A new classification criterion for systemic sclerosis, a chronic autoimmune disease also known as scleroderma, could help physicians diagnose and begin treating patients sooner.
The new criteria, developed by a joint committee formed by the American College of Rheumatology (ACR) and the European League Against Rheumatism, are published in the ACR journal, Arthritis & Rheumatism. They are the first changes in the criteria since 1980.
It’s welcome news to Colleen Sullivan, a National Pain Report columnist, who suffers from scleroderma and other autoimmune diseases.
“I do really believe they need a better diagnostic system. I hear story after story every day about people being misdiagnosed or have no idea what’s going on, said Sullivan.
“More times than not, people just look up their symptoms online and that’s how they discover they might have scleroderma and that just doesn’t seem right to me.”
Scleroderma is a connective tissue disease characterized by the hardening of skin tissue in the hands, arms, or face due to increased collagen deposits (limited cutaneous systemic sclerosis).
It can also take a more aggressive form and attack internal organs, causing fibrous tissue and scarring in the kidneys, esophagus, lungs and heart (diffuse cutaneous scleroderma).
Both forms are rare and, like all autoimmune diseases, difficult to diagnose. The ACR estimates that about 49,000 Americans have scleroderma. African-Americans have a significantly higher risk of developing scleroderma than Americans of European descent.
“There is a need for improved classification criteria for systemic sclerosis,” said Dr. Frank van den Hoogen from St. Maartenskliniek in The Netherlands. “The 1980 ACR criteria were not sensitive enough to identify patients with early disease or limited cutaneous system sclerosis.”
Based on the new criteria, a patient with thickening of the skin in the middle part of the fingers would be classified as having systemic sclerosis. If that criterion was not met, then seven other criteria would need to be assessed in order to diagnose scleroderma; including thickening of skin elsewhere in the fingers, fingertip lesions, pulmonary arterial hypertension, interstitial lung disease, and Raynaud’s phenomenon — an over-reaction to cold temperatures or emotional distress.
“The new systemic sclerosis classification criteria should correctly classify more patients with the disease,” said Dr. Janet Pope from Western University, St. Joseph’s Health Care London in Ontario, Canada. “Criteria that are more specific will allow for earlier identification and better treatment for those with systemic sclerosis.”
Colleen Sullivan believes an earlier diagnosis might have prevented her disease from progressing.
“I think diagnosing scleroderma is way too difficult as it is. They basically wait for a really bad symptom to appear before they will diagnose or treat it. For me the diagnosing factor was lung scarification and once you have that you’re pretty much on the road to interstitial lung disease,” said Sullivan.
“I was told at the very beginning when I got the diagnosis of dermatomyositis (DM) that very few people only get one auto-immune disease. They informed me it’s extremely likely I will develop at least one more. I always knew to be on the lookout for other conditions like scleroderma, RA (rheumatoid arthritis), or lymphoma. If they would have been able to diagnose me with scleroderma at the onset of my DM, I would have no scar tissue in my lungs.”
Tests showed that sensitivity and specificity were both greater than 90% under the new criteria for systemic sclerosis, compared to just 75% under the 1980 criteria.