Patient education: Systemic sclerosis (scleroderma) (The Basics)

What is systemic sclerosis? — This is a type of scleroderma. "Scleroderma" means "hard skin." It is the medical term for a group of conditions that affect the skin and tissues that support the organs inside the body, called "connective tissue."

The other main type of scleroderma is "localized" scleroderma. Doctors also call it "morphea." This type affects the skin and tissue just under the skin.

Systemic sclerosis can affect the skin and organs inside the body, including the heart, lungs, kidneys, and digestive system. The digestive system includes all of the structures and organs that handle and process food, such as the stomach and intestines.

What are the symptoms of systemic sclerosis? — The most common symptoms include:

●"Raynaud phenomenon" – This condition causes the fingers and toes to turn white or purple-blue in response to cold or stress.

●Puffy skin that slowly becomes hard and thicker than normal

●Joints that become stiff, because the skin around them cannot stretch as well as it used to

●Small white lumps in or under the skin on the fingers – These are called "calcinosis cutis." They contain calcium.

Less common symptoms that can happen when other organs are affected include:

●Acid reflux – This is when the acid that is normally in the stomach backs up into the esophagus, the tube that carries food from the mouth to the stomach.

●Trouble swallowing

●Diarrhea

●Shortness of breath

Will I need tests? — Maybe. Your doctor or nurse might be able to tell if you have it by learning about your symptoms and doing an exam. But you might need tests, including:

●Blood tests

●Echocardiogram – This uses sound waves to create an image of your heart as it beats.

●CT scan of the chest – This is an imaging test that creates pictures of the inside of your lungs.

●Breathing tests, also called "pulmonary function tests" – These measure how well your lungs are working.

How is systemic sclerosis treated? — Treatments include:

●Tests every few weeks or months to check your blood pressure and check how well your kidneys and lungs are working

●Prescription medicines to treat symptoms in different parts of the body

●Surgery to remove calcinosis

What can I do on my own to feel better? — You can use special creams with "lanolin" (a greasy substance found in wool) to help keep your skin moist. If itching is a problem, you can take over-the-counter antihistamines, such as diphenhydramine (sample brand name: Benadryl).

What if I want to get pregnant? — If you have scleroderma, you might have a harder time getting pregnant than those without the disease.

During pregnancy, some of your symptoms (such as Raynaud phenomenon) might get better. But other symptoms (such as heartburn) might get worse. If you have kidney problems caused by scleroderma, pregnancy can be very dangerous and even life-threatening.

What will my life be like? — Because scleroderma affects the way you look and, in some cases, your ability to do everyday things, the disease can cause stress and worry. Your doctor can refer you to a counselor or a local scleroderma support group for advice on coping with the condition.

More on this topic

Patient education: Raynaud phenomenon (The Basics)
Patient education: Acid reflux and GERD in adults (The Basics)

Patient education: Raynaud phenomenon (Beyond the Basics)
Patient education: Gastroesophageal reflux disease in adults (Beyond the Basics)