SAPHO syndrome

Rheumatism of palmoplantar pustulosis and SAPHO (Synovitis, Acne, Pustulose, Hyperostosis, Osteitis)

They are close to psoriatic arthritis but much rarer.

Population

The national prevalence of SAPHO Syndrome is estimated at approximately 1/10000 and affects all age groups. For the infantile form, we speak of CRMO: Chronic Recurrent Multifocal Osteomyelitis (CRMO), . The SAPHO syndrome is a condition that affects a little more women than men, and especially the young adult (between 30 and 50 years). However, the attack can occur early in life (and often up to 60 years).

Diagnostic

The diagnosis of SAPHO syndrome is often difficult, but there is no weight loss. Physicians have defined two situations sufficient to diagnose SAPHO syndrome:
  •   sterile bone inflammation that do not affected the thorax, spine or pelvis, with or   without skin lesions,
  •  arthritis affection associated to skin affection (severe acne, psoriasis or palmoplantar pustulosis ...). It is a set of clinical elements, biological, and especially an assessment of images(bone and joint) which make it possible to establish the diagnosis. On x-rays, on recipes including increased density and bone thickness. The absence of associated skin disease does not exclude the diagnosis of SAPHO syndrome. SAPHO syndrome is not contagious.


Symptoms

This is the chronic disease, the bone manifestations represent the achievement of the most typical, true cornerstone of the diagnosis. It is painful, often mechanical, evolutive by inflammatory attacks. There are 3 distinct forms of manifestations:
• bone and joint involvement,
• cutaneous involvement,
• other manifestations (inflammatory bowel diseases such as Crohn's disease, ulcerative colitis ...), as well as celiac disease.

Causes

Given the highly variable forms of this syndrome, there are certainly several factors involved in triggering the disease:
• a genetic predisposition,
• environmental factors,
• an infection,
• a malfunction of immune defenses ...

Treatments

There is no specific treatment for SAPHO syndrome, but several medications can limit the intensity of symptoms, and especially to relieve pain:
• The basic treatment is based on nonsteroidal anti-inflammatory drugs or NSAIDs (not containing cortisone). Their effectiveness is often insufficient, which explains why other treatments are generally associated with them, such as sulfasalazine or methotrexate.
• Bisphosphonates have shown favorable action not only on pain but also on pustulose lesions.
• In rare cases, corticosteroids (such as prednisone) are prescribed, usually at a low dose and for a short time, mainly to fight bone and joint pain. They are sometimes offered in intra-articular injections (infiltrations).
• TNF-alpha biotherapies have already shown good results and can be an interesting alternative.
• The morphine-type analgesics (administered very punctually) have also brought relief to patients.
• Prolonged antibiotic treatment, already offered by some doctors, is still being studied to measure its effectiveness and relevance.
• Physiotherapy, therapeutic education of the patient ... It should be noted that, unlike other rheumatic diseases, SAPHO syndrome is not eligible for preventive or restorative surgery. As for acne and other skin lesions, they must be taken care of by a dermatologist

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