Diffuse systemic sclerosis

external

• Synonyms

  • Scleroderma
  • dcSSc

• Causes

  • Anti-DNA topoisomerase I antibody25.0%
  • Antinuclear antibody
  • Anticentromere antibody
  • Mixed connective tissue disease

• Effects

  • CREST syndrome
  • Microstomia
  • Beaked nose
  • Atrophy of finger pulp
  • Intestinal pseudoobstruction
  • Basal lung fibrosis
  • Secondary amyloidosis
  • Antiphospholipid syndrome
  • Diffuse intestinal disease
  • Bowel bacterial overgrowth
  • Primary biliary cirrhosis
  • Pseudodiverticuli
  • Allen test positive
  • Morphoea
  • Pseudorhagades
  • Nail-fold vasculitis
  • Membranoproliferative glomerulonephritis
  • Raynaud phenomenon
  • Gastric antral vascular ectasia
  • Scleroderma renal crisis

• Parents

  • Collagen vascular disease

• Children

  • Thibierge-Weissenbach syndrome

• Treatments

  • Cyclophosphamide
  • Methotrexate
  • Antithymocyte immunoglobulin
  • Penicillamine
  • Interferon alpha

• Description

  • "90% have Raynaud phenomenon 50% have bowel involvement"