What is the hallmark of systemic sclerosis?
Scleroderma is the hallmark feature of systemic sclerosis (SSc). SSc is a chronic multisystem disease characterized by widespread vascular dysfunction and progressive fibrosis of the skin and internal organs.
What triggers systemic sclerosis?
Doctors don’t know exactly what causes this process to begin, but the body’s immune system appears to play a role. Most likely, scleroderma is caused by a combination of factors, including immune system problems, genetics and environmental triggers.
What is the commonly the first manifestation of progressive systemic sclerosis?
Raynaud’s phenomenon is usually the first manifestation in systemic sclerosis9 and occurs in more than 95% of PSS patients.
What mimics systemic sclerosis?
Results: Diseases that can mimic the cutaneous symptoms of SSc include morphea, scleroderma, diabetic cheirarthritis, scleromyxedema, nephrogenic systemic fibrosis and eosinophilic fasciitis.
How do you test for systemic sclerosis?
Systemic sclerosis is diagnosed based on the presence of various symptoms above and physical examination findings. The diagnosis can be confirmed by the presence of certain autoantibodies in the blood as well as radiographic studies. Particularly, the ANA, or the antinuclear antibody test, is positive, but not always.
What is the life expectancy of someone with systemic sclerosis?
Patients with limited cutaneous systemic sclerosis have a 10-year survival estimate of approximately 90%. However, development of interstitial lung disease (ILD) or pulmonary artery hypertension (PAH) dramatically increases the risk of death.
How fast does systemic scleroderma progress?
In general, without treatment, the skin thickening will reach its peak within 1-2 years after it starts and will then begin to loosen.
Which organ is most frequently affected in systemic sclerosis?
PAH and ILD are progressive conditions and can eventually become life-threatening. Taken together, they account for about half of all deaths related to systemic scleroderma. The most commonly involved part of the gastrointestinal tract in systemic scleroderma is the esophagus.
What can be misdiagnosed as scleroderma?
Diagnosis and Tests
Because it can affect other parts of the body — such as the joints — scleroderma may be initially mistaken for rheumatoid arthritis or lupus. After discussing your personal family medical history, your doctor will perform a thorough physical examination.
What are the markers for scleroderma?
Typically, the rheumatologist will order an ANA (antinuclear antibody, an antibody commonly found in many of the autoimmune diseases), as well as an Scl-70 antibody (positive in some patients with diffuse scleroderma), anticentromere antibody (generally felt to be a marker for limited scleroderma), and an antibody …
How do you rule out systemic sclerosis?
Where does scleroderma usually start?
Limited cutaneous scleroderma comes on gradually and usually affects skin on your fingers, hands, face, lower arms, and legs below the knees. It can also cause problems with your blood vessels and esophagus. The limited form has internal organ involvement, but it is generally milder than in the diffuse form.
How fast does systemic sclerosis progress?
In our cohort, 56% of observed progression occurred within 5 years since the first non-Raynaud symptom. While progression in skin involvement occurred more frequently in early disease, the proportion of patients with lung, heart or GI progression was relatively stable over time.
Is systemic sclerosis serious?
Systemic sclerosis (SSc, scleroderma) is the most serious form of the disease. This form of scleroderma can occur at any age but is most common in women of childbearing age.
What are the last stages of scleroderma?
This type of scleroderma is typically accompanied by shortness of breath, a persistent cough, and the inability to perform routine physical activities. End-stage scleroderma often causes pulmonary fibrosis and/or pulmonary hypertension, both of which can be life-threatening.
What test confirms scleroderma?
Antinuclear antibody (ANA) testing is the most important blood test to screen for scleroderma and other connective tissue diseases.
Can a blood test detect scleroderma?
A blood test alone cannot diagnose scleroderma. Depending on the clinical situation, additional tests may be done, such as: Pulmonary function tests or breathing tests to measure how well the lungs are working. CT chest scan may also be ordered to evaluate the extent of lung involvement.
Can you have mild systemic sclerosis?
Most people with systemic sclerosis will only have mild symptoms. Rarely, it can cause serious problems with internal organs. These complications can be treated. It’s important that potentially serious complications are spotted early.
How quickly does systemic sclerosis progress?
What is the difference between scleroderma and systemic sclerosis?
What’s the difference between scleroderma and systemic sclerosis? Scleroderma is seen only on the skin. However, when the fibrosis involves your other internal organs, this is considered systemic sclerosis.
Can you live a normal life with systemic sclerosis?
Some patients with systemic sclerosis never develop severe involvement of their internal organs and live relatively normal lives. The skin involvement tends to get better on its own over years. The skin will soften and become more flexible.
What is the most serious complication of scleroderma?
Kidney Symptoms and Complications
The typical course of kidney involvement in scleroderma is a slow progression that may produce some damage but does not usually lead to kidney failure. The most serious kidney complication in scleroderma is renal crisis.
What virus causes scleroderma?
One theory suggests that scleroderma is caused by a virus or other infection. For example, a common virus called CMV (cytomegalovirus) is known to infect cells (endothelial cells) that line our blood vessels, which we know are diseased in scleroderma (3).
Does scleroderma affect the eyes?
In some cases, scleroderma can lead to eye problems, including the following: Dry eyes According to research in the journal Archives of Medical Science, the most common eye complication in people with Scleroderma is dry eyes. Dry eyes occur when you cannot produce enough film of tears to keep the eyes lubricated.
Can you live a full life with systemic sclerosis?
Every patient with systemic sclerosis is different, which makes predicting additional complications and life span nearly impossible. Some patients with systemic sclerosis never develop severe involvement of their internal organs and live relatively normal lives.