Systemic Sclerosis

Systemic Sclerosis

Article
Bone, Muscle, & Joint
Dental Health
+8
Contributed byLester Fahrner, MD+1 moreJul 26, 2022

The topic Systemic Sclerosis you are seeking is a synonym, or alternative name, or is closely related to the medical condition Scleroderma.

Quick Summary:

  • Scleroderma is a chronic connective tissue disorder of unknown cause that most commonly affects young and middle-aged women. The term Scleroderma indicates “hard skin”; “sclera” means hard, and “derma” means skin. The condition is also termed Systemic Sclerosis
  • Connective tissues provide structural support to the organs and skin. Individuals with Scleroderma have thick or hard connective tissue. The signs and symptoms are variable and depend upon the Scleroderma type. Systemic Sclerosis manifests in two forms - limited and diffuse
  • The most typical skin findings seen in both limited and diffuse forms, include firm bound down skin of the fingers, Raynaud’s phenomenon of blanching of fingers in response to cold exposure, and tighter, wrinkle-free skin of the face
  • The complications of diffuse Systemic Sclerosis include the hardening of organs due to abnormal fibrosis of the tissue that can severely affect the organ function of the heart, lungs, and kidneys. Limited Systemic Sclerosis, previously called CREST syndrome, besides the skin findings, involves the esophagus, lungs, and intestines
  • There is currently no cure for Scleroderma. But medications, therapy, and surgery may be used to control the symptoms and prevent complications. The treatment measures are dependent upon the tissues or organs affected
  • The way Scleroderma changes and progresses over time vary among individuals. The rapidity of progression of signs and symptoms and the organs involved affects the prognosis. Typically, individuals who respond to treatment have a better prognosis than individuals who do not respond well to treatment

There is currently a debate about how Scleroderma should be classified. A majority believe that it should be classified as an autoimmune rheumatic disorder. Due to this, many researchers believe that the damage is caused by immune cells mistakenly attacking one’s own healthy tissue.

Scleroderma can be classified into two broad categories, which include Localized Scleroderma and Systemic Scleroderma:

  • Localized Scleroderma:
    • It only affects the skin structures  and does not cause harm to the other major organs
    • There are two types of Localized Scleroderma, namely Morphea and Localized Linear Scleroderma
  • Systemic Scleroderma:
    • It affects the skin, the tissues beneath the skin, major organs, and blood vessels
    • Systemic Scleroderma also has two different types, namely Limited Systemic Scleroderma and Diffuse Systemic Scleroderma

Please find comprehensive information on Scleroderma regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Reviewed by

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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