SAPHO Syndrome

What is SAPHO Syndrome?

The disorder called SAPHO syndrome is a chronic condition that affects several parts of the body. Each letter in its name represents a symptom that is present in patients who are afflicted with this disease ^{[1, 2]}.

The name SAPHO is an acronym for the following symptoms: Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis. The symptoms typically manifest in the bone, joints and skin.

Etiology & Risk Factors

The exact cause of this condition is still unknown. There are evidences that show the increased likelihood of developing this syndrome is inherited. Other researchers associate this condition to spondyloarthropathies or the arthritis condition that affects the spine. The presence of slow-growing bacteria like the Propionibacterium acnes can trigger this condition ^{[1, 2, 3]}.

Signs and Symptoms

Synovitis is the inflammation of the synovial membrane and may lead to pain, joint stiffness and swelling. The usual affected parts are the sternoclavicular joints, spine, anterior chest, hips or knees and the sacro-iliac joint. A limited range of motion may also be seen in these areas ^{[2, 4]}.

Source – epainassist.com

Acne is described as the presence of pus formation in the hair follicles of the skin. It typically forms in the face and upper back but patients with SAPHO may develop acne in their hands and feet ^{[1, 5]}.

Pustulosis is the inflammation of the skin that results in fluid-filled blisters called pustules. These pustules are usually found in the palms and soles of the affected individuals. There is also flaking and peeling of the skin that accompanies this symptom. [1, 6].

Figure 2 shows an example of pustulosis that had develop on a patient with this syndrome

Hyperostosis is defined as excessive bone growth. This symptom is usually seen in different musculoskeletal disease condition. The usual site for this symptom to develop are in the places where the tendon attaches to the bone such as the chest area ^{[1, 2, 5]}.

Osteitis is the inflammation of the bone. The inflammation in SAPHO patients occurs in the sacroiliac joints and spine. This would often lead to pain and stiffness of the neck and back area ^{[1, 2, 5]}.

Other symptoms that may be present include diarrhea, abdominal pain, and development of anal fissures or abscesses ^{[1, 2, 5]}.

Diagnosis

Health history & physical examination

The patient would seek consultation to a physician due to the symptoms that they experience. Information about the symptoms will be elicited by the physician. It is essential to know if the symptoms are recurring and if medications are able to manage the symptoms. The physical examination will identify any symptoms that may be present [1, 2].

Imaging studies

Studies such as Magnetic Resonance Imaging (MRI), Computed Tomography Scan (CT Scan) and X-rays are used to confirm the diagnosis of SAPHO syndrome. These tests will be able to show the development of hyperostosis and inflammation of the bones ^{[1, 2, 3]}.

Blood studies

The antigen HLA-B27 is found in patients who has SAPHO syndrome. Performing a blood test to look for this marker will be performed to support the establishment of the diagnosis ^[1].

Treatment

The focus of sapho syndrome treatment is the relief of its symptoms. Initially, the patients will be given are Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) to reduce the inflammation in the joints. If these drugs will not work, corticosteroids in the form of injection, tablets and topical creams will be prescribed to the patient. The presenting acne responds positively with the use of antimicrobials such as doxycycline and the pustulosis is resolved with topical corticosteroids ^{[1, 2]}.

Physiotherapy is helpful as an adjunct treatment for the osteoarticular symptoms of this syndrome. Surgery may also be advised to patients who did not respond positively to the previously tried treatment options ^{[1, 2, 7]}.

Prognosis

Although its age of onset varies great, it is more frequent in those who are between 30-40 years of age. The sapho syndrome prognosis is not predictable. It may involve several bouts of attack but the patient’s positive response to medications makes the outlook more optimal. Medications are important in the management of this condition but there will be remissions that will resolve spontaneously ^{[1, 2, 3]}.

Pictures

References

1. Shiel, W. J. (2015, November 18). SAPHO Syndrome. MedicineNet.com: http://www.medicinenet.com/sapho_syndrome/article.htm
2. Girschick, H., & Hayem, G. (2014, March). SAPHO syndrome. Orphanet: http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=793
3. Di Muzio, B., & Weerakkody, Y. (2005). SAPHO syndrome. Radiopaedia: http://radiopaedia.org/articles/sapho-syndrome
4. Facing Acne. (2016). SAPHO Syndrome and Acne. Facing Acne: http://www.facingacne.com/sapho-syndrome-acne/
5. Health Central. (2015). Synovitis. Retrieved from Health Central: http://www.healthcentral.com/encyclopedia/hc/synovitis-3168399/
6. Matzaroglou, C., Velissaris, D., Karageorgos, A., Marangos, M., Panagiotopoulos, E., & Karanikolas, M. (2009). SAPHO Syndrome Diagnosis and Treatment: Report of Five Cases and
7. Review of the Literature. The Open Orthopaedics Journal, 100-106.
8. Rukavina, I. (2015). SAPHO syndrome: a review. Journal of Children’s Orthopaedics, 19-27.