Department of Orthopaedic Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States; Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States; The Center for Research in FOP and Related Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States.
Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States; The Center for Research in FOP and Related Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States; Clinical Medicine, Division of Endocrinology, Diabetes and Metabolism, Center for Research in FOP & Related Disorders, Penn Medicine University City, 3737 Market Street, 3rd floor, United States.
Bone. 2018 Apr;109:115-119. doi: 10.1016/j.bone.2017.08.009. Epub 2017 Aug 16.
Flare-ups of the hips are among the most feared and disabling complications of fibrodysplasia ossificans progressiva (FOP) and are poorly understood. In order to better understand the nature of hip flare-ups in FOP, we evaluated 25 consecutive individuals with classic FOP (14 males, 11 females; 3-56years old, median age, 17years old) who presented with acute unilateral hip pain.
All 25 individuals were suspected of having a flare-up of the hip based on clinical history and a favorable response to a four day course of high-dose oral prednisone. Ten individuals (40%) experienced rebound symptoms of pain and/or stiffness within seven days after discontinuation of prednisone and all ten subsequently developed heterotopic ossification (HO) or decreased mobility of the affected hip. None of the 14 individuals who experienced sustained relief of symptoms following a course of oral prednisone experienced HO or decreased mobility. Incidental radiographic findings at the time of presentation were multifactoral and included osteochondromas of the proximal femur (18/25; 72%), degenerative arthritis (17/25; 68%), developmental hip dysplasia (15/25; 60%), previously existing heterotopic ossification (12/25; 48%), intra-articular synovial osteochondromatosis (8/25; 32%) or traumatic fractures through pre-existing heterotopic bone (1/25; 4%).
Developmental joint pathology may confound clinical evaluation of hip pain in FOP. The most useful modality for suspecting an ossification-prone flare-up of the hip was lack of sustained response to a brief course of oral prednisone. Evaluation of soft tissue edema by ultrasound or magnetic resonance imaging showed promise in identifying ossification-prone flare-ups and warrants further analysis in prospective studies.
髋关节发作是进行性骨化性纤维发育不良（FOP）最令人恐惧和致残的并发症之一，目前对此了解甚少。为了更好地了解 FOP 中髋关节发作的性质，我们评估了 25 例连续的经典 FOP 患者（男性 14 例，女性 11 例；年龄 3-56 岁，中位数年龄为 17 岁），他们均表现为单侧急性髋关节疼痛。
所有 25 名患者均根据临床病史和对 4 天高剂量口服泼尼松的良好反应怀疑髋关节发作。10 名患者（40%）在停用泼尼松后 7 天内出现疼痛和/或僵硬的反弹症状，且所有 10 名患者随后均发生异位骨化（HO）或受影响髋关节活动度降低。在接受口服泼尼松治疗后症状持续缓解的 14 名患者中，均未发生 HO 或髋关节活动度降低。就诊时偶然发现的放射学表现多因素，包括股骨近端骨软骨瘤（18/25；72%）、退行性关节炎（17/25；68%）、发育性髋关节发育不良（15/25；60%）、先前存在的异位骨化（12/25；48%）、关节内滑膜骨软骨瘤病（8/25；32%）或通过先前存在的异位骨的创伤性骨折（1/25；4%）。
关节发育病理学可能会混淆 FOP 髋关节疼痛的临床评估。怀疑髋关节易发生骨化的最有用方法是对短期口服泼尼松治疗无持续反应。超声或磁共振成像评估软组织水肿在识别易发生骨化的髋关节发作方面显示出前景，值得在前瞻性研究中进一步分析。
