AB0889 Clinical characteristics of 88 patients with pustulotic arthro-osteitis (PAO) in japan

Abstract

Background: The prevalence rate of PPP in Japan is somewhat as 0.13%1) , accounting for 61% of cutaneous manifestations diseases associated with SAPHO syndrome2) . SAPHO syndrome3) is not a new disease concept in which the cause of the disease has been clarified but is a name that classified diseased groups by collecting known diseases. On the other hand, PAO is a concept of disease that is a “proper subset” of SAPHO syndrome and is the smallest unit disease concept. Therefore, detailed information on PAO was sought. Objectives: To examine the clinical features and treatment of PAO Methods: We conducted a multicenter, non-intervention retrospective study of 88 PAO patients who were able to visit directly in 4 hospitals in Japan from January to June 2017. the criteria of “ Sonozaki et al. 1981 4) ” was used for PAO diagnosis. Evaluation items were clinical features, image/blood biochemical examination, treatment methods. Results: The average age at the time of visit was 55.4 years old, palmoplantar pustulosis/PAO onset age was 44.4 years old, 49.3 years old, the onset pattern was skin leading type/simultaneous onset/joint advanced type (66.7/25.6/7.7%). The clinical features were pain joints of 33.0% of the thoracic joints, 22.7% of the shoulder joints and 10.2% of the joints of the ankle joints, and the adhesion flame was the most abundant in the Achilles tendon at 21.4%. ASDAS-CRP was 1.4 ±0.8 on average, breakdown was inactive disease 52.3%, moderate 22.7%, severe 22.7%, very high disease activity 2.3%, and insufficient disease activity remained in 25% of cases. Radiographic change showed in 81.8% of the sternum/ the sternocostoclavicular joint, 33.0% of the spine, 31.0% of the sacroiliac joint (NY IIor more), and 9.8% of the peripheral joint. MRI findings showed changes in intraosseous luminance in 76.9% around the sternum, 52.2% in the spine and 65.2% in the sacroiliac joint. Bone scintigraphy showed abnormal findings in the anterior chest 96.4%, spine 23.6% and sacrum 30.9%. Blood biochemical examination showed CRP 0.35±0.52 mg/dl, RF positive rate 9.4%, ACPA positive rate 5.1%. First-line drugs (NSAIDs, biotin, anti-bacterial drugs) and second-line drugs (Iguratimod sulfasalazine MTX) were selected as therapeutic agents. For other treatments, 64.8% of topical therapy, 12.5% of phototherapy and 18.6% of tonsillectomy were performed. Conclusions: In this study, we reported the clinical features, radiological findings and treatments in 88 Japanese patients with PAO in Japan. The treatment outcome for PAO in Japan is not sufficient, and it is necessary to try to elucidate further disease condition.