SCLERODERMA
It is an autoimmune disease that involves the skin and connective tissues which provide support and framework of the body. In some people it affects only the skin, but in mostly cases, affects other structures beyond skin such as blood vessels, internal organs and digestive tract.
It affects female more than male, most commonly in 30-50 yrs. It is very rare, but family members of those with scleroderma or other autoimmune connective tissue disease such as lupus have higher risk of getting it.
What causes Scleroderma?
Exact cause of scleroderma is not known, though many researchers have found certain genetic linkage and environmental factors (eg exposure to silica or chemical toxins) to play a role.
Classifications of Scleroderma:
It is an autoimmune disease that involves the skin and connective tissues which provide support and framework of the body. In some people it affects only the skin, but in mostly cases, affects other structures beyond skin such as blood vessels, internal organs and digestive tract.
Scleroderma is classified in terms of degree and location of skin and organ involvement.
1. Localized (limited to skin, sometimes bone, muscle and joints are involved but internal organs are spared).
2. Systemic( serious form of disease with involvement of skin, muscles, joints, blood vessels, lungs, kidneys, heart and other organs).
Symptoms of Scleroderma?
Symptoms and signs of Scleroderma vary depending upon which parts of your body are affected.
Skin: hardening and tightening of patches of skin. The patches may be oval or straight lines, mainly covering the wide areas of trunk and limbs. Skin may appear shiny with restricted movement.
Finger or toes: Numbness, pain or color changes in finger and toes when exposed to cold temperatures or emotional distress. This is the earliest sign of scleroderma, and also known as Raynaud’s disease.
Digestive symptoms: Throat discomfort like stickiness and acid reflux.
Heart, lungs and kidney: Affect the function of heart, lungs and kidneys to varying degrees, and if untreated may be life-threatening.
How is Scleroderma diagnosed?
Diagnosis may be tricky as it may present similarly to other diseases and there is no definitive blood test to confirm scleroderma, though numerous antibodies have been associated with this condition. So for the diagnosis clinical history, clinical signs, lab test for inflammatory markers, and auto antibodies test is done. ANA, dsDNA, ENA panel, chest x-ray and chest CT scan to rule out any complications. Nail fold capillaroscopy (NFC), which is also available in our center, is a must for early diagnosis of Scleroderma and also to identify whether they are in a risk for development of vascular and visceral complications and death.
Treatment of Scleroderma:
No drug has been developed that can stop the underlying process of scleroderma, but variety of medications are available to control the symptoms and its complications.
- Raynaud’s can be treated with calcium channel blocker or PDE-5 inhibitors.
- Heart burn can be treated with antacids drugs like PPI(pantoprazole, omeprazole, esmoprazole.etc)
- Scleroderma kidney disease may be managed with ACE inhibitors.
- Muscle pain and weakness can be treated with anti-inflammatory drugs like steroids and/or immunosuppressive drugs.
- Interstitial lung disease managed with intravenous cyclophosphamide or mycophenolate mofetil.
Still many researches are going on for newer treatments in scleroderma.
Physiotherapy:
Patients with scleroderma should also consult with therapist who can help them to:
- Manage pain
- Improve the strength and mobility
- Maintain independence with daily tasks.
Health impact of Scleroderma:
Living with Scleroderma is quite challenging and depressing though, symptoms may vary. As it may involve multiple organs, everyday activities sometimes become very difficult leading to pain and physical limitations.
· Patients are asked to go for the regular visit with consultant doctors especially rheumatologist, pulmonologist.
· They are asked to eat more frequent small meals than fewer large meals to relieve form acid reflux.
· They are advised to keep their skin well-moisturized to lessen stiffness and preventing trauma to finger tips by avoiding work like gardening, cooking etc.
· Avoidance from cold exposure by wearing socks, gloves and boots.
Most important is the support of family and friends which also plays a vital role in maintainaing a good quality of life.