Scleroderma and CREST syndrome
In scleroderma (systemic sclerosis), excessive collagen deposition occurs. On radiographs, this manifests as soft tissue and bone changes, but without destruction of the joints themselves.
Key radiological findings:
- Soft tissue calcinosis: amorphous deposits, most commonly in the fingers.
- Acroosteolysis: resorption of the tufts of the distal phalanges (especially digits I, II, and III of the hand). Resorption may also affect the angle of the mandible and the ribs.
- Atrophy: thinning of the soft tissues over the distal phalanges and flexion contractures.
Joint erosions are not characteristic of scleroderma, but arthropathy is frequently accompanied by osteopenia.
Dermatomyositis and polymyositis
These diseases affect skeletal muscles, and dermatomyositis also affects the skin.
The main radiological sign is extensive calcification of subcutaneous tissues along muscle groups and fascia. The most typical localization is the proximal lower extremities (thighs) and pelvis.
Joints are rarely involved: radiographs may show only periarticular osteopenia or edema. To assess the state of the muscles themselves (edema, fatty infiltration, atrophy), MRI is preferred.
Systemic lupus erythematosus (SLE)
Arthropathy in SLE occurs in the majority of patients. The hands and wrists are most commonly affected. The main finding is synovitis, which is best visualized on ultrasound or MRI.
Specific radiographic signs:
- Deforming non-erosive arthropathy: erosions are atypical (their presence suggests overlap syndrome with rheumatoid arthritis).
- Reversibility of deformities: subluxations (e.g., ulnar deviation of the metacarpophalangeal joints) and finger hyperextension are caused by ligamentous laxity. When positioning the patient for imaging, these displacements may reduce, and the joint appears normal.
- Osteonecrosis: the femoral and humeral heads are frequently affected.
Diffuse idiopathic skeletal hyperostosis (DISH)
Forestier's disease (DISH) is a condition of elderly individuals characterized by marked ossification of entheses (sites of ligamentous attachment to bone).
The classic picture is observed in the spine (most commonly in the thoracic region Th7–Th12):
- "Flowing" ossification of the anterolateral surface of at least 4 contiguous vertebrae.
- Intervertebral disc height is preserved (unlike osteoarthritis).
- Absence of facet joint ankylosis and sacroiliac joint involvement (unlike ankylosing spondylitis).
- Ossification predominantly occurs along the anterolateral right surface (aortic pulsations on the left suppress bone formation).
The disease has a minimal clinical presentation, but occasionally hyperostosis in the cervical spine causes dysphagia.
Hemophilic arthropathy
Joint involvement in hemophilia (A and B) occurs due to recurrent intra-articular hemorrhages. Blood causes synovial inflammation, which triggers a cycle of destruction.
Radiological stages and signs:
- Acute hemarthrosis and hyperemia lead to periarticular osteoporosis. In children, chronic hyperemia causes premature closure of growth plates and epiphyseal hypertrophy.
- Chronic synovitis leads to cartilage loss (joint space narrowing) and erosions.
- Secondary osteoarthritis develops: subchondral cysts, osteophytes, sclerosis.
Specific signs include widening of the intercondylar notch of the knee joint and enlargement of the trochlear notch of the elbow joint. Ultrasound and MRI are used for early diagnosis (detection of blood, synovitis, hemosiderin).
Briefly: answers to topical questions
What radiological signs are characteristic of pulmonary involvement in systemic sclerosis?
Pulmonary involvement in systemic sclerosis is characterized by signs of fibrosis detected on high-resolution computed tomography. The main radiological manifestations include:
- Peripheral reticular opacities (reticular opacities) — a typical sign of fibrosis.
- Ground-glass attenuation (ground-glass attenuation) — associated with traction bronchiectasis.
- Honeycomb destruction (honeycomb destruction) — encountered rarely.
Changes are predominantly localized in the lower lobes of the lungs.
What muscle MRI changes are characteristic of the active phase of dermatomyositis?
The active phase of dermatomyositis on MRI is characterized by signs of soft tissue and muscle edema:
- Soft tissue edema — may be visualized on T1-weighted images.
- Soft tissue and muscle edema — more clearly defined in STIR mode with fat suppression.
- Perifascicular fluid (perifascicular fluid) — also better detected in STIR mode.
What ultrasound signs of synovitis are detected in systemic lupus erythematosus?
In systemic lupus erythematosus, ultrasound is used to detect synovitis, although specific sonographic signs are not detailed in the sources. It is known that power Doppler ultrasound is used for diagnosis. This method acts as an adjunct to clinical examination and allows confirmation of the presence of synovitis, as well as concomitant tenosynovitis in complex clinical situations.
CREST syndrome in scleroderma: Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia.
Class and exam questions
How does arthropathy in SLE differ from rheumatoid arthritis on radiographs?
In SLE, arthropathy is non-erosive, and deformities (caused by ligamentous laxity) are often reversible and may not be visualized on imaging due to proper hand positioning.
What is the main radiological difference between Forestier's disease (DISH) and osteochondrosis?
In DISH, bony "bridges" form between at least 4 vertebrae, while intervertebral disc height remains relatively preserved.
What X-ray changes in the fingers are typical for scleroderma?
Acroosteolysis (resorption of the distal phalanges) and amorphous soft tissue calcinosis without destruction of the joints themselves are most characteristic.
What else to study on this topic
- Rheumatic fever and Jaccoud's arthropathy
- Mixed connective tissue disease (MCTD)
- MRI semiotics of intramuscular hematomas in hemophilia
- Differential diagnosis of soft tissue calcinosis
- Complications of Forestier's disease: dysphagia and mechanical blocks
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