anterior chest wall, and sometimes accompanied by dermatologic manifestations. The clinical and radiographic features of this syndrome are well described, but few studies have examined the histologic features of the bone lesions. We describe the clinical, radiographic, and histologic features of the osseous lesions encountered in eight patients with a clinical diagnosis of SAPHO syndrome. The patients included five female and three male patients ranging in age from 5 to 63 years (mean, 35.3 years). The most common clinical presentation was pain related to the sites of osseous involvement. Two patients also had some form of pustular dermatosis. The radiographic features of the osseous lesions varied but often suggested the possibility of a neoplasm. Nine pathologic specimens were available for review, five from the clavicle, two from the first rib, one from the calcaneus/cuboid, and one from the tibia. The histologic features varied but seemed related to the duration of the patients' musculoskeletal symptoms. Early lesions contained acute inflammation, edema, and prominent periosteal bone formation, histologically indistinguishable from ordinary bacterial osteomyelitis, whereas late lesions demonstrated markedly sclerotic bone trabeculae with prominent marrow fibrosis and only mild chronic inflammation; one of these biopsies appeared virtually identical to Paget's disease. One biopsy was performed after an intermediate duration of symptoms and contained prominent chronic inflammation only. The histologic findings in SAPHO are variable and nonspecific and may depend on the duration of disease, but it is important to recognize the spectrum of histologic changes possible in the syndrome and to realize that clinicopathologic correlation is necessary to avoid misdiagnosis and unnecessary long-term antibiotic therapy.

Sternocostoclavicular hyperostosis presenting with thoracic sinus formation.

Sternocostoclavicular hyperostosis (SCCH) is a condition which is well described in the Japanese literature but is rare in Western Europe. It is characterised by pain and swelling in the upper anterior part of the chest, which tends to be progressive. A patient is described with bilateral chronic discharging sinuses over the anterior ends of the clavicles in whom the diagnosis appeared to be one of SCCH.

[Reflexions on the Tietze syndrome. Clinical contribution]. / Considerazioni sulla sindrome di Tietze. Contributo clinico.

The authors report 4 cases of Tietze's syndrome. In 3 of them the syndrome onset during a rheumatic disease (psoriatic arthritis, sternoclavicular hyperostosis). It is thus necessary to distinguish between the idiopathic of unknown ethiology and the secondary form due to, a well defined disease, in 3 cases observed.