【双语病例】系统性硬化症(硬皮病)一例X线影像表现

4 8月

History

Patient presents with a knot on his left foot.

患者因左足结节就诊。

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

1.Which of the following does NOT belong in the differential diagnosis for causing a calcified soft-tissue/periarticular mass?

下列鉴别诊断中,哪个不会引起软组织钙化/关节旁肿块?

Gout 痛风

Scleroderma 硬皮病

Pigmented villonodular synovitis (PVNS) 色素沉着绒毛结节性滑膜炎

Calcific myositis 钙化性肌炎

Renal osteodystrophy 肾病性骨营养不良

Additional Questions

Additional skeletal radiographs are shown below. Click to enlarge.

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

2.What is the most likely diagnosis?

最有可能的诊断是什么?

Gout 痛风

Calcific bursitis 钙化性滑囊炎

Calcific myositis 钙化性肌炎

Scleroderma 硬皮病

Old frostbite 陈旧性冻伤

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

选择题答案:

  1. Pigmented villonodular synovitis (PVNS)

  2. Scleroderma

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

Findings and Diagnosis

FindingsThe left foot has rounded and amorphous calcifications in the plantar soft tissues at the level of the fifth metatarsophalangeal (MTP) joint. Hands show soft-tissue calcifications with erosive changes of the tufts of the distal phalanges of the thumb and index fingers of the left hand. There is joint space narrowing of the interphalangeal joints. Elbow shows calcifications in the soft tissues of the posteromedial right elbow.

左足底第五跖趾关节处、软组织内可见多发类圆形钙化灶。

双手软组织多发钙化,左手拇指、食指远端指骨骨质略受侵。指间关节间隙狭窄。

肘关节后内侧软组织内可见钙化灶。

Differential Diagnosis

  • Scleroderma 硬皮病
  • Crystalline arthropathy (gout or calcium pyrophosphate deposition disease)晶体性关节病(痛风或焦磷酸盐沉积钙化)
  • Calcific bursitis 钙化性滑囊炎
  • Myositis (trauma) 创伤性肌炎
  • Renal osteodystrophy 肾病性骨营养不良
DiagnosisScleroderma

硬皮病

【双语病例】系统性硬化症(硬皮病)一例X线影像表现

Discussion

Systemic sclerosis (the umbrella term encompassing scleroderma) is a disease caused by inflammation with subsequent excessive collagen types 1 and 3 deposition with resultant fibrosis and microvascular occlusion. The prevalence is approximately 240 cases per million population with an average life expectancy of 12 years from diagnosis. Black people (particularly young, black women) are more commonly affected. Involvement of the skin and soft tissues is often most readily apparent, although the disease affects many internal organs and vascular structures.系统性硬化症(包括硬皮病)是指炎症引起胶原蛋白I和III过量沉积,最终导致纤维化和微血管栓塞。该病的发病率约240/1,000,000,确诊该病后的平均生存时间约12年。

黑人(特别是年轻的黑人女性)好发。该病可累及全身多个脏器及血管,但最多发生于皮肤和软组织。

Radiologic overviewWith regard to musculoskeletal imaging, the most common findings of scleroderma include dystrophic soft-tissue calcifications as seen in this case. Acro-osteolysis (resorption of the tuft of the distal phalanx) can occur due to tight atrophic skin. There is often soft-tissue atrophy and erosive changes of the distal and proximal interphalangeal joints.

骨关节X线检查,硬皮病的最常见的表现为软组织内营养不良型钙化。指骨远端骨质紧贴萎缩的皮肤,有时可见肢端骨质溶解。软组织萎缩,远节指间关节、近节指间关节可见侵蚀性改变。

Other common radiologic findings in scleroderma include pulmonary fibrosis and dilation and dysmotility of the gastrointestinal tract (most commonly affecting the esophagus).

硬皮病其他常见的影像学表现包括:肺纤维化、消化道蠕动异常(最常见于食管)。

Key Points

  • Scleroderma is a progressive disease resulting in fibrosis of any organ system.

    硬皮病是一种渐进性疾病,可累及全身任何器官,引起器官的纤维化。

  • Findings include soft-tissue calcification, acro-osteolysis, skin atrophy, and interphalangeal joint erosions.主要表现包括软组织钙化、肢端骨质溶解、皮肤萎缩、指间关节受侵。
  • If the diagnosis is considered, search for findings in other examinations for supportive evidence.如果怀疑硬皮病,可以检查其他部位,发现其他组织器官的异常以支持诊断。

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