Scleroderma

Clinical History

A 61 year old female with cutaneous scleroderma and chemotherapy treated lymphoma in the past presented with abdominal distension, weight loss and shortness of breath.
A staging CT was requested to exclude a lymphoma recurrence or scleroderma affecting the bowel.

Imaging Findings

CT showed dilatation of the lower and mid oesophagus with air fluid level in the upper oesophagus (Fig 1-3). A barium study confirmed the findings (Fig 4) and demonstrated reflux on fluoroscopy. There was extensive fatty replacement of the skeletal muscles in the whole body with dystrophic muscular calcification (Fig 5-7).
There are insufficiency fractures in the finger (which needed orthopaedic fixation as treatment of non healing fractures probably due ischaemia) and ankle (Fig 8, 9).
MR angiogram showed arterial narrowing which is more obvious in the right distal iliac and both common femorals (Fig 10)

Discussion

Scleroderma is an autoimmune condition in which the cells in the connective tissue produce too much collagen, causing fibrosis of the tissue. Collagen is found in the skin, blood vessels, joints and internal organs, such as the kidneys, heart, lungs and bowels. There are several types of collagen and different forms are found in different parts of the body. The symptoms depend on the organs affected and the severity of the condition and vary from heart burn due to oesophageal smooth muscle affection to Raynaud's phenomenon due to vascular involvement. Proximal muscle involvement in Scleroderma is indistinguishable from other myopathies [1].
The unusual clinical presentation of abdominal distention and weight loss was due to fatty replacement of the normal musculature. Raynaud's phenomenon is a known association [Fig1] but diffuse muscle atrophy is not a known association.
Anti-PM-Scl antibody - strongly associated with the combination of myositis and scleroderma was present.
Symptom management is the usual treatment [2].

This patient was referred for increasing shortness of breath and weight loss; possibly lymphoma recurrence. This is a case where review of old imaging findings could explain the presenting symptoms. In this case the weight loss was due to muscle atrophy and changes in the lungs were due to aspiration.
Take home message: Explain presenting symptoms which might be due to disease or associated complications with the help of recent and previous imaging findings.

Differential Diagnosis List

Final Diagnosis

Scleroderma

URL:	https://www.eurorad.org/case/12585
DOI:	10.1594/EURORAD/CASE.12585
ISSN:	1563-4086