[SAPHO syndrome : An overview and nosological differentiation of 35 disease cases]. / SAPHO-Syndrom : Ein Überblick und nosologische Differenzierung von 35 Krankheitsfällen.

The SAPHO syndrome is not a single entity but an inhomogeneous, nosologically heterogeneous complex of symptoms with unknown etiology and heterogeneous pathogenesis. Clinically subacute, recurrent or chronic disease processes and a common skin-bone association (skibo disease) can be found. Under the acronym SAPHO, chronically recurrent multifocal osteomyelitis (CRMO) is the most common disease that can occur in youth as well as adolescence. Spondylarthritis hyperostotica pustulo-psoriatica with the triad palmoplantar pustulosis, sternoclavicular hyperostosis and ossifying spinal manifestations is the most common SAPHO form found in adults. Abortive disease forms are the inflammatory anterior chest wall syndrome, extended sternoclavicular hyperostosis syndrome of the clavicle bone, acne CRMO and acne spondylarthritis. The SAPHO disease usually heals with a relatively favorable prognosis but there are also unfavorable courses with functional limitations. The diagnosis should be made based on clinical examination, imaging (x-ray, scintigraphy, magnetic resonance imaging) and/or histological bone biopsy analysis. Treatment should be interdisciplinary. Antibiotic treatment is obsolete. This article provides an overview of the SAPHO syndrome and a clinical-rheumatological imaging differentiation as well as classification of 35 cases at first presentation.

A Rare Case of Nontraumatic Thoracic Compression Fracture in a 28-Year-Old Man.

ABSTRACT: A 28-yr-old African American man with a history of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome, tobacco use, and sickle cell trait was referred to a physiatrist at a multidisciplinary spine center with a 1-yr history of nontraumatic thoracic back pain that had significantly worsened over the previous 4 wks. In the context of recurrent infections requiring hospital admissions and the patient's immunosuppressed status, magnetic resonance imaging of his thoracic spine was obtained, showing an acute or subacute anterior compression deformity of the T7 vertebral body. He was subsequently provided with a hyperextension brace, physical therapy referral, and a trial of intranasal calcitonin. The patient reported significant improvement in pain at his 3-mo follow-up appointment and continued to show pain and functional improvement in physical therapy sessions up to 6 mos later.

Síndrome SAPHO: Presentación de un caso clínico / SAPHO syndrome. A case report

El síndrome SAPHO incluye cinco entidades: Sinovitis, Acné, Pustulosis, Hiperostosis y Osteítis. Su característica principal es la asociación de múltiples afecciones osteoarticulares inflamatorias con trastornos cutáneos específicos. A propósito del caso clínico de un adolescente de 15 años con síndrome SAPHO, nos propusimos poner en conocimiento de la comunidad científica la importancia del diagnóstico precoz y el tratamiento acertado de esta patología. Las enfermedades de baja prevalencia han originado problemas diagnósticos en la práctica clínica; en el caso del síndrome SAPHO, tiene una demora diagnóstica promedio de ocho meses. El diagnóstico es fundamentalmente clínico. Cabe destacar que es un síndrome generalmente benigno, autolimitado y crónico, con períodos de exacerbaciones y remisiones. La antibioticoterapia y los procedimientos invasivos no son de elección. Nivel de Evidencia: IV

SAPHO syndrome involves synovitis, acne, pustulosis, hyperostosis, and osteitis. Its most important characteristic is the association of osteoarticular inflammatory conditions with specific skin disorders. We present a 15-year-old boy with SAPHO syndrome. The purpose of this paper is to raise awareness within the medical community of the importance of an early diagnosis and correct treatment. Low prevalence diseases have generated diagnostic problems in clinical practice. Delay in diagnosis of SAPHO syndrome averages 8 months. Diagnosis is mainly clinical. This is a generally benign, selflimited and chronic syndrome, with exacerbations and remissions. Antibiotic therapy and invasive procedures are not a choice. Level of Evidence: IV

Clinical heterogeneity of SAPHO syndrome: challenging diagnose and treatment.

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare disease which is often misdiagnosed and under-recognized, because of its peculiar and heterogeneous clinical presentation. Its main features consist of cutaneous and osteoarticular manifestations, the latter affecting more often the anterior chest wall and having typical radiologic findings. There are no validated diagnostic criteria for SAPHO and no guidelines for treatment, due mainly to its rarity; as a consequence, therapy is empirical and aimed to control pain and modifying inflammatory process. To date, the use of anti-TNF agents has been proved to be a valid alternative for patients unresponsive to conventional treatments, such as NSAIDs, corticosteroids, DMARDs and biphosphonates. The clinical heterogeneity of the disease, possibly due to differences in pathogenic mechanism of different manifestations, is challenging for both diagnosis and treatment, which should aim to control both skin and bone involvement in different clinical subsets. Here, we summarize the current status of knowledge about the SAPHO syndrome and present two cases of patients with very different disease manifestations, suggesting the need for personalized treatment.

Síndrome de Sapho: relato de caso clínico / SAPHO syndrome: clinical case report

El síndrome de SAPHO es una condición relativamente rara, la cual designa varios desordenes que gana su nombre del epónimo por la sinovitis, acné, pustulosis, hiperostosis, osteítis. Relato de caso. Masculino, 15 años de edad, blanco, estudiante que hace dos años se encuentra en tratamiento por acné conglobata, que hace dos meses evoluciona con artralgia en pierna izquierda, tarsitis derecha y lumbalgia inflamatoria. Al examen físico se encuentra, palidez cutánea, pies invertidos y planos, rodillas en valgo, sacroileítis acentuada a la izquierda, tarsitis derecha, dolor en entesis (calcáneos) piel con lesiones cutáneas acneiformes extensas en rostro, dorso y tórax anterior. Exámenes complementarios: anemia normocitica y normo crómica, RX: sacro iliacas con señales de sacroileítis bilateral con psuedo alargamiento en la parte izquierda. USG de los pies normales, fondo de ojo: normal, HLAB27: negativa. Se inició indometacina y fisioterapia. Discusión: cuadro clínico de acné, artropatía inflamatoria característica del síndrome SAPHO, tratamiento conservador.

SAPHO syndrome is a relatively rare condition which designates several disorders that gain its name from the eponymous by synovitis, acne, pustular, hyperostosis, osteitis. Case report. Male, 15 years old, white, student who two years ago is in treatment for acne conglobata, who two months ago evolves with left leg arthralgia, right tarsitis and inflammatory low back pain. Physical examination includes skin paleness, inverted and flat feet, valgus knees, left sore sacroiliitis, right tarsitis, pain in entesis (calcaneus) skin with extensive acneiform skin lesions on face, dorsum and anterior thorax. Complementary examinations: normocytic anemia and normal chromosome, RX: iliac sacrum with signs of bilateral sacroiliitis with psuedo elongation in the left side. USG of normal feet. Fundus of eye: normal, HLAB27: negative. Indomethacin and physiotherapy were started. Discussion: clinical picture of acne, inflammatory arthropathy characteristic of SAPHO syndrome, conservative treatment.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome - A challenging diagnosis not to be missed.

SAPHO syndrome manifests as chronic inflammation of bones and joints, which may or may not be accompanied by skin changes. The term SAPHO is an acronym that stands for synovitis, acne, pustulosis (usually palmoplantar), hyperostosis and osteitis. The bones most commonly affected are those in the anterior chest wall (mainly the sternum, clavicles and sternocostoclavicular joints), the vertebrae and the sacroiliac joints, but peripheral and flat bones may also be involved, especially in children. There are no validated diagnostic criteria for SAPHO, and diagnosis is based on clinical and radiological findings. One of the main challenges in diagnosis is that the clinical features may occur many years apart. Additionally, patients may not develop all manifestations. Delayed diagnosis, as a result of a lack of awareness of SAPHO, can lead to patients suffering ongoing pain and disfiguring skin manifestations. One theory is that Propionibacterium acnes (isolated from bone biopsies in many SAPHO patients) triggers an auto-immune mediated chronic inflammation in genetically predisposed individuals. Treatment involves the use of nonsteroidal anti-inflammatory drugs, intra-articular steroids, bisphosphonates and biologicals. The course of SAPHO is often prolonged but, despite the challenges in diagnosis and treatment, the long-term prognosis is good.

[SAPHO syndrome]. / Le syndrome SAPHO.

SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome is a rare entity characterized by the association of heterogeneous osteoarticular and cutaneous manifestations that have for common denominator an aseptic inflammatory process. The etiopathogeny of this disease is still a matter of debate. Although it has been related to the spondylarthritis family, an infectious origin is suggested. Diagnosis is based on the presence of at least one of the three diagnostic criteria proposed by Kahn. The treatment includes NSAIDs, antibiotics, corticosteroids, methotrexate and more recently the bisphosphonates and the TNFα inhibitors.

The inflammasomes in autoinflammatory diseases with skin involvement.

During the past years, significant progress in the understanding of the complexity, regulation, and relevance of innate immune responses underlying several inflammatory conditions with neutrophilic skin involvement has been made. These diseases belong to the novel class of autoinflammatory diseases, and several are caused by mutations in genes regulating the function of innate immune complexes, termed inflammasomes, leading to enhanced secretion of the proinflammatory cytokine IL-1ß. Consequently, targeting of IL-1ß has proven successful in the treatment of these diseases, and the identification of related pathogenic mechanisms in other more common skin diseases characterized by autoinflammation and neutrophilic tissue damage also provides extended opportunities for therapy by interfering with IL-1 signaling.

Evaluation and treatment of musculoskeletal chest pain.

This article summarizes the evaluation and treatment of musculoskeletal causes of chest pain. Conditions such as costochondritis, rib pain caused by stress fractures, slipping rib syndrome, chest wall muscle injuries, fibromyalgia, and herpes zoster are discussed, with emphasis on evaluation and treatment of these and other disorders. Many of these conditions can be diagnosed by the primary care clinician in the office by history and physical examination. Treatment is also discussed, including description of manual therapy and exercises as needed for some of the conditions.

Autoinflammatory bone diseases.

Autoinflammatory bone disease is a new branch of autoinflammatory diseases caused by seemingly unprovoked activation of the innate immune system leading to an osseous inflammatory process. The inflammatory bone lesions in these disorders are characterized by chronic inflammation that is typically culture negative with no demonstrable organism on histopathology. The most common autoinflammatory bone diseases in childhood include chronic nonbacterial osteomyelitis (CNO), synovitis, acne, pustulosis, hyperostosis, osteitis syndrome, Majeed syndrome, deficiency of interleukin-1 receptor antagonist, and cherubism. In this article, the authors focus on CNO and summarize the distinct genetic autoinflammatory bone syndromes.