强直性脊柱炎

强直性脊柱炎（AnkylosingSpondylitis,AS）是一古老疾病。在近一个世纪里，人们一直将AS和类风湿关节炎混为一种疾病，称AS为类风湿关节炎的中心型，或称为类风湿关节炎的变异型，直至本世纪60年代才将AS从类风湿关节炎区分出来作为一独立疾病。AS是一种慢性、进行性、灭性疾病，主要侵犯骶髂关节、脊柱骨突关节、脊柱旁软组织及外周（四肢）关节，也可发生关节外病变。本清的特点是椎间盘纤维环和纤维环附近结缔组织的纤维化和骨化，以及受累关节的强直。由于很少病例发展为脊柱“弯曲”，故AS不是一个理想的名称。一、流行病学AS…     

尿黑酸症的临床及影像学表现(附3例报告)

目的探讨尿黑酸症发病机制临床及影像学表现。方法通过对3例确诊为尿黑酸症患者的临床及影像学资料进行分析,参考以前文献总结其特征性表现。结果尿黑酸症患者体内过量的尿黑酸能够解释其临床表现三联征:黑尿病(过量的尿黑酸静置氧化颜色变深)、褐黄病(尿黑酸氧化产物在连接组织沉积导致其变色)、褐黄病性关节炎(尿黑酸斑片积累致组织脆性增加逐步形成关节炎)。蓝色耳廓和眼巩膜棕褐色色素沉着为患者的临床相貌特征。由于临床症状首先出现在脊柱,患者多由腰背痛前来就诊。最具特征性脊柱影像学表现为进行性椎间盘软骨板钙化、椎间盘变性突出、椎间隙变窄。结论影像学检查可发现病变的部位和骨质改变程度,尤其在脊柱表现典型,能为临床确诊本病提供可靠依据。     

以关节屈曲挛缩为首发表现的淀粉样关节病5例并文献复习

目的 报道5例以关节屈曲挛缩为首发表现的淀粉样关节病病例，并进行文献复习，以提高对该病的认识。方法 收集2012年1月-2022年2月就诊于解放军总医院第一医学中心风湿免疫科的、以关节屈曲挛缩为首发表现的、免疫球蛋白轻链(AL)型淀粉样变性相关的淀粉样关节病患者的临床资料进行回顾性分析，检索中国知网、万方数据知识服务平台、PubMed数据库，结合文献报道总结AL型淀粉样变性相关的淀粉样关节病的临床表现、实验室及影像学检查结果。结果 通过病例回顾，本中心共有5例以关节屈曲挛缩为首发表现的淀粉样关节病患者，其中男4例，女1例。检索中国知网、万方数据知识服务平台、PubMed数据库(截至2022年2月)，筛选出2例符合条件的患者，均为男性。共纳入7例，男女比例6∶1，中位年龄30岁(19～73岁)。脊柱及四肢大小关节均受累，尤其是手关节(7例，100%)。共有5例(71.4%)出现软组织受累：腕管综合征4例，巨舌3例，肩垫征2例，其他部位肌肉肿胀2例。5例被误诊为类风湿关节炎(RA),1例被误诊为脊柱关节炎(SpA)，延迟诊断的中位时间为24(2～204)个月。实验室检查结果发现，3例(42...     

进行性骨化性肌炎临床X线分析

报告了５例进行性骨化性肌炎。其临床特点是婴幼儿期发病，全身反复出现软组织包块，包块渐固定，并伴有进行性关节活动受限，最后发展到关节僵直。Ｘ线特点是软组织内骨柱形成，颈部软组织最易受累，其次为腰、背、胸部和四肢。笔者结合文献讨论了本病的发病机理、病理改变、临床及Ｘ线表现。     

晚发型脊柱骨骺发育不良伴进行性骨关节病的影像学诊断

目的 探讨晚发型脊柱骨骺发育不良伴进行性骨关节病(SEDT-PA)的影像学表现及其病理基础. 资料与方法搜集经临床及影像学检查确诊的SEDT-PA同胞姊妹3例,摄双手及腕关节、双肘关节、双髋关节正位X线片,双膝关节及脊柱正侧位片;均行胸腰椎MRI扫描.结果 X线片及MRI片均可见椎体变扁,呈"横置花瓶"状和"子弹头"状改变,椎间隙明显变窄.X线片上表现为关节间隙变狭窄,骨端膨大及明显骨质增生.MRI上可见椎间盘变薄,纤维环和髓核结构不能分辨.结论根据特征性的影像学表现,结合临床特点能够明确SEDT-PA的诊断.     

强直性脊柱炎的CT诊断

强直性脊柱炎(AS)是一种血清阴性脊柱关节病。本病以骶髂关节、椎体及小关节突、髋关节等部位的慢性进行性炎症为特征的全身性疾病。90%的患者HLA-B27为阳性,类风湿因子多为阴性。笔者回顾我院2003—2005年间4例临床资料、影像及化验检查等资料完整的强直性脊柱炎病例,着重探讨其CT表现,以提高对本病的认识。均27岁。4例患者临床均表现为下腰部疼痛不适,伴有不同程度的运动受限(其中1例弯腰及转身困难),“晨僵”较明显,活动后可缓解。病程均在半年以上,本组病例类风湿因子均为阴性,HLA-B27均为阳性。1.2检查方法4例患者均行CT扫描和X…     

家族性进行性骨干发育不良

进行性骨干发育不良又称Engelmann's病,较罕见[1].关于本病的X线表现,国内所见报道仅30余例[2],CT表现报道较少,特别是四肢、头颅、脊柱及骨盆的螺旋CT表现未见报道.本研究结合文献总结分析了我院诊断一家族2例的全身X线片和螺旋CT表现,旨在提高对本病的认识.     

I型高雪病的影像诊断(附一例报告及文献复习)

目的提高对Ｉ型高雪病骨骼系统改变影像学表现的认识。方法报告１例Ｉ型高雪病的Ｘ线平片和ＭＲＩ所见,并作文献复习。结果本病的Ｘ线平片有特征性改变,ＭＲＩ既能显示骨骼病变纵向累及范围,又能观察骨髓内部受累的变化。结论ＭＲＩ是诊断高雪病骨骼受累的最佳方法。     

假性软骨发育不全（附两例报告）

本病为罕见的软骨发育障碍疾患。1959年Lamy等首先报道3例，1960年Maroteaux对脊柱骨骺发育不良予以分类，将本病命名为脊柱骨骺发育不良－－假性软骨发育不全型，1961年Ford又报告3例，国内尚未见公开报道。我们得见两例，报道如下。     

胸肋锁骨肥厚症的影像学表现

目的探讨胸肋锁骨肥厚症的影像学表现并对其影像学分期予以商榷。方法回顾性分析4例患者的临床和X线平片、CT影像资料。结果 4例患者中,1例早期仅表现为软组织肿胀,胸锁关节及邻近的胸、肋、锁骨骨质破坏,并有沙粒样死骨,锁骨可见骨膜反应。其余3例表现为受累胸、肋、锁骨局部骨骼增粗,骨、骨髓腔及肋软骨密度增高,邻近软组织骨化,受累关节呈骨性融合;但1例锁骨未受累,肋锁韧带亦无骨化。结论胸肋锁骨肥厚症早期可能均存在局部的骨质侵蚀,进而软组织钙化或骨化,产生所谓"肥厚"表现,故其分期应予补充完善。     

40例类风湿性关节炎X线观察

目的:进一步了解类风湿性关节炎(简称RA)患者手部小关节的X线特征。资料:选择16岁～60岁共40例RA中期女性患者。方法:均拍摄双手正位平片,结合部分临床资料分析。结果:(1)指间关节、掌指关节、腕掌关节及腕骨问关节呈不对称性损害者分别占65％、50％、80％及82.5％;双手整体观呈不对称性损害者26例,占65％。(2)比较病程从1周到10年不等的患者,X线损害并未随病程增长而逐步加重。结论:双手临床表现同其X线平片表现并不一致;病程长短同X线损害程度也不一致。     

Spondyloepiphyseal dysplasia tarda with progressive arthropathy

The clinical and roentgenographic manifestations of six cases suffering from spondyloepiphyseal dysplasia tarda with progressive arthropathy are presented. The roentgenographic features consist of generalized platyspondyly and varying degrees of epiphyseal involvement with conspicuous enlargement of both ends of the short tubular bones of the hands. This disorder appears at an earlier age, and is more crippling than the usual form of spondyloepiphyseal dysplasia tarda. It is suggested that orthopaedic measures are essential for all patients with this disorder, as profound disability may be expected by progressive involvement of the major joints.     

Progressive pseudorheumatoid chondrodysplasia: a report of nine cases in three families

Nine cases of progressive pseudorheumatoid chondrodysplasia in subjects aged 7–60 years are reported. Six of them were members of one large family with several consanguineous marriages. Evaluation of six enerations of this family suggested an autosomal recessive mode of inheritance. The presentation of the disease was between the ages of 3 and 8 years with waddling gait and fusiform swelling of the interphalangeal joints of the hands, clinically resembling juvenile rheumatoid arthritis. Most of the other joints become progressively involved, with crippling disability. One of the patients had typical features of Blount disease at presentation.     

Psoriasis arthropathica. A review of the literature, general considerations and the authors' personal experience]

A retrospective study has been carried out on 897 psoriatic patients in order to verify the incidence and radiological patterns of psoriatic arthropathy (PA). Site of involved joints, appearance and degree of involvement were correlated with extent of skin disease and blood test results. Sixty-five patients (7%) showed "clinical" arthritis whereas only 20 cases (2.2%) were radiologically positive; among them, 35% were classified as "severe" forms. Peripheral arthritis was observed in all cases, involving the hand in 85% of patients. The distal interphalangeal joints were the most affected location in the feet (78%) and the proximal interphalangeal joints in the hands (94%). The extant small peripheral joints were involved in decreasing rates proceeding proximally. Bilateral and asymmetric involvement was observed in most of the patients; hypertrophic interphalangeal joints erosion was the typical pattern in 100% of cases, evolving in rheumatoid-like ankylosis in 23% of them. Spondylitis and/or sacroiliitis were associated in 50% of patients. No correlation between degree of arthritis and skin disease was found in our series, with the exception of proximal and axial joints arthritis, in which extensive skin disease and severe small joints involvement were associated in 80% of cases. Clear differential features were demonstrated between peripheral PA and rheumatoid arthritis, and between spinal involvement in PA and ankylosing spondylitis. Poor correlation of distal arthritis to psoriatic nail involvement was also demonstrated.     

Ultrasound and power Doppler evaluation of the hand and wrist in patients with psoriatic arthritis

Purpose: To evaluate the ability of high-resolution and power Doppler sonography in detecting joint and tendon abnormalities in patients with psoriatic arthritis (PsA) of the hands and wrists compared with clinical and radiological findings.

Material and Methods: Thirty-six patients with psoriatic arthritis of the hands and wrists and 10 healthy controls were examined with ultrasound (US). The degree of synovial proliferation, tenosynovitis, presence of joint effusion as well as the vascularity of synovial tissue was estimated. US findings were scored using a newly devised scoring system.

Results: Thirty-two patients had articular synovial proliferation and/or tenosynovitis/tendinitis or joint effusion in one or more joints according to US. Twenty-two patients had tendon changes; only five had joint effusion. The synovial, Doppler, and total articular-teno scores were all significantly correlated to the number of swollen joints. The scores, however, did not correlate to other clinical or laboratory measurements of disease activity.

Conclusion: US proved effective in demonstrating PsA involvement of the hands and wrists and was more sensitive than clinical examination in detecting pathology. Long-term follow-up studies are needed to evaluate whether this can change the traditional approach for assessing involvement of joints and tendons in PsA.     

Value of the radiographic criteria "fingers and toes" in the early diagnosis of psoriatic arthritis

PURPOSE: To assess the value of Fournie's radiological criteria "fingers and toes" in the early diagnosis of psoriatic arthritis. MATERIALS AND METHODS: 47 patients with psoriatic arthritis were studied retrospectively. Clinical and radiological features at the hands and fingers were assessed. Radiographic analysis was performed using Fournie's criteria. RESULTS: Of 47 patients studied, 25 patients developed hand and finger involvement. Asymmetrical joint distribution was observed in 76% of cases. Distal interphalangeal arthritis was noted in 60% of cases. Nail lesions were present in 11 cases and dactylitis in 3 cases. Eight patients had erosions of the distal interphalangeal joints. Osteolysis with pencil-in-cup deformity was noted in 3 cases. Ankylosis of interphalangeal joints was found in 7 cases and acro-osteolysis in 3 cases. CONCLUSION: Psoriatic arthritis frequently affects the small joints of the hands and fingers. Asymmetrical and distal interphalangeal joint involvement are a characteristic feature of this arthropathy. Some radiological lesions are suggestive and contribute to the early diagnosis of psoriatic arthritis.     

The hand in psoriatic arthropathy. Radiographic and xeroradiographic comparison

The hands of 45 cases of psoriasis with arthritis and of 100 cases of rheumatoid arthritis without psoriasis were studied by radiographic and xerographic techniques. The characteristic findings of both methods were recorded separately and compared later. In 44% of cases of psoriatic arthritis, radiographic and xerographic changes are indistinguishable from typical rheumatoid arthritis, but with predominant involvement of distal interphalangeal joints, often bilaterally asymmetric. On the contrary, in 31% of cases, erosive polyarthritis associated with proliferation of subperiosteal new bone (enthesopathy) is present, also with characteristic feature of arthritis mutilans. In 25% of cases, psoriasis of the skin and/or nails is associated with clinical manifestations of rheumatoid arthritis, without roentgenographic findings of arthropathy. The xerographs and radiographs of each patient were compared, assessing the degree of subperiosteal erosion, soft tissue swelling, acro-osteolysis, distruction of small joints and osteolysis of the phalanges, bony ankylosis. The soft tissue swelling ("sausage" finger) was more easily demonstrated by xeroradiography than by conventional radiography, because of its wide recording latitude. However, radiograph remains the technique of choice in the assessment of bone changes of the hand, especially at the first stage of arthritides.     

Radiology of the hand in progressive systemic sclerosis

Radiographs and xerographs of the hands of 35 patients with progressive systemic sclerosis (PSS), as defined by the ARA, were reviewed. Patients with "overlap" syndromes (i.e., mixed connective tissue disease, systemic lupus erythematosus or rheumatoid arthritis) have been excluded. Soft tissue changes included atrophy (hide-bound skin), and dystrophic calcifications, particularly in CREST patients (calcinosis, Raynaud phenomenon, esophageal dysmobility, sclerodactily and telangectasia). The most common bony change is resorption of distal phalanges; diffuse osteoporosis is also frequent; the distal interphalangeal and first carpometacarpal joints involvement appear as a distinctive feature of this erosive arthritis.     

Carpal predominance in rheumatoid arthritis

Radiographic and clinical evaluation of the relative severity of wrist versus hand involvement in 101 patients with rheumatoid arthritis revealed more severe changes in the wrists in 60%, equal involvement in wrists and hands in 37%, and more severe changes in the hands in 3%. There were severe changes in the wrists but little or no bone or joint change in the metacarpophalangeal and proximal interphalangeal joints in 43 (21%) of the 202 extremities studied. Serial examinations showed that, in time, the hand changes tended to overtake those in the wrist. Appreciation of this progression of wrist and hand changes can help the physician avoid diagnostic difficulties in a significant percentage of patients.     

Progressive pseudorheumatoid dysplasia

A rare case of progressive pseudorheumatoid dysplasia (PPD) in a 9-year-old girl is presented. Clinically, chronic painless swollen joints, accompanied by progressive motion restriction and progressive walking difficulties, were found. Radiologically, there was enlargement of the epimetaphyseal portions of the large joints, metacarpal heads, and phalanges, and generalized platyspondyly with irregular delineation of the endplates of the vertebral bodies. The radioclinical features at the peripheral joints were originally misdiagnosed as juvenile rheumatoid arthritis (JRA), and the structural spinal abnormalities were neglected and interpreted as Scheuermann's disease. However, the absence of active inflammatory parameters argues against JRA, whereas the low age of onset of the irregularities at the vertebral endplates is an argument against the diagnosis of Scheuermann's disease. The combination of the dysplastic abnormalities of the spine, with platyspondyly and Scheuermann-like lesions at an unusually low age of onset, and radiological features mimicking JRA of the peripheral joints, is the clue to the diagnosis of this rare autosomal-recessive disease. This case is the first to document the MRI features of PPD of the spine. Received: 22 February 2000; Revised: 5 May 2000; Accepted: 9 May 2000