SAPHO syndrome

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Classification according to ICD-10
M86.3 (0-9) Chronic multifocal osteomyelitis (including SAPHO syndrome)
ICD-10 online (WHO version 2019)

The SAPHO syndrome is a rare disease from the group of rheumatic diseases with an age peak in young men and women. The acronym SAPHO stands for the symptoms that occur:

Not all symptoms need to be fully developed. The risk markers HLA-B27 and HLA-B8 are not found frequently. Associations with psoriasis , sacroiliitis , inflammatory bowel disease and Lyme borreliosis are also discussed. Some of the patients have significant functional limitations and often feel left alone by the medicine when they are subjected to severe subjective stress from the pain and the disfigurement caused by the severe skin symptoms. Overall, if diagnosed early, the prognosis is good. However, it is stressful for the patient that the syndrome is often only diagnosed in its entirety at an advanced stage because of the interdisciplinary symptoms.

The etiopathogenesis is assumed that a subclinical infection (e.g. acne) develops into an immune-reactive primary chronic osteomyelitis, accompanied by periostitis with phase-wise bone pain and neighboring synovitis.

Diagnostics include the imaging procedures skeletal scintigraphy and MRI . Laboratory findings are uncharacteristic.

Therapeutically, symptomatic nonsteroidal anti-inflammatory drugs (NSAIDs) are used, physiotherapy , immunosuppression with corticosteroids , and avoidance of painful stress. Also described: MTX , colchicine . Another therapeutic approach are bisphosphonates (especially zoledronic acid), certain antibiotics ( doxycycline , azithromycin ), interferon therapy , and the TNF-α inhibitors infliximab and etanercept , under which acne, which is already severe, can possibly worsen.

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  • Fritz Schilling: The SAPHO Syndrome. Clinical-rheumatological and radiological differentiation and classification of a patient population of 86 cases. In: Journal of Rheumatology. 59 (2000), pp. 1-28.
  • Otto Braun-Falco, Gerd Plewig, Helmut Heinrich Wolff, Walter Burgdorf (eds.): Dermatology and venereology. 5th edition. Springer-Verlag, Berlin / Heidelberg / New York 2005, ISBN 3-540-40525-9 , p. 517 ff. ( Online version )
  • EM Veys, CJ Menkes, P. Emery: A randomized, double-blind study comparing twenty-four-week treatment with recombinant interferon-gamma versus placebo in the treatment of rheumatoid arthritis. In: Arthritis Rheum 1997 Jan; 40 (1), pp. 62-68. PMID 9008601
  • I. Olivieri, A. Padula, G. Ciancio, C. Salvarani, L. Niccoli, F. Cantini: Successful treatment of SAPHO syndrome with infliximab: report of two cases. In: Ann Rheum Dis . 2002 Apr; 61 (4), pp. 375-376. PMID 11874849 , PMC 1754058 (free full text)
  • R. Gutzmer, RA Herbst, A. Kapp, J. Weiss: SAPHO syndrome. Case description of 3 patients with acne conglobata and osteoarticular symptoms. In: dermatologist. 1997 Mar; 48 (3), pp. 186-190. PMID 9182090

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