About scleroderma

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What is scleroderma?

Scleroderma is an autoimmune disease that affects many different areas of the body. Inflammation and scarring result in the disruption of structures and functions. While different organs, such as lungs, intestine, heart, kidneys or joints can be involved; skin involvement is most common. In patients, skin of the hands and feet, chest, abdomen, and/or face may become thick and hard over time1.

The main symptoms of scleroderma

Scleroderma results from when the body's immune system begins to malfunction and attacks healthy tissue in various parts of the body, causing inflammation and damage. The most common tissue affected by scleroderma is the skin. When the immune system attacks the skin in scleroderma, the body responds by producing too much collagen, resulting in a thickening, and hardening of the skin. The word scleroderma comes from Latin and translates to “hard skin".

Joint pain and stiffness in the hands can present at disease onset. In some forms of scleroderma, internal organs may also be affected. Lung or heart involvement leads to shortness of breath, cough or light-headedness. The upper and lower gastrointestinal tracts can also be involved, resulting in heart burn, difficulty swallowing, and chronic diarrhea. Although kidney involvement is rare, it can be serious and patients can rapidly develop kidney failure1,2.

Who can develop scleroderma?

Scleroderma affects approximately one in 5000 globally3. Like many other forms of arthritis, scleroderma occurs more commonly in women than in men – women develop scleroderma approximately four times more frequently than men do. Scleroderma affects people of all ages, from babies to the elderly, but it tends to occur most often between the ages of 45 and 653.

While the exact cause or causes of scleroderma remain unknown, some research indicates that people with a family history of scleroderma or other rheumatic autoimmune diseases, like rheumatoid arthritis, may be at a higher risk for developing scleroderma. As well, some researchers believe that frequent exposure to solvents and heavy metals—for example, people who work in manufacturing or refining plants—may increase the risk of developing scleroderma.

There are two main types of scleroderma2:
  • Systemic scleroderma, including:
    • Diffuse scleroderma—the most serious form of the disease, appearing as skin thickening of the upper arms, thighs, chest, and/or abdomen. In this form of the disease, there is often significant internal organ involvement, particularly of the lungs, intestine, heart, and kidneys.
    • Limited scleroderma—skin thickening restricted to the fingers, hands, forearms, face, or lower limbs from toes up to knees, which does not progress beyond these areas. This disease sub-type was previously known as the CREST syndrome, but physicians now recognize that this term does not accurately separate diffuse from limited scleroderma.
  • Scleroderma sine scleroderma—a form of systemic scleroderma in which skin thickening is absent but other features (internal organ involvement, blood antibodies) are characteristic of scleroderma.
  • Localized scleroderma—a form of scleroderma, where disease limited to skin, typically presenting with scattered areas of skin hardening. Generally, internal organs are not affected. The two main types of localized scleroderma are:
    • Morphea—characterized by round or oval patches of rough, hardened skin most often appearing on the chest and abdomen, though arms, legs, and face may also be involved.
    • Linear—characterized by a line or streak of thickened skin most often appearing on the torso, arms, or legs, though sometimes linear scleroderma can appear on the face or neck, particularly in children. Skin thickening penetrates through the skin underlying fat, rarely affecting the bone. Children and teenagers who develop scleroderma more often have the linear type of the disease.
  • It is important to note that not all people with a particular type of the disease will necessarily develop all the problems associated with that type.

    Getting a diagnosis of scleroderma4

    If your doctor believes you could have scleroderma, you will usually be referred to a rheumatologist—a specialist in the treatment of scleroderma and other diseases which affect joints. Rheumatologists have additional training and experience in the diagnosis and treatment of scleroderma and related conditions.

    The diagnosis of scleroderma is based on a combination of medical history, physical examination, and laboratory test results. Blood antibodies characteristic of scleroderma are important, although not diagnostic. Additional diagnostic tests should be performed to determine if there is involvement of the intestine, lungs, heart, or kidneys.

    Treatment for scleroderma1

    Once your rheumatologist has confirmed the diagnosis of scleroderma, there are treatments available which vary depending on the form of the disease and the involved organs. There is no known cure for scleroderma, and treatments therefore focus on controlling symptoms and preventing disease progression.

    Medications1,5 6,10

    Medication for scleroderma depends on the severity of symptoms, and involvement of internal organ. Medications used for treatment can be summarized based on the organ involvement:
    • Skin: Disease-modifying anti-rheumatic drugs (DMARDs), such as mycophenolate mofetil (CellCept®), methotrexate, and cyclophosphamide. Biologics such as Rituximab.
    • Raynauds: Calcium channel blockers, such as Nifedipine and Amlodipine. PDE-5 inhibitors ⎼ sildenafil (Viagra), tadalafil (Cialis).
    • Arthritis: Non-steroidal anti-inflammatory drugs (NSAIDs). Disease-modifying anti-rheumatic drugs (DMARDs), such as mycophenolate mofetil (CellCept®), methotrexate, sulfasalazine, and hydroxychloroquine. Biologics, similar to those used for rheumatoid arthritis.
    • Lung disease: Disease-modifying anti-rheumatic drugs (DMARDs), such as mycophenolate mofetil (CellCept®), azathioprine, methotrexate, and cyclophosphamide. Biologics such as IL-6 inhibitors and Rituximab. Anti-fibrotic medications, such as Nintedanib.
    • Pulmonary hypertension: Various classes of vasodilator medications that reduce resistance in the blood vessels by increasing their size. PDE-5 inhibitors ⎼ sildenafil (Viagra), tadalafil (Cialis). Endothelin-1 receptor antagonists — Bosentan, macitentan, Ambrisentan. Prostacyclin agonist – Epoprostenol, Treprostinil, Iloprost, Selexipag.
    • Kidney disease: ACE inhibitors are medications that help relax the blood vessels to lower blood pressure.
    The goal with medications is reduce the risk of complications, to bring symptoms under control and slow the progression of the disease, as well as to prevent or minimize damage to internal organs.

    Surgical and interventional therapy6,7
    • Amputation – can be considered for severe finger ulcers that do not improve with medical therapy.
    • Stem cell transplant – may be an option for patients with diffuse scleroderma in the early disease stage.
    • Lung transplant – often considered for patients with severe interstitial lung disease or pulmonary arterial hypertension.
    • Renal replacement therapy – considered for patients with acute kidney failure secondary to scleroderma kidney disease.
    Risk of kidney disease and blood pressure monitoring6,7

    Often used in the early stages of kidney disease in patients with scleroderma, especially those with rapidly progressive skin involvement. Patients’ kidney functions are monitored by blood work but an early sign of kidney disease is high blood pressure. Regular monitoring of blood pressure is essential. In addition, use of glucocorticoid (steroid medication) may increase the risk of kidney disease and rheumatologists should be consulted.

    Other internal organ monitoring6,7

    In patients with scleroderma, regular monitoring should be done for pulmonary hypertension (high blood pressure in the lungs) and interstitial lung disease (scarring of lungs). This cannot be tested with a blood pressure cuff. An ultrasound of the heart (echocardiogram) and pulmonary function test should be done every 6 to 12 months in most patients with scleroderma to look for early signs of these treatable complications.

    Non-medical management7

    There are things that people can do at home to self-care or “manage” scleroderma symptoms. It is important to keep skin well-moisturized and joints protected. Recommendations for people with scleroderma include:
    • Using a humidifier at home to keep skin from becoming dry
    • Keeping skin moisturized with lotions
    • Avoiding skin creams containing strong perfumes
    • Moisturizing eyes with eyedrops
    • Not smoking or quitting smoking
    • Getting sufficient physical activity, including range-of-motion exercises
    • Avoiding foods that cause heartburn/acid reflux, and avoiding meals late in the evening

    Maintaining a healthy lifestyle is also an important part of any scleroderma treatment plan. Eating foods with excess amounts of simple sugars may worsen symptoms. Well balanced meals and nutritious snacks can help to fuel the body and regulate hormones. Daily movement such as yoga and stretching help to keep muscles and joints mobile. While these habits may seem small, they can have a profound impact on one’s life. Remember to start small, and progress from there.

    Key take-aways
    • Scleroderma is not simply a condition of the skin, but a systemic disease that can affect the internal organs.
    • A holistic self-care routine includes medications, blood pressure monitoring and the adoption of lifestyle habits including a whole food diet, mindfulness, and daily movement.
    Resources Thank you to Dr. Hyein Kim, a rheumatologist based in Vancouver, British Columbia., for her medical review of the content on this page.

    1ACR website
    2NIH website
    3Bairkdar et al. (2021) Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis.
    4AADA website
    5Fett, N. (2013). Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.
    7John Hopkins Medicine
    8Poole, J. L. (2010). Musculoskeletal rehabilitation in the person with scleroderma.
    9Khanna (2019). Eating Well With Scleroderma.
    10Scleroderma Canada (2021). Medication guide.