SAPHO Syndrome: About an Observation and Review of the Literature

Abstract

Introduction: SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is autoinflammatory rheumatism of the spondyloarthritis group. It is rare, it prevalence is 1/10000 inhabitants. The average age at diagnosis is 38 years, with no gender predominance, first described by Chamot and colleagues in 1987. Objective: To present observation and review of the literature on SAPHO syndrome which is rarely diagnosed in our daily practice. Observation: This was a 28 year old female patient, Senegalese, living in Dakar, followed in obstetrics gynecology for the desire of maternity, the family context of sickle cell disease who consulted for diffuse mechanical and articular inflammatory pains involving the proximal interphalangeal (IPP) of the left 5th toe, the left shoulder, the sternocostal and dorsolumbar joints evolving for 3 years. The clinical examination found: A painful swelling with a visual analog scale (VAS) of 6/10 of the right sternocostal joint and of the interphalangeal joint (IP) of the right hallux, diffuse acne, no palmoplantar pustulosis, no psoriasis, no gluteal or talalgia. The biology shows an inflammatory syndrome (VS at 37 mn at the 1st hour and CRP at 41mg/l), a decrease of 25-OH-Vitamin D3 at 22 ng/ml (N: 25-30 ng/ml), the positive latex test, Ac anti-CCP: 43 IU/ml (N < 5 IU/ml), positive Ac anti-HBc , Ac anti HBs > 1000 IU/l on the other hand blood calcium, blood phosphorus, G6PD, PTH, TSH us, free T4, anti ECT Ac, HLA-B27 Ag with no particularities. The scintigraphy showed hyper fixation of the right 1st toe, sacroiliac joints, dorsolumbar spine, left shoulder, manubrium sternal junction, and bilateral sternoclavicular junction realizing a classical bull horn image in SAPHO syndrome. Because of this clinical and paraclinical manifestation helped by the 1994 diagnostic criteria of Kahn at 1/3 (chronic joint involvement associated with severe acne), the SAPHO syndrome was retained and the treatment instituted after the normal pre-therapeutic analysis and systematic deworming was: Salazopyrine 2000mg/day (dosage reached by increments of 500 mg/week); Uvedose (vitamin D3 ) 100000 IU / month; bisphosphonates 70 mg /week and paracetamol + codeine 1g x 2/day as needed for 3 months with a favorable follow-up by the regression of signs (pain VAS at 2/10). Conclusion: The SAPHO syndrome is the prerogative of the young adult subject, bone scintigraphy is very valuable for its diagnosis, its better knowledge would avoid diagnostic and therapeutic erratic. Keywords: SAPHO syndrome, bone scan, Dakar, Senegal.