Rheumatology Unit, Department of Medical Sciences, Surgery and Neuroscience, University of Siena and Azienda Ospedaliero-Universitaria Senese, Viale Mario Bracci 16, 53100 Siena, Italy.
Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University of Ljubljana and University Medical Centre Ljubljana, Bohoričeva ulica 20, 1000 Ljubljana, Slovenia; Department of Pediatrics, Faculty of Medicine, University of Ljubljana, Bohoričeva ulica 20, 1000 Ljubljana, Slovenia.
Joint Bone Spine. 2024 Dec;91(6):105772. doi: 10.1016/j.jbspin.2024.105772. Epub 2024 Sep 12.
(1) characterizing a group of spondyloarthritis (SpA) patients with systemic auto-inflammatory symptoms (S-SpA); (2) comparing SpA features with and without auto-inflammatory symptoms; (3) comparing the auto-inflammatory features of S-SpA and Still's disease (SD).
Retrospective observational study. Clinical data of adult and pediatric patients with S-SpA, SD or SpA were collected retrospectively and analyzed.
Forty-one subjects with S-SpA, 39 with SD and 42 with SpA were enrolled. The median latency between systemic and articular manifestations in S-SpA was 4.4 (IQR: 7.2) years. S-SpA and SpA had similar frequency of peripheral arthritis and enthesitis (N.S.), while tenosynovitis was more frequent (P=0.01) and uveitis less frequent (P<0.01) in S-SpA. MRI showed signs of sacroiliac inflammation and damage in both S-SpA and SpA equally (N.S.). S-SpA patients had less corner inflammatory lesions (P<0.05) and inflammation at the facet joints (P<0.01), more interspinous enthesitis (P=0.01) and inter-apophyseal capsulitis (P<0.01). Compared to SD, S-SpA patients had lower-grade fever (P<0.01), less rash (P<0.01) and weight loss (P<0.05), but more pharyngitis (P<0.01), gastrointestinal symptoms (P<0.01) and chest pain (P<0.05). ESR, CRP, WBC, ANC, LDH tested higher in SD (P<0.01). Resolution of systemic symptoms was less frequent in S-SpA than SD on corticosteroid (P<0.01) and methotrexate (P<0.05) treatment. When considering all SD patients, a complete response to corticosteroids in the systemic phase significantly reduced the likelihood of developing SpA (OR=0.06, coefficient -2.87 [CI: -5.0 to -0.8]).
SpA should be actively investigated in patients with auto-inflammatory manifestations, including undifferentiated auto-inflammatory disease and SD.
(1)描述一组具有全身炎症症状的脊柱关节炎（SpA）患者；(2)比较具有和不具有全身炎症症状的 SpA 特征；(3)比较全身炎症性 SpA（S-SpA）和斯蒂尔病（SD）的全身炎症特征。
回顾性观察性研究。回顾性收集成人和儿科 S-SpA、SD 或 SpA 患者的临床资料并进行分析。
共纳入 41 例 S-SpA、39 例 SD 和 42 例 SpA 患者。S-SpA 患者全身和关节表现之间的潜伏期中位数为 4.4（IQR：7.2）年。S-SpA 和 SpA 的外周关节炎和附着点炎频率相似（N.S.），而腱鞘炎更常见（P=0.01），葡萄膜炎较少见（P<0.01）。MRI 显示 S-SpA 和 SpA 的骶髂关节炎和损伤均有相同的迹象（N.S.）。S-SpA 患者的角炎性病变较少（P<0.05），关节突关节炎症较少（P<0.01），棘间附着炎和椎间关节囊炎更多（P=0.01）。与 SD 相比，S-SpA 患者的发热程度较低（P<0.01），皮疹（P<0.01）和体重减轻（P<0.05）较少，但咽炎（P<0.01）、胃肠道症状（P<0.01）和胸痛（P<0.05）更多。SD 患者的 ESR、CRP、WBC、ANC、LDH 测试结果更高（P<0.01）。S-SpA 患者在接受皮质类固醇（P<0.01）和甲氨蝶呤（P<0.05）治疗时，全身症状缓解频率低于 SD。当考虑所有 SD 患者时，全身期皮质类固醇完全缓解显著降低了发生 SpA 的可能性（OR=0.06，系数-2.87 [CI：-5.0 至-0.8]）。
应积极调查具有全身炎症表现的患者，包括未分化的自身炎症性疾病和 SD，以发现 SpA。
