The Importance of Cancer Screenings in Patients with Scleroderma, Dermatomyositis


This year, because of COVID-19, many healthcare-related events and symposiums have been moved online. However, that doesn’t mean that the insights are any less helpful! In the virtual ACR State-of-the-Art Clinical Symposium, Dr. Ami Shah, MD, spoke not only about the importance of cancer screenings for patients with scleroderma and dermatomyositis, but how to properly address these screenings.


Doctors don’t quite know the cause of dermatomyositis, but some believe that the rare inflammatory disease shares elements of an autoimmune disorder. Dermatomyositis is categorized by a distinctive skin rash as well as muscle weakness. It tends to affect females more than males.

Symptom onset usually occurs between the ages of 40-60. These include:

  • A scaly scalp or hair loss
  • Tender, weak, and easily fatigued muscles
  • Rashes on the eyelid margins, knuckles, upper outer thighs, and any other exposed skin
  • Intense and itchy skin lesions
  • Skin reddening on the mid-face

Learn more about dermatomyositis here.

Systemic Scleroderma

Systemic scleroderma is also known as systemic sclerosis. This autoimmune disorder occurs when excess collagen in the body leads to the development of scar tissue (fibrosis) on the skin and organs. It affects females 4x more often than males.

Symptom onset usually occurs in middle age. Common symptoms include:

  • Joint pain and swelling
  • Gastrointestinal distress
  • Open sores on the fingers
  • Hard, tight patches of skin
  • Raynaud’s phenomenon
  • Heartburn
  • High blood pressure
  • Shortness of breath
  • Kidney issues

Learn more about scleroderma here.

Performing Cancer Screenings

As stated by Dr. Shah, there is a high cancer risk during scleroderma and dermatomyositis onset. But cancer screenings play an important role in patient outcomes beyond just early detection. First, it can change the type of treatment. For example, says Shah, cancer could worsen autoimmune diseases. Alternately, inflammatory diseases can signify cancer. As a result, doctors need to consider whether cancer therapy can actually be used to treat patients who may be showing another condition.

So, understanding how patients might progress will play a crucial role in their treatment. Additionally, cancer screenings can identify patients with a high risk to ensure that they – and doctors – are monitoring their condition.

What Should Screenings Include

Targeted cancer screenings should consider:

  • Age
  • Sex
  • Risk factors
  • The presence of specific antibodies
    • For example, patients with scleroderma who are positive for anti-RNA polymerase III or anti-RNPC3, or negative for RNA polymerase III and centromere topoisomerase 1, have a higher risk of developing cancer or renal failure.
      • Anti-RNA polymerase III may cause a sudden, but intense, period of external fibrosis. Patients may not immediately present with Raynaud’s phenomenon, but it might develop later. Additionally, patients who are positive for this antibody are also at a higher risk of renal crisis, gastric antral vascular ectasia (gastrointestinal bleeding categorized by an internal “watermelon stomach” appearance), and myopathy (muscle weakness).
    • Alternately, 83% of patients with cancer-related or associated dermatomyositis show the presence of anti-TIF1 gamma and anti-NXP2 antibodies.
  • Age during disease onset
    • Older ages at disease onset are more at risk of developing cancer.
  • Family history
  • Large-scale, unintended weight loss
  • Failure to respond to treatment
  • Aggressive disease progression
  • Atypical disease presentation

Cancer Screenings for Dermatomyositis, Scleroderma

Screening for high-risk females should, starting at age 40, include:

  • A pelvic exam
  • Ultrasound
  • Mammography

However, if the patient has a family history of cancer or any physical characteristics (such as a lump), they should be screened earlier.

Screening for high-risk males should, starting at age 40, include:

  • Colonoscopy
  • Testing for prostate-specific antigens

Abnormal levels of prostate-specific antigens suggests cancer. Additionally, Shah notes that African-American patients are at a higher risk of developing both dermatomyositis and cancer, so screening for this group is especially important.

From Shah’s own experience, she also recommends:

  • Potential CT scans of the chest, abdomen, and pelvis for patients with dermatomyositis
  • Performing a physical exam, which includes a breast exam
  • Referring patients to gynecologists for necessary examinations
  • Examining the ear, nose, and throat in patients with scleroderma if they have trouble swallowing or feel like something is stuck in their throat

Read the original article here.

Jessica Lynn

Jessica Lynn

Jessica Lynn has an educational background in writing and marketing. She firmly believes in the power of writing in amplifying voices, and looks forward to doing so for the rare disease community.

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