Systemic sclerosis- A sneak peek Systemic Sclerosis is a kind of multisystem auto-immune disorder which results into increased fibroblast activity. It causes vascular damage and fibrosis. Fibrosis is a disorder that affects skin, gastro intestinal tract and other internal body parts. Types Limited Cutaneous Systemic Sclerosis or popularly known as limited scleroderma This disease affects majority of people Affect is on limited body parts like face, forearms and lower legs up till knees The older version for limited scleroderma is called as CREST syndrome that means Calcinosis, Raynaud’s disease, Esophageal dysmobility Type 2- Diffuse Cutaneous Systemic Sclerosis or popularly known as diffuse scleroderma It affects 30% of people The major body parts affected are upper arms, thighs and trunk. Other Kind These are rare kind of disorders including systemic sclerosis sine scleroderma, in which internal organ is involved without causing any change in the skin. Causes of Systemic Sclerosis It is an auto-immune disorder that means the immune system damages attacks its own body’s cells. Normally, human body is made from proteins called as antibodies that help to fight against infections; for instance, when we are exposed to flu or have sore throat these anti-bodies kills the germ causing infection. In systemic sclerosis, the immune system damages connective tissue cells, which are commonly available in many body parts. The connective tissue binds or separates other tissue and organs of the body. It acts as packing tissue for the body. So, the systemic sclerosis can also be classified as connective tissue disorder disease. It not only causes systemic sclerosis, but there is an inherited tendency of developing it. It may be further triggered by certain germs, chemicals and virus. The cells called as fibroblasts affect the immune system and make excessive amount of collagen in human body. This collagen gets stored in skin, blood vessels and other internal organs. This cause scarring and thickening called as fibrosis. Who get affected by this disease? This disease affects 1 in 10,000 people in the UK, which means around 6000 people suffer from this disorder. Women are more likely to get affected by this disease. It usually starts between the age group of 30 to 50 years, but it can affect people from any age group. This disorder is rarely seen in children. Symptoms There are lot of indicators of this disease. Though it may vary person to person Raynaud’s Phenomenon The earliest symptom is called as Raynaud phenomenon. It is a stage where temporary changes appear to the skin in response to stress or cold- generally in fingers, nose and toes. The skin changes its colour to blue, pale and then red. It returns to normal when it is not stressed. Other symptoms
Patches of hard skin appears. Swelling of toes and finger is an early symptom. Hand movement is difficult Ulcer on the finger tips Chalky hard swelling known as calcinosis appears on the skin Dry or itchy skin with minimal hair growth General symptoms Fatigue Weight Loss
Oesophagus Symptoms
Heartburn or acid reflux Feeling full after meals Bloating of tummy Constipation
Treatment for systemic sclerosis Presently, there is no remedy for systemic sclerosis. However, the aim of treatment can be:
To relieve complications To prevent the disorder from progressing To detect and treat complications To minimize disability
Aim 1- to relieve from common symptoms Certain exercises are recommended Heated Gloves Stomach and gut infections can be reduced with the help of medicines like omeprazole to reduce acid. If swallowing food is problem, then it is important to increase intake of water. Surgery may be needed in difficult cases, particularly if there is partial blockage or bowel incontinence.