Rheumatology Book

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Systemic SclerosisAka: Scleroderma

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  1. Pathophysiology
    1. Idiopathic collagen disease
    2. Progressive muscle atrophy and fibrosis
    3. Esophagus changes
      1. Loss of peristalsis
      2. Sphincter reduction
  2. Symptoms
    1. Gradual symptom onset
    2. Anorexia
    3. Dyspnea
    4. Dysphagia
    5. Reduced sweating
    6. Arthralgias
  3. Signs
    1. Low grade fever
    2. Skin contracted with immobility and symmetric deformity
    3. Muscular weakness
    4. Limited chest expansion
    5. Limited jaw opening
  4. Complications
    1. Esophageal Dysmotility
      1. Substantial Gastroesophageal Reflux Disease
      2. Barrett's Esophagus
  5. Management: Esophageal Dysmotility
    1. Chew food well and drink adequate liquids with food
    2. Gastric acid reduction
      1. H2 Blocker (e.g. Ranitidine)
      2. Proton Pump Inhibitor (e.g. Prilosec)

Systemic Scleroderma (C0036421)

Definition (MSH)A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.
Definition (CSP)systemic disorder of the connective tissue; manifested by hardening and thickening of the skin, by abnormalities involving the microvasculature and larger vessels, and by fibrotic degenerative changes in various body organs including the heart, lungs, kidneys, and gastrointestinal tract.
ConceptsDisease or Syndrome (T047)
ICD9710.1
EnglishProgressive systemic sclerosis, PSS, Scleroderma, Scleroderma syndrome, Systemic Scleroderma, Systemic sclerosis
Spanishesclerodermia, esclerosis sistémica, esclerosis sistémica progresiva, esclerosis sistemica, esclerosis sistemica progresiva, síndrome de esclerodermia, sindrome de esclerodermia
CreditsDerived from the NIH UMLS (Unified Medical Language System)



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