SAPHO Syndrome


SAPHO Syndrome

Description, Causes and Risk Factors:

Synovitis-acne-pustulosis-hyperostosis osteomyelitis.

The acronym SAPHO was first used in 1987 by Dr. Chamot and includes a group of diseases with osteoarticular and cutaneous involvement mainly affecting children and young adults. The etiology of this syndrome is unknown, although the most accepted hypothesis is seronegative arthropathy.

The precise cause of SAPHO syndrome is not known. It is felt that the tendency toward developing SAPHO syndrome can be inherited. Genetic predisposition is suggested by the higher prevalence of HLA-B27, an inherited blood marker, in patients with SAPHO syndrome.

Pathogenesis is clearly of an enthesopathic nature and can ben attributed to an immunopathologic phenomenon. No infectious agent has been found in sterile osteomyelitis of CRMO (chronic recurrent multifocal osteomyelitis). An interesting hypothesis has been put forward by researchers: since in several cases anaerobic hypovirulent germs normally located on the skin were found, propionibacterium acnes (a species of bacteria commonly found in acne pustules, although it occurs in other types of lesions in humans and even as a saprophyte in the intestine, skin, hair follicles, and in sewage) may play an important role as potential antigenic trigger. Thus under special circumstances, there germs may trigger moderate inflammation of the bone marrow, typically immunological with lymphoplasmacellular infiltrates inducing a sclerosing and hyperostosing reaction that may lead to the picture of sclerosing osteomyelitis.

Some researchers feel that the SAPHO syndrome is related to the group of arthritis conditions that typically affect the spine, called the spondyloarthropathies.

Since the introduction of this syndrome by Chamot diagnostic criteria have been established, although there are no specific confirmatory diagnostic tests. The SAPHO syndrome should be suspected on the presence of osteoarticular and/or dermatological clinical manifestations with compatible imaging tests and the implementation of bone scintigraphy acquiring a fundamental role. The dermatologic manifestations, mainly pustulosis, may or may not be associated and may be simultaneous or posterior to the articular symptomatology and may precede these symptoms in 50 % of the cases.

Prevalence of SAPHO syndrome is not known completely because it is a new concept and has mixed clinical findings. It is estimated that its prevalence is not more than 1/10000. Published reports of SAPHO syndrome are generally from France, Germany, Japan, Australia and Scandinavian countries. SAPHO Syndrome can be seen in all ages, but it is more frequent in children and young adults.

Symptoms:

SAPHO syndrome causes inflammation of joints with pain, stiffness, swelling, warmth, and redness. Joints affected can be spinal or away from the spine (peripheral joints) such as the fingers, wrists, or knees. Acne of the skin and pustules of the palms of the hands and soles of the feet are characteristic.

Diagnosis:

Diagnosis of SAPHO syndrome is more often basedof clinical findings. Although many diagnostic criteriafor SAPHO syndrome have been published, there areno acceptable diagnostic criteria. Diagnostic criteriafor SAPHO syndrome were suggested for the first timein 2003 at the 67th annual scientific meeting ofAmerican College of Rheumatology. Suggestedinclusion criteria are: a) bone and/or joint involvementassociated with palmoplantar pustulosis and pustularpsoriasis, b) bone and/or joint involvement associatedwith severe acne, c) in adults isolated sterilehyperostosis/osteitis, d) in children chronic recurrentmultifocal osteomyelitis and, e) bone and/or jointinvolvement associated with chronic bowel diseases.Exclusion criteria are: a) infectious osteitis, b) tumoralconditions of bone and, c) non-inflammatorycondensing lesions of bone.The blood test marker antigen HLA-B27, when present, supports the diagnosis.

Diagnosis can be established after evidence of sterile inflammation of the bone marrow revealed by typical MRI sequences and showing inflammatory bone marrow edema at characterstictic boney areas. Finally hestopatholgic with sterile evidence of a plasma cell sclerotic infalmmatory cells precise the diagnosis.

Treatment:

Treatment of patients with SAPHO syndrome is directed toward the individual symptoms that are present. Generally, treatment involves medications that reduce inflammation in the particular tissues affected. Examples of medications that are used for inflammation include nonsteroidal anti-inflammatory drugs (NSAIDs, such as aspirin, ibuprofen [Advil, Motrin], and naproxen [Aleve]) and cortisone medications (either in the form of topical creams, tablets, or by injection into the involved area). Topical cold applications can also help reduce inflammation in some tissues. For patients with persisting joint symptoms, both sulfasalazine (Azulfidine) and methotrexate (Rheumatrex, Trexall) have been tried with varying degrees of success. Newer biologic medications, including infliximab (Remicade), have also been used successfully.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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