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.
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)
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.
Scleroderma
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Based on the provided whole-body and chest-abdominal imaging, the following main features can be observed:
Combining the patient’s medical history (scleroderma, previous lymphoma) with the above imaging findings, the following diagnostic directions can be considered:
Based on the patient’s disease background (scleroderma and positive anti-PM-Scl antibodies) and the imaging evidence of widespread muscle atrophy and fat replacement, along with clinical symptoms (weight loss, difficulty breathing possibly due to swallowing/esophageal involvement and decreased muscle strength), the most likely diagnosis is myopathic changes in scleroderma overlapping with polymyositis.
If there is still doubt, further tests such as a muscle enzyme panel (CK, AST, LDH, etc.), electromyography, or muscle biopsy can be performed to clarify the degree and nature of the muscle lesion. Excluding lymphoma recurrence or other malignancies is also essential; if necessary, PET-CT or further imaging follow-up can be undertaken.
For treating scleroderma complicated by myositis, the standard approach generally includes:
When formulating an exercise rehabilitation plan, it is necessary to consider the patient’s cardiopulmonary function and muscle status, following the principles of gradual progression and individualization. A possible reference plan is as follows:
This report provides a reference analysis based on the existing imaging and clinical history and cannot substitute for face-to-face consultation or professional medical advice. Specific diagnoses and treatment plans should be made by a specialist physician using clinical examinations, further imaging, and laboratory results for a comprehensive evaluation.
Scleroderma