- Causes of the SAPHO team
- SAPHO syndrome: symptoms
- How is the SAPHO team going?
- SAPHO team: research
- SAPHO team: treatment
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SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis, SAPHO syndrome) is a chronic rheumatic disease belonging to seronegative spondyloarthritis. What are the causes and symptoms of SAPHO syndrome? How is his treatment going?
SAPHO syndrome(Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis,SAPHO syndrome ) usually develops in patients between 20 and 20 60 years of age and it affects women and men with the same frequency. The highest incidence in the world is recorded in Japan, while in Europe most cases are reported in France and the Scandinavian countries.
The SAPHO syndrome is characterized by joint inflammation (synovitis), acne (acne), pustulosis, excessive bone formation (hyperostosis) and osteitis.
Causes of the SAPHO team
The reasons for the development of the SAPHO team have not yet been identified. It is suspected that some genetic predisposition may play a role in the initiation of the disease process, as the presence of this syndrome has been described in members of the same family. The influence of severe stress and infections with microorganisms such as Chlamydia or Yersinia is also considered. However, it was not possible to isolate these bacteria from the body fluids and tissues of patients. The only pathogen that can be grown from skin and bone lesions that causes acne-like skin lesions is the anaerobic bacterium Propionibacterium acnes.
Interestingly, about 10% of patients may coexist with inflammatory bowel diseases - Crohn's disease and ulcerative colitis.
SAPHO syndrome: symptoms
The disease image is dominated by changes in the bone and joint system and in the skin. The appearance of skin lesions may precede the involvement of the osteoarticular system - these changes may occur simultaneously or several years after joint and bone symptoms. It is rarely possible to have the presence of general symptoms, most often in the form of fatigue, weight loss or low-grade fever.
The main symptom of SAPHO syndrome is arthritis of the anterior chest wall. It most often includes the sternoclavicular joints and sternum-costal connections. In addition, the inflammatory process may affect the joints of the spine andsacroiliac joints (unilaterally or bilaterally) as well as peripheral joints of the hands and feet.
In the case of arthritis of the sternoclavicular joints and / or the sternoclavicular joints, pain, swelling, increased heat and sometimes redness are characteristic in their area. When the spine is involved, its compression pain and restriction of mobility are noted.
Inflammation of peripheral joints such as wrists, metacarpophalangeal joints, interphalangeal joints of the hands and joints of the feet is manifested by symmetrical swelling and morning stiffness. It is also possible to involve the temporomandibular or knee joints. However, it should be borne in mind that the incidence of peripheral arthritis is much lower than that of the axial skeleton.
How is the SAPHO team going?
In the course of SAPHO syndrome, there may also be recurrent skin lesions that run with periods of exacerbation and remission. They do not always accompany osteoarticular symptoms and are also not necessary for diagnosis. These include psoriasis vulgaris, pustular psoriasis on the palms and plantar surfaces of the feet, ulcerative or concentrated acne, and inflammation of the sweat glands that occurs less frequently.
Plaque psoriasis affects the hairy and hairless skin and nails. In pustular psoriasis, the palmar surfaces of the hands and the plantar surfaces of the feet are affected. It takes the form of red, scaly pustules and vesicles. The course with periods of exacerbations and remissions is also characteristic for her.
Acne is most often located on the skin of the back and chest and is mild, in the form of single eruptions. It is also possible to have a decaying or ulcerative form.
In the SAPHO syndrome, excessive bone formation may also occur, which mainly affects the skeletal system of the anterior chest wall, spine, pelvis and pubic symphysis. Inflammatory lesions also affect the anterior chest wall, pelvis, and vertebrae, but can also occur in the long bones - especially the humerus, tibia, and femur. It is possible to develop inflammation in the flat bones of the skull and the mandible. Inflammatory changes are accompanied by pain, tenderness to touch and increased warmth. The course may also be asymptomatic.
SAPHO team: research
Laboratory tests show accelerated ESR, an increase in the concentration of C-reactive protein (CRP) and leukocytosis - markers of inflammation. Additionally, there is an increase in the activity of alkaline phosphatase and the concentration of alpha2-globulins. There are no sick peoplethe presence of rheumatoid factor or antinuclear antibodies. The HLA-B27 antigen can be demonstrated in approximately 15-30% of cases.
Imaging tests such as X-ray and scintigraphy are also used to diagnose the SAPHO syndrome. In the initial stage of the disease, a chest X-ray shows erosions in the joints accompanied by subchondral sclerosis or signs of periostitis. Later, productive changes and the formation of the so-called bone blocks. In patients with a long course of the disease, sclerotization within the sternum and calcification of the costoclavicular ligaments can be observed.
When the axial skeleton is involved, erosions and reactive sclerotization are visible within the vertebral bodies adjacent to the intervertebral disc. There are also marginal syndesmophytes and perivertebral calcifications. Sclerotization can also occur in the area of the hip and pubic bones.
More than 1/3 of patients have sacroiliitis. Rarely, there are erosions in the peripheral joints of the hands and feet.
The use of skeletal system scintigraphy using technetium (99mTc) enables the demonstration of increased tracer uptake in areas affected by the disease process.
Bone biopsy (bone biopsy) is also performed for diagnostic purposes. In the early stage of the disease, inflammatory cells, monocytes, plasmocytes and individual giant cells are visible in bone marrow preparations. Later on, monocytes and fibrous changes predominate. The cortex layer of the bone is thickened and compacted - as in the case of infectious osteomyelitis. The downloaded content is usually sterile.
SAPHO team: treatment
Treatment initially involves non-steroidal anti-inflammatory drugs and analgesics. In the absence of improvement and persistent inflammatory markers, oral glucocorticosteroids and intra-articular injections are used.
Sulfasalazine is especially recommended for the involvement of sacroiliac joints, the intensification of psoriasis lesions or the coexistence of inflammatory lesions within the intestines. Leflunomide and methotrexate are used in the case of involvement of peripheral joints with the presence of erosions and high activity of the inflammatory process. Also, infliximab and etanercept - anti-TNF-alpha antibodies - have use.
For the treatment of osteolytic lesions, calcitonin is used, which inhibits bone resorption as well as has an analgesic and anti-inflammatory effect. Panidronate by intravenous infusion also has an anti-resorptive effect in the bone. Additionally, it inhibitsactivity of pro-inflammatory cytokines such as IL-1, IL-6 or TNF-alpha.
In the treatment of SAPHO syndrome, one should not forget about physiotherapy treatments, proper physical rehabilitation and psychotherapy.