Scleroderma &/or lymphoma ?

[shell_l_d]

Hi, My mum (54yo) has suspected scleroderma, which started in her big toe apparently 3yrs ago, it has now moved up her legs & into her buttocks, it was also evident in her hands. Purple, shiny, swollen, hard. At least one of her ankles won't turn due to tightness/swelling (think she said).

She had a CT scan & ECG done the other day via her GP. She has the CT scan results which she read to me & included such things as:
* many enlarged lymph nodes
* mesenteric nodes
* pelvic phleboliths
* pelvic chain nodes
* inguinal nodes
* left renal hilum
* concerns about: aorta / IVC / mesentery
* tests to rule out lymphoma should be done
* esophigas (sp?) is affected - possible scleroderma)

The ECG results don't come in til Wed, but she is going out of her mind with worry at the moment. When the GP has all these results then he'll see her & refer her to a specialist, but her GP aptmt isn't until 12Dec apparently.

Any insight please? Thanks so much.

She also has narcolepsy (well a rarer form, whereby the only diff is she doesn't go into REM sleep) & medication is not helping, she see's this specialist 1-3mthly & he said something like 99% of his patients episodes are controlled by the meds, whereas hers is not. It is far worse in the warmer weather.

She also has permanent back/neck injuries.

[shell_l_d]

Furthermore...
there were also lymph nodes near her liver & aorta that were concerning & they had alot of trouble trying to inject the dye into her veins for the CT scan so ended up having to give her some type of drink instead, so her veins appear to be affected too. The person at radiology before the scan said looks like u have scleroderma! I was reading on the internet about scleroderma & it sounds like systemic scleroderma... & somewhere it said life expentancy up to 5yrs from onset!!!

[Dr. Safaa Mahmoud]

Hello,
Systemic sclerosis is used to describe involvement of different body organs and tissues with fibrosis and chronic inflammation. These organs include the skin, the viscera (commonly esophagus), the lung, the kidney and the immune system.

The disease is diagnosed mainly on clinical basis with major and minor criteria provided by the American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis

The major criterion is symmetric thickening, tightening, and induration of the skin of the fingers (proximal to the metacarpophalangeal or metatarsophalangeal joints) that may extend to the entire extremity as well as the head and the trunk.

Confirmatory tests include the presence of autoantibodies (ANA) in the blood of the majority of cases and histopathological characters of tissue biopsy if taken.

Blood vessels can be affected in different ways and lymph node involvement as a secondary effect to changes in the involved organs drained by them is not uncommon.

The association between scleroderma and lymphoma is uncommon and there are no data that support a casual relationship. If these lymph nodes are small enough to be reactionary, the physician may not consider biopsy. Otherwise the only way to reach the diagnosis would be through a biopsy from the most significant and the most accessible lymph node.

If it is a lymphoma associated with scleroderma her doctor may treat her as lymphoma since scleroderma symptoms have been shown to improve also with lymphoma treatment. Symptomatic treatment and local skin measures are essential parts of the treatment aproach.

Life expectancy in scleroderma is very individualized, and depends on the extend of skin and visceral involvement as well as their response to therapy.
In general, in limited disease, majority of patients are expected to live up to 20 years while those with extensive diseases are expected to live 5 years or more.

Unfortunately you will have to wait for the evaluation of the specialist to reach the diagnosis.

I would advise you to follow up with her doctor. Only by complete history and direct clinical examination the proper management can be reached.
Please keep us updated.
Best regards.

[shell_l_d]

Thanks so much for your reply Dr Mahmoud.

Mum's GP trying to get urgent oncologist & rheumatoligist appointments. She has another aptmt with him tomorrow. When she got home, she rec'd a letter from bowel cancer screening saying her results were positive, so yet another matter to look into.

Will keep u posted. The GP didnt order the ANA blood tests nor the biopsies, he said it should be all handled by specialist instead.