Systemic sclerosis
(Diseases)
On this page, you will find everything you need to know about systemic sclerosis.
What is systemic sclerosis?
Systemic sclerosis, also known as systemic scleroderma, is a rare connective tissue disorder of unknown cause with a complex pathophysiology. It typically presents with systemic symptoms involving internal organs. Its manifestations often overlap with those of other rheumatologic or autoimmune diseases. The severity of symptoms may vary depending on the timing of diagnosis.
- What are the types of systemic sclerosis?CREST)”.
- Systemic sclerosis can be classified into two main types:
Limited cutaneous systemic sclerosis (previously referred to as CREST syndrome).
Diffuse cutaneous systemic sclerosis classified based on clinical examination and laboratory findings.
- What are the symptoms of systemic sclerosis?
- Symptoms may include:
- Skin thickening on the fingers, hands, face, forearms, and legs
- Constipation
- Fatigue
- Shortness of breath
- Joint pain and swelling
- Small red spots on the face and chest (telangiectasia)
- Difficulty swallowing
- Muscle weakness
- Dry eyes or mouth (Sjgren’s syndrome)
- Unexplained weight loss
Heartburn
Diarrhea
- What causes systemic sclerosis?
- The exact cause of systemic sclerosis is unknown, but several factors may contribute to its development:
- Although systemic sclerosis is not considered a hereditary disease that is passed from parents to children like some genetic disorders, genes can increase the likelihood of some individuals developing systemic sclerosis and may also play a role in determining the type of the disease
Environmental exposures, such as certain chemicals and silica dust, may play a role.
Immune system dysregulation can trigger cellular changes leading to excessive collagen production.
- How is systemic sclerosis diagnosed?
- Diagnosis involves taking a thorough medical history and conducting a physical examination. Additional tests may include:
- Skin examination to identify areas of thickening or hardening
- Nailfold capillaroscopy to assess swollen microvessels under the nails
- Ultrasound imaging to monitor disease progression
- Blood tests for autoantibodies
- GastrointestinalEKGevaluations
- Pulmonary function tests, such as diffusion capacity testing
Cardiac evaluations, including ECG or right heart catheterization
Skin biopsy for microscopic examination
- How is systemic sclerosis treated?
- There is no definitive cure for systemic sclerosis, as treatment focuses on managing specific symptoms and the severity of the disease. Treatment may involve multiple approaches or be tailored as needed, and may include the following:
- Physical therapy to relieve pain, improve muscle strength and mobility, and enhance daily functioning.
- Phototherapy
- Stem cell transplantation
- Organ transplantation, when necessary
- Topical creams and moisturizers to prevent dryness and relieve itching
- Immunosuppressive medications to limit The immune system attacks on tissues
- Anti-inflammatory drugs, such as ibuprofen, can be used to reduce inflammation and pain
Vasodilators to improve blood flow
Antacids and reflux medications to manage digestive symptoms
Can systemic sclerosis be prevented?
There is no definitive way to prevent systemic sclerosis due to its complex autoimmune nature. However, some measures may help reduce symptom severity, such as avoiding smoking, using sunscreen when outdoors, and maintaining proper skin hydration.
- What are the complications of systemic sclerosis?
- Systemic sclerosis may affect various organs, leading to a range of complications depending on disease type (limited or diffuse) and extent of involvement:
- Severe skin tightening, resulting in decreased mobility, especially in fingers and joints
- Digital ulcers and wounds
- Pulmonary complications such as shortness of breath and chest pain
- Kidney complications like sudden high blood pressure thatmay cause kidney failure
- Malnutrition due to swallowing difficulties andother digestive issues.
Anxiety and depression
Increased risk of certain cancers, such as lung, esophageal, and lymphoma
When should you see a doctor?
- Consult a healthcare provider if you notice symptoms of systemic sclerosis or develop new complications. Early evaluation may help prevent disease progression or internal organ involvement.
- Frequently Asked Questions
- Who is most at risk for systemic sclerosis?
- Hormonal or immune system differences between men and women may play a role. Adult women between the ages of 30 and 50 are at greater risk than men.
