SAPHO Syndrome

Overview

The authors present the case-report of a patient where after 7 years of the disease duration,examinations and treatments in different departments, finally in the Institute of Rheumatology thediagnosis of the SAPHO syndrome was established. During the past two months another two patientswith this syndrome were encountered. The acronym SAPHO symbolizes the main components ofthis disease – synovitis, acne, pustulosis, hyperostosis and osteitis. Synovitis is manifested asoligoarthritis, can affect any joint of the peripheral and axial skeleton, it has a tendency to attackin particular joints and articulations of the anterior thoracic wall. Osteitis is a s a rule oligofocal butcan be also multifocal: it affects in particular epiphyses of bones and the spine. The position is similaras regards periostitis. Dermatosis is represented by some form of acne (A. pustulosa, A . conglobata,and A. fulminans) or psoriasis (P. vulgaris, P. pustulosa). The disease, due to some features, is closeto spondylarthritis (oligoarthritis, affection of the spine incl. possible syndesmophytes, absence ofRF). It is however not associated with HLA B-27. Specific treatment is not known, the prognosisquoad vitam is favourable, the course is however usually chronic and can make the patie nt’s lifedifficult. The differential diagnosis is important as it can be easily mistaken for osteomyelitis orbone tumours.

Key words:
SAPHO, osteitis, osteomyelitis, acne, pustulosis, hyperostosis