Aljuhani Faisal, Tournadre Anne, Tatar Zuzana, Couderc Marion, Mathieu Sylvain, Malochet-Guinamand Sandrine, Soubrier Martin, Dubost Jean-Jacques
From the Department of Rheumatology, Gabriel Montpied Hospital, Centre Hospitalier Universitaire de Clermont-Ferrand, Clermont-Ferrand, France.F. Aljuhani, MD; A. Tournadre, MD; Z. Tatar, MD; M. Couderc, MD; S. Mathieu, MD; S. Malochet-Guinamand, MD; M. Soubrier, MD, PhD; J-J. Dubost, MD; Department of Rheumatology, Gabriel Montpied Hospital, Centre Hospitalier Universitaire de Clermont-Ferrand.
J Rheumatol. 2015 Feb;42(2):329-34. doi: 10.3899/jrheum.140342. Epub 2014 Dec 15.
The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis) is a rare disease and only a few series have been published about it. We describe the experience of a rheumatology department with a focus on the therapeutic aspects.
All cases of SAPHO fulfilling the Benhamou criteria and seen between 1992 and 2013 in our unit were retrospectively included.
Forty-one patients (11 men and 30 women) were included. The average age was 45 years (20-80 yrs) at the time of diagnosis. Nineteen patients had palmoplantar pustulosis, 3 had psoriasis vulgaris, 3 had severe acne, and 15 had no dermatological involvement. Bone symptoms involved mainly the anterior chest wall (n = 28, 68%), followed by the spine (n = 16, 39%) and sacroiliac joints (n = 12, 29%). Four patients had peripheral osteitis and 2 had mandibular osteitis. Ten patients had peripheral arthritis affecting mainly the knees and wrists. None of the 36 patients tested was HLA-B27-positive. At least a partial response was achieved with colchicine, methotrexate, or sulfasalazine in 0/6, 2/4, and 1/6 of patients, respectively. Antibiotic therapy (azithromycin, n = 7; doxycycline, n = 2) was effective in 2/9 patients. Pamidronate was prescribed in 26 patients with bone involvement and 18/22 patients evaluable at 6 months responded to this therapy. Two patients were treated with tumor necrosis factor blockers: 1 with infliximab and 1 with adalimumab, followed by etanercept.
Based on our experience, SAPHO can be diagnosed in the elderly. It was not associated with HLA-B27. Soft tissue involvement was common and the bisphosphonates were generally effective on bone involvement.
滑膜炎、痤疮、脓疱病、骨肥厚、骨炎综合征（SAPHO综合征）是一种罕见疾病，关于它的系列报道很少。我们描述了一个风湿病科在治疗方面的经验。
回顾性纳入1992年至2013年间在我们科室就诊且符合贝纳穆标准的所有SAPHO病例。
共纳入41例患者（11例男性和30例女性）。诊断时的平均年龄为45岁（20 - 80岁）。19例患者有掌跖脓疱病，3例有寻常型银屑病，3例有重度痤疮，15例无皮肤受累。骨骼症状主要累及前胸壁（n = 28，68%），其次是脊柱（n = 16，39%）和骶髂关节（n = 12，29%）。4例患者有外周骨炎，2例有下颌骨炎。10例患者有外周关节炎，主要累及膝关节和腕关节。36例接受检测的患者中无一例HLA - B27呈阳性。秋水仙碱、甲氨蝶呤或柳氮磺胺吡啶分别使0/6、2/4和1/6的患者至少获得部分缓解。抗生素治疗（阿奇霉素，n = 7；强力霉素，n = 2）使2/9的患者有效。26例有骨骼受累的患者使用了帕米膦酸盐，22例可在6个月时评估的患者中有18例对该治疗有反应。2例患者接受了肿瘤坏死因子阻滞剂治疗：1例使用英夫利昔单抗，1例使用阿达木单抗，随后使用依那西普。
基于我们的经验，SAPHO在老年人中也可诊断。它与HLA - B27无关。软组织受累常见，双膦酸盐对骨骼受累一般有效。
