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Systemic Sclerosis


Extra Corporeal Membranous Oxygenation (ECMO)

Systemic Sclerosis (also known as Scleroderma) is an autoimmune disease causing skin thickening and tightening along with involvement of other organs of the body. It affects women more often than men, and most commonly occurs between the ages of 30 and 50. One of the earliest symptom of Systemic Sclerosis is Raynaud's phenomenon, which can appear many years prior to skin involvement. In this, fingers and/or toes becomes pale and then turns blue, during cold weather and/or on exposure to cold water. These changes may last upto few minutes, before fingers becomes normal again. Patient may experience multiple such episodes in a single day, but few patients may experience it occasionally only during winters. In severe cases this may cause recurrent ulcers over the fingertips which are painful and difficult to heal. Nearly everyone who has Systemic Sclerosis experiences hardening and tightening of skin, which can be limited only to the limbs and face or may involve whole body. Skin can appear shiny because of this tightness and movement of the affected areas may be restricted. Skin tightness usually progresses gradually over few years, and during this time organs other than skin can also be affected, such as lungs, kidneys, heart, and digestive tract. Lung involvement results in reduced lung function, which can cause breathing difficulty and impaired tolerance for exercise. Digestive problems cause heartburn, difficulty swallowing, bloating, constipation or diarrhoea. Involvement of kidneys leads to sudden rise in blood pressure and decrease in kidney functioning. These problems if left undetected and untreated, can become life-threatening. Variety of treatment options are available which can ease symptoms, prevent progression of disease in the affected organs, and also improve the quality of life. Regular follow up with a rheumatologist is extremely important in order to monitor disease course and to promptly identify and treat organ involvement.


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