In Scleroderma, there are two main groups of illness which are systemic sclerosis or localized scleroderma. Localized scleroderma involves only the skin, however, systemic scleroderma is the most serious form of the disease which has severe skin thickening and high risk of developing internal organ involvement. This disease is generally classified as an autoimmune disease that can occur at any age but it is most common in women of childbearing age.
The scleroderma prognosis is mainly influenced by the presence of damage to the lungs, kidneys, heart and gastrointestinal systems. When the disease invades those major internal organs, it carries a survival rate of only 55% at 10 years after diagnosis. Scleroderma is known as the most fatal of all connective tissue disease. In the United States, there are an estimated 125000 active cases and 2.5 million worldwide. Therefore, a thorough examination is needed when scleroderma is suspected in order to know any involvement of multisystem.
Symptoms of scleroderma may vary from person to person and may be visible when the skin is affected and maybe not if the internal organs are affected. The organs that scleroderma does affect are many, which one of those is hands. In hand, patients may develop a digital ulcer, an open sore on their fingertips. It happens among 40% of patients with scleroderma because of poor blood flow to the fingertips which is in turn related to the narrowing of blood vessels, whereas others will experience sclerodactyly and joint contractures.
Another typical early symptom of scleroderma is involving the musculoskeletal system which is swelling or puffiness of hands that may occur especially in the morning due to muscle inactivity at night. The swelling may cause patients difficulty to close the hand into a fist and can be reduced by the exercise of fingers and toes. Scleroderma also may be manifested by pain, warmth, stiffness or tenderness of the joints due to the arthritis-like joint inflammation. Raynaud phenomenon is the most common example of Scleroderma affecting the vascular system, where the endothelium layer of blood vessels is damaged.
Next, the involvement of the gastrointestinal system may develop after the Raynaud phenomenon and skin thickening. This is a prominent feature of systemic scleroderma due to the overproduction of collagen and causes thickening and fibrosis of the connective tissue from mouth to the anal canal. Patients will have oesophagal dysfunction when scleroderma weakens the sphincter in the oesophagus and leads to swallowing difficulties. Patients may have diarrhoea when it involves a small bowel because of loss of normal muscle contraction and bacteria grow in there. Constipation happens when there is large bowel involvement. It is because of the weakening of the muscles of the large bowel.
The lung is a vital organ that can also be affected by scleroderma. This can make patients go breathless due to the build-up collagen thickens lung tissue and causes fibrosis or scarring, making the transport of oxygen into the bloodstream more difficult. Scleroderma also can lead to pulmonary fibrosis where there is a build-up of scar tissue in the lungs and pulmonary hypertension where blood pressure in the blood vessels of the lungs is high and lead to failure of the right side of the heart. The heart also can be affected when the heart muscle becomes thickened and fibrous scar tissue accumulates, then may ultimately result in heart failure due to a decrease of the pumping force.
Another vital organ is the kidney/ The disease becomes serious and dangerous when a renal crisis has occurred which is manifested by a sudden rise in blood pressure, shortness of breath, chest pain or discomfort, or mental confusion. When this happens, patients need to be treated promptly or else, they can develop kidney failure. Dialysis may be required as the last choice in cases of severe kidney failure. Hence, blood pressure and kidney function need to be monitored at regular intervals for people with scleroderma. The prognosis of scleroderma can be good if people may recover successfully from a renal crisis, but only if the problem is recognized and treated quickly.