Articles and Publications
DOCUMENTS ON VARIOUS TOPICS RELATED TO SCLERODERMA
Scleroderma, or systemic sclerosis, is a relatively misunderstood chronic disease affecting about one in 5,000 people. In Quebec, it is estimated that several thousands of people suffer from scleroderma. Disease onset usually occurs in the fourth decade of life and is five time more prevalent in women than men. Scleroderma is one of the so-called “autoimmune” diseases in which the body’s antibodies attack its own cells… Read more
The name «scleroderma» is derived from the Greek words «sclero», meaning hard and «derma», meaning skin. Thus, the characteristic feature of scleroderma is the hardening of the skin. Scleroderma is generally divided into two main forms: localized scleroderma and systemic scleroderma (or systemic sclerosis)… Read more
The most common and earliest problem observed in systemic sclerosis is Raynaud’s phenomenon (RP). This phenomenon is due to a narrowing of the blood vessels in the fingers caused by exposure to cold and strong emotions, and is manifested by a change in colour of the fingers, which turn white, then blue and finally red… Read more
Pulmonary fibrosis is a common manifestation of systemic sclerosis, being present in about half of the patients. However, pulmonary fibrosis is severe in only about 15% of patients… Read more
PAH in systemic sclerosis is due to an exaggerated and progressive narrowing of the small blood vessels in the lungs. This is caused, on the one hand, by the increased presence of molecules (chemical signals) that promote the contraction and obliteration of the pulmonary arteries… Read more
Cardiac involvement occurs in 10 to 30% of patients with systemic sclerosis. Cardiac involvement occurs in both the limited and diffuse forms of the disease, but is generally more frequent and severe in patients with diffuse disease… Read more
The digestive tract is involved in nearly all systemic sclerosis patients and can be present even in the absence of symptoms in half of patients. The frequency is similar in diffuse and limited forms of systemic sclerosis. However, severe manifestations are uncommon, occurring in less than 10% of patients… Read more
The kidneys play a critical role in removing chemical waste products from the body, maintaining body fluid volume and controlling blood pressure. Kidney abnormalities encountered in systemic sclerosis are relatively common and, fortunately, most often with few consequences… Read more
The main symptom of myositis is usually muscle weakness, mainly in the shoulders and hips. People with myositis may have difficulty lifting their arms above their shoulders, lifting heavy objects, climbing stairs or getting up from a seat… Read more
The prevalence of osteoporosis is increased in people with scleroderma, affecting approximately 30% of individuals. This increased risk may be related to the presence of risk factors for osteoporosis, such as advanced age, early menopause use of glucocorticoid drugs, malabsorption related to bowel involvement, vitamin D deficiency and chronic inflammation… Read more
There are two types of scleroderma in children.
The first is called «localized scleroderma». This is the most common in children. It affects the skin in one area of the body only. The other type is called «systemic scleroderma». This can affect many areas of the skin, as well as the organs of the body. It is rare in children… Read more
The diagnosis of systemic sclerosis (SSc, systemic scleroderma) is usually based on the presence of a combination of symptoms and signs typical of systemic sclerosis: Raynaud’s phenomenon; skin thickening or puffy «sausage-like» swelling of the fingers; autoantibodies associated with systemic sclerosis in a blood sample; abnormalities in small blood vessels at the base of the nails; other skin and internal organ involvement associated with systemic sclerosis… Read more
Nailfold capillaroscopy is a simple, non-invasive, painless examination mainly performed on the hands that allows the study of small blood vessels, called capillaries, located around the nail beds. After depositing a drop of oil to make the skin more transparent, the periungual capillaries are observed under a microscope… Read more
The evolution of systemic sclerosis is variable, depending on the extent of skin thickening (limited or diffuse systemic sclerosis), the presence of specific autoantibodies in the blood and the presence of internal organ involvement… Read more
Information booklet for Scleroderma patients and their family members/informal caregivers.
Systemic Sclerosis (SSc) is an autoimmune disease that can affect various organs, in addition to the skin. In almost all cases, the thickening and loss of flexibility of the skin affect the fingers. Raynaud’s phenomenon is very common in SSc patients. It can sometimes lead to painful fingertip ulcers… Read more
Medication and treatments
Miscellaneous articles and other publications
Publication of Éric Rich, M.D.