Are You Sclero-aware?

Let’s talk about Scleroderma, also known as systemic sclerosis.

It’s June so that means it’s Scleroderma Awareness Month. Scleroderma is a rare, incurable, autoimmune connective tissue disease that affects connective tissues and blood vessels, resulting in fibrosis and thickening of the skin, as well as damage to internal organs. There is excess accumulation of collagen, leading to a hardening and tightening of the skin and tissues. The word scleroderma comes from Greek: sclero = hard, derma = skin. And that is literally what happens to folk with scleroderma.

Scleroderma can affect the skin, lungs, heart, kidneys, and gastrointestinal tract. It is characterized by fibrosis, inflammation, and vascular damage. It is broadly classified as either limited cutaneous systemic sclerosis (lcSSc) or diffuse cutaneous systemic sclerosis (dcSSc). Limited Cutaneous Systemic Sclerosis (lcSSc) affects the skin of the fingers, face, and neck, and can also cause Raynaud’s phenomenon and pulmonary hypertension. Diffuse Cutaneous Systemic Sclerosis (dcSSc) affects the skin of the fingers, hands, and arms, as well as the trunk and legs. It affects the lungs differently and is more liking to cause Interstitial Lung Disease (ILD).

Q. How rare is it?

The estimated prevalence is 50 cases per million population. It is more common in women than men, with a female-to-male ratio of 4:1 (like most autoimmune disease). Scleroderma usually develops between the ages of 30 and 50, although it can occur at any age, including children. People of African and Hispanic descent have a higher incidence.

 No one knows exactly why scleroderma occurs, but it is believed to be a combination of genetic and environmental factors. Risk factors include a family history of autoimmune disease, exposure to certain chemicals, and viral infections.

❌ Beware Raynaud’s developing late in life❌

The most common symptom is Raynaud’s phenomenon, which causes the fingers and toes to turn white, red & blue in response to cold or stress. Primary Raynaud’s is a common, non-threatening circulation issue that develops in young people (teenage years/early 20’s) BUT developing Raynaud’s late in life is usually indicative of underlying disease, especially autoimmune connective tissue disease.

Other symptoms include skin thickening, joint pain, muscle weakness, fatigue, and shortness of breath. Scleroderma can affect internal organs, and cause pulmonary fibrosis, pulmonary hypertension, heart failure, renal failure, and intestinal dysmotility.

❌ If you have scleroderma and you develop a persistent dry cough or get breathless easily, this could be a sign of lung involvement so see your specialist ASAP❌ 

While we don’t yet have a cure, there are treatments available to help manage some of the symptoms and complications, such as digital ulcers and ILD. I’ll delve into this in future blogs and also look at the benefits of yoga and meditation for scleroderma warriors

How has scleroderma affected you?
Share your experience, connect with others and spread awareness ⤵️