SAPHO Syndrome
Encyclopedia
SAPHO syndrome includes a variety of inflammatory bone disorders
that may be associated with skin changes. These diseases share some clinical, radiologic, and pathologic characteristics.
An entity initially known as Chronic recurrent multifocal osteomyelitis
(CRMO) was first described in 1972. Subsequently, in 1978, several cases of CRMO were associated with blisters on the palms and soles (palmoplantar pustulosis
). Since then, a number of associations between skin conditions and osteoarticular disorders have been reported under a variety of names, including sternocostoclavicular hyperostosis
, pustulotic arthro-osteitis, and acne-associated spondyloarthropathy
. The term SAPHO was coined in 1987 to represent this spectrum of inflammatory bone disorders that may or may not be associated with dermatologic pathology.
Spine (33% of patients): Segmental, usually involving the thoracic spine. The four main presentations include spondylodiscitis, osteosclerosis, paravertebral ossifications, and sacroiliac joint involvement.
Long bones (30% of patients): usually metadiaphyseal and located in the distal femur and proximal tibia. It looks like chronic osteomyelitis but will not have a sequestrum or abscess.
Flat bones (10% of patients): mandible and ilium.
Peripheral arthritis has been reported in 92% of cases of SAPHO as well.
In children, the SAPHO syndrome is most likely to affect the metaphysis
of long bones in the legs (tibia, femur, fibula), followed by clavicles and spine.
Treatment with tumor necrosis factor alpha antagonists (TNF inhibitor
s) have been tried in few patients with limited success. Other drugs which are used in psoriatic arthritis
, to which SAPHO syndrome is closely related, have also been used in this condition. They include NSAIDs, corticosteroids, sulfasalazine, methotrexate
, ciclosporin
and leflunomide
.
Some patients have responded to antibiotics. The rationale for their use is that Propionibacterium acnes
, a bacterium known for its role in acne, has been isolated from bone biopsies of SAPHO patients.
Osteitis
Osteitis is a general term for inflammation of bone. More specifically, it can refer to one of the following conditions:* Alveolar osteitis or "dry socket"* Condensing osteitis * Osteitis deformans...
that may be associated with skin changes. These diseases share some clinical, radiologic, and pathologic characteristics.
An entity initially known as Chronic recurrent multifocal osteomyelitis
Chronic recurrent multifocal osteomyelitis
Chronic recurrent multifocal osteomyelitis is also known as Chronic recurring multifocal osteomyelitis.-CRMO :Chronic recurrent multifocal osteomyelitis is a rare condition , in which the bones have lesions, inflammation, and pain. Its definition is evolving...
(CRMO) was first described in 1972. Subsequently, in 1978, several cases of CRMO were associated with blisters on the palms and soles (palmoplantar pustulosis
Pustulosis
Pustulosis is highly inflammatory skin condition resulting in large fluid-filled blister-like areas - pustules. Pustulosis typically occurs on the palms of the hands and/or the soles of the feet. The skin of these areas peels and flakes ....
). Since then, a number of associations between skin conditions and osteoarticular disorders have been reported under a variety of names, including sternocostoclavicular hyperostosis
Hyperostosis
Hyperostosis is an excessive growth of bone. It may lead to exostosis. It occurs in many musculoskeletal disorders.-See also:* Infantile cortical hyperostosis* SAPHO syndrome* Hyperostosis frontalis interna...
, pustulotic arthro-osteitis, and acne-associated spondyloarthropathy
Spondyloarthropathy
Spondyloarthropathy is any joint disease of the vertebral column. Spondyloarthropathy with inflammation is called spondylarthritis. In contrast, spondylopathy is a disease of the vertebra itself, but many conditions involve both spondylopathy and spondyloarthropathy...
. The term SAPHO was coined in 1987 to represent this spectrum of inflammatory bone disorders that may or may not be associated with dermatologic pathology.
Definition
- SynovitisSynovitisSynovitis is the medical term for inflammation of the synovial membrane. This membrane lines joints which possess cavities, known as synovial joints. The condition is usually painful, particularly when the joint is moved. The joint usually swells due to synovial fluid collection.Synovitis may...
- AcneAcne vulgarisAcne vulgaris is a common human skin disease, characterized by areas of skin with seborrhea , comedones , papules , pustules , Nodules and possibly scarring...
— commonly involving the face and upper back. - PustulosisPustulosisPustulosis is highly inflammatory skin condition resulting in large fluid-filled blister-like areas - pustules. Pustulosis typically occurs on the palms of the hands and/or the soles of the feet. The skin of these areas peels and flakes ....
— usually involving the palms of the hands and/or soles of the feet (palmo-plantar pustulosis). - HyperostosisHyperostosisHyperostosis is an excessive growth of bone. It may lead to exostosis. It occurs in many musculoskeletal disorders.-See also:* Infantile cortical hyperostosis* SAPHO syndrome* Hyperostosis frontalis interna...
- OsteitisOsteitisOsteitis is a general term for inflammation of bone. More specifically, it can refer to one of the following conditions:* Alveolar osteitis or "dry socket"* Condensing osteitis * Osteitis deformans...
Radiologic Findings
Anterior chest wall (most common site, 65–90% of patients): Hyperostosis, sclerosis and bone hypertrophy especially involving the sternoclavicular joint, often with a soft tissue component.Spine (33% of patients): Segmental, usually involving the thoracic spine. The four main presentations include spondylodiscitis, osteosclerosis, paravertebral ossifications, and sacroiliac joint involvement.
Long bones (30% of patients): usually metadiaphyseal and located in the distal femur and proximal tibia. It looks like chronic osteomyelitis but will not have a sequestrum or abscess.
Flat bones (10% of patients): mandible and ilium.
Peripheral arthritis has been reported in 92% of cases of SAPHO as well.
In children, the SAPHO syndrome is most likely to affect the metaphysis
Metaphysis
The metaphysis is the wider portion of a long bone adjacent to the epiphyseal plate. It is this part of the bone that grows during childhood; as it grows, it ossifies near the diaphysis and the epiphyses...
of long bones in the legs (tibia, femur, fibula), followed by clavicles and spine.
Treatment
Bisphosphonate therapy has been suggested as a first-line therapeutic option in many case reports and series.Treatment with tumor necrosis factor alpha antagonists (TNF inhibitor
TNF inhibitor
Tumor necrosis factor promotes the inflammatory response, which in turn causes many of the clinical problems associated with autoimmune disorders such as rheumatoid arthritis, ankylosing spondylitis, Crohn's disease, psoriasis, hidradenitis suppurativa and refractory asthma. These disorders are...
s) have been tried in few patients with limited success. Other drugs which are used in psoriatic arthritis
Psoriatic arthritis
Psoriatic arthritis is a type of inflammatory arthritis that, according to the National Psoriasis Foundation, will develop in up to 30 percent of people who have the chronic skin condition psoriasis...
, to which SAPHO syndrome is closely related, have also been used in this condition. They include NSAIDs, corticosteroids, sulfasalazine, methotrexate
Methotrexate
Methotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...
, ciclosporin
Ciclosporin
Ciclosporin , cyclosporine , cyclosporin , or cyclosporin A is an immunosuppressant drug widely used in post-allogeneic organ transplant to reduce the activity of the immune system, and therefore the risk of organ rejection...
and leflunomide
Leflunomide
Leflunomide is a medication of the DMARD type, used in active moderate to severe rheumatoid arthritis and psoriatic arthritis. It is a pyrimidine synthesis inhibitor.-Basic chemical, pharmacological, and marketing data:...
.
Some patients have responded to antibiotics. The rationale for their use is that Propionibacterium acnes
Propionibacterium acnes
Propionibacterium acnes is a relatively slow growing, typically aerotolerant anaerobic gram positive bacterium that is linked to the skin condition acne; it can also cause chronic blepharitis and endophthalmitis, the latter particularly following intraocular surgery...
, a bacterium known for its role in acne, has been isolated from bone biopsies of SAPHO patients.