Systemic Sclerosis or Scleroderma
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Questions and Answers

What is the primary mechanism of endothelial cell injury in scleroderma?

  • Cytokine-mediated
  • Environmental toxin-mediated
  • Autoantibody-mediated (correct)
  • Oxidative stress-mediated

    • Which of the following is NOT a common trigger for scleroderma?

  • Epoxy resin exposure
  • Radiation exposure
  • Vinyl chloride exposure
  • Infection with Streptococcus pneumoniae (correct)

    • What is the characteristic skin finding in scleroderma?

  • Thickening and fibrosis of the skin (correct)
  • Erythema nodosum
  • Purpura
  • Maculopapular rash

    • Which of the following is a type of localized scleroderma?

    <p>Morphea</p> Signup and view all the answers

    What is the significance of anti-topoisomerase-1 (anti-Scl-70) antibodies in scleroderma?

    <p>Serologic marker for autoimmune response</p> Signup and view all the answers

    What is the primary immune cell involved in scleroderma pathogenesis?

    <p>CD4+ T lymphocyte</p> Signup and view all the answers

    What is the most common age group affected by scleroderma?

    <p>40-50 years</p> Signup and view all the answers

    Which of the following is a rare but possible complication of scleroderma?

    <p>Pulmonary hypertension</p> Signup and view all the answers

    What is the diagnostic criterion for systemic sclerosis as per the American College of Rheumatology?

    <p>One major criterion and two or more minor criteria</p> Signup and view all the answers

    What is the characteristic skin change in proximal scleroderma?

    <p>Symmetrical thickening, tightening, and induration of the skin</p> Signup and view all the answers

    What is the radiological feature of bibasilar pulmonary fibrosis?

    <p>Bilateral reticular pattern of linear or lineonodular densities</p> Signup and view all the answers

    What is the term for thickening, induration, and tightening of the skin limited only to fingers?

    <p>Sclerodactyly</p> Signup and view all the answers

    What is the diagnostic test required for the diagnosis of scleredema, scleromyxedema, and nephrogenic systemic fibrosis?

    <p>Full thickness biopsy</p> Signup and view all the answers

    What is the sensitivity and specificity of the ACR classification criteria for systemic sclerosis?

    <p>97% sensitive and 98% specific</p> Signup and view all the answers

    What is the primary mechanism of skin and subcutaneous tissue hardening in localized scleroderma?

    <p>Excessive collagen deposition</p> Signup and view all the answers

    Which of the following autoimmune diseases is commonly associated with morphea?

    <p>Systemic lupus erythematosus</p> Signup and view all the answers

    What is the most common presentation of plaque morphea?

    <p>One or a few oval, rounded areas of induration on the trunk or proximal extremities</p> Signup and view all the answers

    What is the primary complication of linear morphoea?

    <p>Contractures and deformities</p> Signup and view all the answers

    What is the treatment of choice for localized scleroderma?

    <p>Topical corticosteroids</p> Signup and view all the answers

    Which type of scleroderma is characterized by visceral involvement without skin involvement?

    <p>Sine scleroderma</p> Signup and view all the answers

    What is the aetiology of en coup de sabre?

    <p>Unknown</p> Signup and view all the answers

    What is the primary site of involvement in en coup de sabre?

    <p>Scalp and face</p> Signup and view all the answers

    Study Notes

    Systemic Sclerosis (Scleroderma)

    • A generalized disorder of connective tissue characterized by thickening and fibrosis of the skin (scleroderma) and distinctive forms of involvement of internal organs, notably the heart, lungs, kidneys, and gastrointestinal tract.

    Definition

    • A multisystemic, autoimmune disease affecting small arteries, microvessels, and fibroblasts, resulting in vascular obliteration, collagen accumulation, and scarring (fibrosis) of skin and internal organs.
    • Serologic specificity of the disease is the presence of ANA, directed against cellular nuclear enzymes, like DNA topoisomerase-1 (anti-Topo 1) and RNA polymerase, as well as centromeric proteins (anticentromere Ab).

    Epidemiology

    • Scleroderma affects all races, but it is more destructive in blacks than in Caucasians.
    • The incidence is higher among people in their 40's, and females are affected more than males.

    Etiology

    • Environmental factors that trigger or accelerate the development of SSc include:
      • Silica exposure
      • Vinyl chloride
      • Trichloroethylene
      • Epoxy resins
      • Benzene
      • CCl4
      • Radiation exposure/radiotherapy
      • Infection - CMV, HHV 5, PBV B19
      • Drugs - bleomycin, pentazocine, l-tryptophan
      • Common in coal and gold miners

    Pathology

    • Pathogenic mechanisms always present:
      • Endothelial cell injury
      • Fibroblast activation
      • Cellular and humoral immunologic derangement
      • Activation of the immune system is an outstanding disease feature

    Types of Scleroderma

      1. Localized (majority of Paediatric cases):
      • A. Morphea:
        • Single patch
        • Multiple patches
      • B. Linear:
        • Face, forehead, and scalp (en coup de sabre)
        • Limb (en bande)
      • C. Generalized morphoea
      1. Diffuse (systemic sclerosis)
      1. Limited scleroderma (CREST syndrome)
      1. Sine Scleroderma (visceral involvement without skin involvement)
      1. Overlap Syndromes

    Linear Morphoea

    • Linear morphoea is usually solitary and unilateral in distribution.
    • Affects the underlying skeletal muscle and even bone, giving rise to contractures and deformities.

    Localized Scleroderma/Morphea

    • Morphea involves isolated patches of hardened skin - there is generally no internal organ involvement.
    • Aetiology: ?Autoimmune, higher frequencies of anti-histone and anti-topoisomerase IIa antibodies.

    Clinical Features

    • Focal thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition.

    Treatment

    • Topical, intra-lesional, and systemic corticosteroids
    • Antimalarials (hydroxychloroquine or chloroquine)
    • Methotrexate
    • Topical tacrolimus
    • Penicillamine
    • Phototherapy

    Diagnostic Criteria

    • The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis:
      • One major criterion, two or more minor criteria for diagnosis.
      • Major criterion: Proximal scleroderma - characterized by symmetrical thickening, tightening, and induration of the skin of the fingers and the skin proximal to the MCP /MTP joints.
      • Minor criteria:
          1. Sclerodactyly - thickening, induration, tightening of the skin limited only to fingers.
          1. Digital pitting scars or loss of substance from the finger pads - due to ischemia.
          1. Bibasilar pulmonary fibrosis - b/l reticular pattern of linear or lineonodular densities in basilar portions of the lung on CXR.

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    Description

    A quiz about Systemic Sclerosis or Scleroderma, a multisystemic autoimmune disease affecting small arteries, microvessels, and fibroblasts.

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