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- SAPHO syndrome facts
- What is SAPHO syndrome?
- What causes SAPHO syndrome, and what are risk factors for developing it?
- Is SAPHO syndrome related to other joint conditions?
- What are symptoms and signs of SAPHO syndrome?
- How is SAPHO syndrome diagnosed?
- What is the treatment for SAPHO syndrome?
- What is the outlook (prognosis) for patients with SAPHO syndrome?
- Can SAPHO syndrome be prevented?
SAPHO syndrome facts
- SAPHO syndrome is a chronic disorder that involves the skin, bone, and joints.
- SAPHO is an acronym for the combination of synovitis, acne, pustulosis, hyperostosis, and osteitis.
- Some researchers feel that the SAPHO syndrome is related to the group of arthritis conditions which typically affect the spine, called the spondyloarthropathies.
- Treatment of SAPHO syndrome typically involves medications which reduce inflammation.
What is SAPHO syndrome?
SAPHO syndrome is a chronic disorder that involves the skin, bone, and joints. SAPHO is an acronym for the combination of synovitis, acne, pustulosis, hyperostosis, and osteitis.
Synovitis means inflammation of the joint lining (synovium). Typically, this is manifests as warmth, tenderness, pain, swelling, and stiffness of involved joints (arthritis).
Acne is a skin condition featuring tiny areas of inflammation with pus formation at the hair follicles. Acne occurs most commonly on the face and upper back.
Pustulosis is a very inflammatory skin condition resulting in large fluid-filled blister-like areas (pustules), typically on the palms of the hands and/or the soles of the feet. The skin of these areas peels and flakes (exfoliates).
Hyperostosis means abnormal excessive growth of bone. The hyperostosis of the SAPHO syndrome frequently is located at the points of the bone where tendons attach, generally in the chest wall.
Osteitis means inflammation of the bone. Patients with SAPHO syndrome can develop inflammation of the sacroiliac joints (sacroiliitis) as well as inflammation of the spine (spondylitis) which leads to stiffness and pain of the neck and back.
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What causes SAPHO syndrome, and what are risk factors for developing it?
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.
Is SAPHO syndrome related to other joint conditions?
Some researchers feel that the SAPHO syndrome is related to the group of arthritis conditions that typically affect the spine, called the spondyloarthropathies. For further information, please read the Ankylosing Spondylitis and Reactive Arthritis articles.
What are symptoms and signs of SAPHO syndrome?
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.
How is SAPHO syndrome diagnosed?
SAPHO syndrome is diagnosed clinically by identifying the characteristic features of the syndrome including synovitis, acne, pustulosis, hyperostosis, and osteitis. The blood test marker antigen HLA-B27, when present, supports the diagnosis.
What is the treatment for SAPHO syndrome?
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.
What is the outlook (prognosis) for patients with SAPHO syndrome?
To some degree, the outlook for patients with SAPHO syndrome is not predictable but depends on response to medications. When sulfasalazine or methotrexate quiet the symptoms and signs of inflammation, the outlook is optimal.
Can SAPHO syndrome be prevented?
Because the tendency toward developing SAPHO syndrome is inherited, it is not possible to prevent the disease at this time.
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Klippel, John H., et al., eds. Primer on the Rheumatic Diseases. 13th ed. New York: Springer and Arthritis Foundation, 2008.
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acetylsalicylic acidAspirin (Aspirin, Arthritis Foundation Safety Coated Aspirin, Bayer Aspirin, Bayer Children's Aspirin, Ecotrin, and many others) is a NSAID used to treat fever, pain, and inflammation in the body that results from forms of arthritis, and soft tissue injuries. Aspirin is also used for decreasing the risk of heart attacks and strokes. Side effects, drug interactions, pregnancy information, and pregnancy safety information should be reviewed prior to taking any medication.
AcneAcne is a localized skin inflammation as a result of overactivity of oil glands at the base of hair follicles. This inflammation, depending on its location, can take the form of a superficial pustule (contains pus), a pimple, a deeper cyst, congested pores, whiteheads, or blackheads. Treatments vary depending on the severity of the acne.
ArthritisArthritis is inflammation of one or more joints. When joints are inflamed they can develop stiffness, warmth, swelling, redness and pain. There are over 100 types of arthritis, including osteoarthritis, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, lupus, gout, and pseudogout.
ArthroscopyDuring arthroscopy, a tube-like viewing instrument (called an arthroscope) is used to examine the internal structure of a joint for diagnosis or treatment. Arthroscopy is useful when attempting to diagnose or treat various types of arthritis and joint injuries. This surgical procedure may often be performed in an outpatient setting.
Aspirin and Antiplatelet Medications
Aspirin belongs to a drug class called NSAIDs (nonsteroidal anti-inflammatory drugs), and has an important inhibitory effect on platelets in the blood. Platelets are needed in order for blood clots to form. Because aspirin inhibits blood clotting, it is used to reduce the risk of recurrent stroke and near-stroke (transient ischemic attack). Aspirin therapy is used to prevent heart attacks and treat heart attacks. Common side effects of aspirin include:
- Easy bruising
- Ringing in the ears
The U.S. Preventive Services Task Force has recommendations and guidelines for the use of aspirin therapy and heart disease prevention. Aspirin therapy is used for the treatment and prevention of:
- Heart attacks
Heart Attack and Atherosclerosis Prevention
Heart disease and heart attacks can be prevented by leading a healthy lifestyle with diet, exercise, and stress management. Symptoms of heart attack in men and women include chest discomfort and pain in the shoulder, neck, jaw, stomach, or back. Women experience the same symptoms as men; however, they also may experience:
- Extreme fatigue
- Pain in the upper abdomen
Leading a healthy lifestyle with a heart healthy low-fat diet, and exercise can help prevent heart disease and heart attack.
Ibuprofen is a nonsteroidal anti-inflammatory drug (NSAID) used to reduce mild to moderate pain, inflammation, and fever. Ibuprofen works by blocking an enzyme that makes prostaglandin (a hormone-like substance that participates in a variety of body functions), which results in lower levels of prostaglandins in the body. Lower levels of prostaglandins reduce pain, inflammation, and fever.
Ibuprofen is prescribed to treat diseases and conditions that cause mild to moderate pain, fever, and inflammation. For example, Pain from strains and sprains; pain from cuts, scrapes, and puncture wounds; muscle aches and pains; tooth pain; common cold; mild headache; some arthritis conditions; joint pain; and to reduce fever.
Common side effects of ibuprofen include, diarrhea, constipation, nausea, heartburn, belly pain, drowsiness, headaches, tinnitus (ringing in the ears), and mild rash.
More serious side effects and adverse effects include, increased bleeding after injury, stomach ulcers, impaired kidney function, severe allergic reaction (anaphylaxis), blood clots, heart attack, heart failure, and high blood pressure.
The maximum dose prescribed under a doctor's care is 3.2 g daily. Otherwise, the over-the-counter (OTC) maximum daily dose is 1.2 g daily. Dosage depends upon the age, weight, and any current medical conditions of the patient. Several drugs interact with ibuprofen so check with your doctor, pharmacist, or other health care professional with questions in regard to this drug. Doctors don't know if it is safe to take ibuprofen if your are pregnant, therefore it is not recommended if you are pregnant. According to the American Academy of Pediatrics, ibuprofen is safe to take while breastfeeding.
REFERENCE: FDA Prescribing Information.
infliximabInfliximab (Remicade) is a drug prescribed for inflammation of Crohn's disease, rheumatoid arthritis, psoriasis, ankylosing spondylitis, and psoriatic arthritis. Side effects, dosing, drug interactions, and pregnancy safety should be reviewed prior to taking this medication.
methotrexateMethotrexate (Rheumatrex, Trexall) is a drug prescribed to treat cancer, psoriasis, inflammatory diseases of the skin, arthritis (rheumatoid arthritis in adults and children), psoriatic arthritis, polymyositis, lupus, and to induce miscarriage in women with ectopic pregnancies. Side effects drug interactions, dosage, and pregnancy safety information should be reviewed prior to taking this drug.
naproxenNaproxen (Aleve, Anaprox, Naprelan, Naprosyn) is in the class of drugs called nonsteroidal anti-inflammatory drugs (NSAIDs). Naproxen is prescribed for the treatment of mild to moderate pain, inflammation, and fever. Side effects, drug interactions, and pregnancy information should be reviewed prior to taking this medication.
Nonsteroidal Antiinflammatory DrugsNonsteroidal antiinflammatory drugs (NSAIDs) are a class of drugs are used to treat inflammation, mild to moderate pain, and fever. Examples of the most common NSAIDs include: aspirin salsalate (Amigesic), diflunisal (Dolobid), ibuprofen (Motrin), ketoprofen (Orudis), nabumetone (Relafen), piroxicam (Feldene), naproxen (Aleve, Naprosyn,) diclofenac (Voltaren), indomethacin (Indocin), sulindac (Clinoril), tolmetin (Tolectin), etodolac (Lodine), ketorolac (Toradol), oxaprozin (Daypro), celecoxib (Celebrex).
Palmoplantar Pustulosis PicturePalmoplantar pustulosis, also referred to as pustular psoriasis of the palms of the hands. See a picture of Palmoplantar Pustulosis and learn more about the health topic.
Psoriatic ArthritisPsoriatic arthritis is a disease that causes skin and joint inflammation. Symptoms include painful, stiff, and swollen joints, tendinitis, and organ inflammation. Treatment involves anti-inflammatory medications and exercise.
Reactive ArthritisReactive arthritis is a chronic, systemic rheumatic disease characterized by three conditions, including conjunctivitis, joint inflammation, and genital, urinary, or gastrointestinal system inflammation. Inflammation leads to pain, swelling, warmth, redness, and stiffness of the affected joints. Non-joint areas may experience irritation and pain. Treatment for reactive arthritis depends on which area of the body is affected. Joint inflammation is treated with anti-inflammatory medications.
sulfasalazineSulfasalazine (Azulfidine) is a drug prescribed for the treatment of mild to severe ulcerative colitis and the treatment of rheumatoid arthritis. Sulfasalazine (Azulfidine) has also been prescribed "off label" for Crohn's disease and ankylosing spondylitis. Side effects, warnings and precautions, drug interactions, and safety during pregnancy should be reviewed prior to taking this medication.
tolmetintolmetin (Tolectin [Discontinued Brand]) is a nonsteroidal anti-inflammatory drug (NSAID) prescribed for the treatment of inflammation and pain that results from rheumatoid arthritis, juvenile arthritis, or osteoarthritis. Side effects, drug interactions, dosing, and pregnancy safety information should be reviewed prior to taking this medication.