Diagnostic Radiology/Musculoskeletal Imaging/Joint Disorders/Scleroderma

Radiologic Images

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Radiographic findings in the extremities include acroosteolysis, soft tissue calcifications (calcinosis), and atrophy of the distal finger tips. Compared with hand involvement in scleroderma, foot involvement has a later onset and is relatively less frequent but can be disabling. Soft tissue changes included flexion deformities, generalized or localized atrophy, and dystrophic calcifications. In a study by Bassett et al.[1], resorption of distal phalanges was the most common bony change in patient's with scleroderma, though osteolysis in other sites (feet, ribs, and mandibles) was also frequent. Nearly 20% showed radiographic evidence of inflammatory arthritis and joint destruction that could not be attributed to overlap with rheumatoid arthritis or mixed connective tissue disease.

Radiographic findings of other organ systems include fibrosis in the lungs and abnormal esophogram related to dysmotility.

File:Scleroderma.jpg

Differential Diagnosis

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The differential diagnosis for acroosteolysis includes diseases that affect peripheral nerve sensation such as diabetes, leprosy, syrinx, myelomeningocele and congenital insensitivity to pain; injury such as frostbite, thermal or electrical burns, or snake bite; trauma; psoriasis; congenital abnormality such as with Hadju-Cheney; metabolic causes such as hyperparathyroidism. Soft tissue calcifications in the extremities can also be seen with dermatomyositis, heterotopic ossification, and metastatic calcification (hyperparathyroidism, chronic renal disease).

Treatment

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No treatment for acroosteolysis. The treatment for scleroderma varies depending on organ involvement and is beyond the scope of this discussion, but includes anti-inflammatory medication, both non steroidal and steroids.

Prognosis

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Depends on the type and degree of systemic sclerosis. There is no cure for scleroderma and most people die from involvement of the heart, lungs and/or kidney . Involvement of visceral organs is not uncommon such as GI tract (esphageal and small bowel dismotility).

References

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