Diagnosis & Treatment of Scleroderma

Scleroderma Education

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Signs & Symptoms

The most characteristic feature of scleroderma is skin thickening or fibrosis. Less visible but of major importance are the tiny lesions that occur in small blood vessels (vascular lesions), which may involve major organs.  Pain, ranging in severity from uncomfortable to debilitating, is a common characteristic of the disease. Other common symptoms may include, but are not limited to the following:

Diagnosis

Depending on your particular symptoms, a diagnosis of scleroderma may be made by a general internist, a dermatologist (a doctor who specializes in treating diseases of the skin, hair, and nails), an orthopaedist (a doctor who treats bone and joint disorders), a respirologist (a lung specialist), or a rheumatologist (a doctor specializing in treatment of musculoskeletal disorders and rheumatic diseases).  A diagnosis of scleroderma is based largely on the medical history and findings from the physical exam.

To make a diagnosis, your doctor will ask you a lot of questions about what has happened to you over time and about any symptoms you may be experiencing.

Once your doctor has taken a thorough medical history, he or she will perform a physical exam. Finding one or more of the following factors can help the doctor diagnose a certain form of scleroderma:

Finally, your doctor may order lab tests to help confirm a suspected diagnosis. At least two proteins, called antibodies, are commonly found in the blood of people with scleroderma:

A number of other scleroderma-specific antibodies can occur in people with scleroderma, although less frequently. When present, however, they are helpful in clinical diagnosis and may give additional information as to the risks for specific organ problems.

Because not all people with scleroderma have these antibodies and because not all people with the antibodies have scleroderma, lab test results alone cannot confirm the diagnosis.

In some cases, your doctor may order a skin biopsy (the surgical removal of a small sample of skin for microscopic examination) to aid in or help confirm a diagnosis. However, skin biopsies also have their limitations: biopsy results cannot distinguish between localized and systemic disease.

Diagnosing scleroderma is easiest when a person has typical symptoms and rapid skin thickening. In other cases, a diagnosis may take months, or longer, as the disease unfolds and reveals itself and as the doctor is able to rule out some other potential causes of the symptoms. In some cases, a diagnosis is never made, because the symptoms that prompted the visit to the doctor go away on their own.

Some patients have some symptoms related to scleroderma and may fit into one of the following groups:

For more information about medical tests involved initial and ongoing medical monitoring for people with scleroderma, download the Scleroderma Information Pamphlet “Medical Tests”  developed by the Scleroderma Society of Canada by clicking here.

Treatment

 

Currently, there is no treatment that controls or stops the underlying problem - the overproduction of collagen - in all forms of scleroderma. Thus, treatment and management focuses on relieving symptoms and limiting damage. Your treatment will depend on the particular problems you are having. Some treatments will be prescribed or given by your doctor. Others are things you can do on your own.  Work with your health care professionals, experienced in the care of people living with scleroderma, in order to develop a personalized treatment plan.

Because scleroderma can affect many different organs and organ systems, you may have several different doctors involved in your care. Typically, care will be managed by a rheumatologist (a doctor specializing in treatment of musculoskeletal disorders and rheumatic diseases). Your rheumatologist may refer you to other specialists, depending on the specific problems you are having. For example, you may see a nephrologist for kidney complications, a cardiologist for heart complications, a gastroenterologist for problems of the digestive tract, and a pulmonary specialist for lung involvement.

In addition to doctors, professionals such as nurse practitioners, physical or occupational therapists, psychologists, patient educators, and social workers may play a role in your care. Dentists, orthodontists, and even speech therapists can treat oral complications that arise from thickening of tissues in and around the mouth and on the face.

Following describes some medical and nonmedical treatments associated with different areas of concerns for people with systemic sclerosis:

Fingers / Raynaud’s Phenomenon

More than 90 percent of people with scleroderma have this condition, in which the fingers and sometimes other extremities change color in response to cold temperature or anxiety. For many, Raynaud’s Phenomenon precedes other manifestations of the disease. In other people, however, Raynaud’s Phenomenon is unrelated to scleroderma, but may signal damage to the blood vessels supplying the hands arising from occupational injuries (from using jackhammers, for example), trauma, excessive smoking, circulatory problems, drug use, or exposure to toxic substances. For some people, cold fingers and toes are the extent of the problem and are little more than a nuisance. For others, the condition can worsen and lead to puffy fingers, finger ulcers, and other complications that require aggressive treatment. If you have Raynaud’s Phenomenon, the following measures may make you more comfortable and help prevent problems:

For more information, download the Scleroderma Patient Education Booklet “Raynaud’s, Finger and Skin Care”  developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

 

Skin

When too much collagen builds up in the skin, it crowds out sweat and oil glands, causing the skin to become dry and stiff. If your skin is affected, try the following:

For more information, download the Scleroderma Patient Education Booklet “Raynaud’s, Finger and Skin Care”  developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

 

Joints, Pain & Fatigue

In diffuse systemic sclerosis, hand joints can stiffen because of hardened skin around the joints or inflammation within them. Other joints can also become stiff and swollen.

For more information, download the Scleroderma Patient Education Booklet “Pain and Fatigue”  developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Mouth, Dental, & Facial

Dental problems are common in people with scleroderma for a number of reasons. Tightening facial skin can make the mouth opening smaller and narrower, which makes it hard to care for teeth; dry mouth caused by salivary gland damage speeds up tooth decay; and damage to connective tissues in the mouth can lead to loose teeth. You can avoid tooth and gum problems in several ways:

For more information, download the Scleroderma Patient Education Brochure “Oral and Dental Problems”  developed by the Scleroderma Australia by clicking here

Gastrointestinal (GI)

Systemic sclerosis can affect any part of the digestive system. As a result, you may experience problems such as heartburn, difficulty swallowing, early satiety (the feeling of being full after you’ve barely started eating), or intestinal complaints such as diarrhea, constipation, and gas. In cases where the intestines are damaged, your body may have difficulty absorbing nutrients from food. Although GI problems are diverse, here are some things that might help at least some of the problems you have:

For more information, download the Scleroderma Patient Education Booklet “Gastrointestinal Tract”  developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

 

Lungs

Virtually all people with systemic sclerosis have some loss of lung function. Some develop severe lung disease, which comes in two forms: pulmonary fibrosis (hardening or scarring of lung tissue because of excess collagen) and pulmonary hypertension (high blood pressure in the artery that carries blood from the heart to the lungs). Treatment for the two conditions is different:

Regardless of your particular lung problem or its medical treatment, your role in the treatment process is essentially the same. To minimize lung complications, work closely with your medical team by doing the following:

For more information, download the Scleroderma Patient Education Booklet “Heart, Lungs & Kidneys”  developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

 

Kidneys

Renal crisis occurs in about 10 percent of all patients with scleroderma, primarily those with early diffuse scleroderma. Severe uncontrolled high blood pressure can quickly lead to kidney failure. It’s very important that you take measures to identify and treat the hypertension as soon as it occurs. These are things you can do:

For more information, download the Scleroderma Patient Education Booklet “Heart, Lungs & Kidneys”  developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Heart

Common among people with scleroderma, heart problems include scarring and weakening of the heart (cardiomyopathy), inflamed heart muscle (myocarditis), and abnormal heartbeat (arrhythmia). All of these problems can be treated. Treatment ranges from drugs to surgery and varies depending on the nature of the condition.

For more information, download the Scleroderma Patient Education Booklet “Heart, Lungs & Kidneys”  developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

 

Cosmetic

Even if scleroderma doesn’t cause any lasting physical disability, its effects on the skin’s appearance—particularly on the face—can take their toll on your self-esteem. Fortunately, there are procedures to correct some of the cosmetic problems scleroderma causes:

Chronic Disease Management / Coping & Psychological

Having a chronic disease can affect almost every aspect of your life, from family relationships to holding a job. For people with scleroderma, there may be other concerns about appearance or even the ability to dress, bathe, or handle the most basic daily tasks. Here are some areas in which scleroderma could intrude.

Appearance and self-esteem: Aside from the initial concerns about health and longevity, people with scleroderma quickly become concerned with how the disease will affect their appearance. Thick, hardened skin can be difficult to accept, particularly on the face. Systemic scleroderma may result in facial changes that eventually cause the opening to the mouth to become smaller and the upper lip to virtually disappear. Linear scleroderma may leave its mark on the forehead. Although these problems can’t always be prevented, their effects may be minimized with proper treatment. Also, special cosmetics - and in some cases plastic surgery - can help conceal scleroderma’s damage.

Caring for yourself: Tight, hard connective tissue in the hands can make it difficult to do what were once simple tasks, such as brushing your teeth and hair, pouring a cup of coffee, using a knife and fork, unlocking a door, or buttoning a jacket. If you have trouble using your hands, consult an occupational therapist, who can recommend new ways of doing things or devices to make tasks easier. Devices as simple as Velcro fasteners and built-up brush handles can help you be more independent.

Family relationships: Spouses, children, parents, and siblings may have trouble understanding why you don’t have the energy to keep house, drive to soccer practice, prepare meals, or hold a job the way you used to. If your condition isn’t that visible, they may even suggest you are just being lazy. On the other hand, they may be overly concerned and eager to help you, not allowing you to do the things you are able to do or giving up their own interests and activities to be with you. It’s important to learn as much about your form of the disease as you can and to share any information you have with your family. Involving them in counseling or a support group may also help them better understand the disease and how they can help you.

Sexual relations: Sexual relationships can be affected when systemic scleroderma enters the picture. For men, the disease’s effects on the blood vessels can lead to problems achieving an erection. For women, damage to the moisture-producing glands can cause vaginal dryness that makes intercourse painful. People of either sex may find they have difficulty moving the way they once did. They may be self-conscious about their appearance or afraid that their sexual partner will no longer find them attractive. With communication between partners, good medical care, and perhaps counseling, many of these changes can be overcome or at least worked around.

Pregnancy and childbearing: In the past, women with systemic scleroderma were often advised not to have children. But thanks to better medical treatments and a better understanding of the disease itself, that advice is changing. (Pregnancy, for example, is not likely to be a problem for women with localized scleroderma.) Although blood vessel involvement in the placenta may cause babies of women with systemic scleroderma to be born early, many women with the disease can have safe pregnancies and healthy babies if they follow some precautions.  One of the most important pieces of advice is to wait a few years after the disease starts before attempting a pregnancy. During the first 3 years, you are at the highest risk of developing severe problems of the heart, lungs, or kidneys that could be harmful to you and your unborn baby. If you haven’t developed severe organ problems within 3 years of the disease’s onset, your chances of such problems are less and pregnancy would be safer. But it is important to have both your disease and your pregnancy monitored regularly. You’ll probably need to stay in close touch with both the doctor you typically see for your scleroderma and an obstetrician who is experienced in guiding high-risk pregnancies.

For more information about living with scleroderma, download the following Scleroderma Patient Education Booklets developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley and Drs. Maureen Mayes and Khanh Ho from the Scleroderma Foundation:

 

References:

 

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