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Dr. Andrew Rynne
MD
Dr. Andrew Rynne

Family Physician

Exp 50 years

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Pulmonary Hypertension, Possible Scleroderma ,

My daughter age 37 just diagnosed with Pulmonary hypertension - did right cath today. She has been otherwise healthy and now saying possibly underlying scleroderma . This is not good by what I have been reading - please tell me what the prognosis with drugs and what her lifestyle will be like. Thanks. Concerned parent!
Wed, 16 May 2012
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General & Family Physician 's  Response
Hello
Scleroderma is an autoimmune disease of the connective tissue featuring skin thickening, spontaneous scarring, blood vessel disease, varying degrees of inflammation, associated with an overactive immune system. Autoimmune diseases are illnesses that occur when the body's tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body. This leads to thickness and firmness of involved areas. Scleroderma, when it's diffuse or widespread over the body, is also referred to as systemic sclerosis.
Treatment of scleroderma is directed toward the individual feature(s) affecting different areas of the body.

Aggressive treatments of elevations in blood pressure have been extremely important in preventing kidney failure. Blood-pressure medications, particularly the angiotensin converting enzyme (ACE) inhibitor class of drugs, such as captopril (Capoten), are frequently used.

Some research indicates that colchicine can be helpful in decreasing the inflammation and tenderness that periodically accompanies the calcinosis nodules in the skin. Skin itching can be relieved with lotions (emollients) such as Eucerin, Lubriderm etc.
  Our understanding of the effects of the immune system in scleroderma is greatly improving. A vast number of clinical research trials are under way that are evaluating potential treatments for various aspects of the disease to improve the prognosis for patients with scleroderma.
Thanks
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Pulmonary Hypertension, Possible Scleroderma ,

Hello Scleroderma is an autoimmune disease of the connective tissue featuring skin thickening, spontaneous scarring, blood vessel disease, varying degrees of inflammation, associated with an overactive immune system. Autoimmune diseases are illnesses that occur when the body s tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body. This leads to thickness and firmness of involved areas. Scleroderma, when it s diffuse or widespread over the body, is also referred to as systemic sclerosis. Treatment of scleroderma is directed toward the individual feature(s) affecting different areas of the body. Aggressive treatments of elevations in blood pressure have been extremely important in preventing kidney failure. Blood-pressure medications, particularly the angiotensin converting enzyme (ACE) inhibitor class of drugs, such as captopril (Capoten), are frequently used. Some research indicates that colchicine can be helpful in decreasing the inflammation and tenderness that periodically accompanies the calcinosis nodules in the skin. Skin itching can be relieved with lotions (emollients) such as Eucerin, Lubriderm etc.   Our understanding of the effects of the immune system in scleroderma is greatly improving. A vast number of clinical research trials are under way that are evaluating potential treatments for various aspects of the disease to improve the prognosis for patients with scleroderma. Thanks