Pubmed eg: [ 1, 2,] - hallmarks of Systemic Sclerosis (SSc):
- triad of
- obliterative small-vessel vasculopathy in multiple vascular beds (ultimately loss of microvasculature)
- Raynauds
- cutaneous telangiectasia
- nail fold capillary alterations
- pulmonary arterial hypertension
- gastric antral vascular ectasia
- scleroderma renal crisis with malignant hypertension
- inflammation
- autoimmunity
- leading to: progressive interstitial and perivascular fibrosis
- skin
- lungs
- GIT
- heart
- tendon
- ligaments
- endocrine glands
- perivascular
- Types
- diffuse cutaneous SSc
- rapidly progressive fibrosis of skin, lungs, other organs
- mortality: 55% in 10yrs [2007]
- AutoAb in diffuse disease: U3RNP=U3 ribonuclear protein, Topo=Topoisomerase, Pol-III=polymerase III
- limited cutaneous SSc
- usu. vascular manifestations, only slow and limited fibrosis of skin and internal organs
- AutoAb in limited disease: ACA=anticentromere Ab, Th/To=....?, U1RNP=U1 ribonuclear protein
- positive FHx = strongest risk factor (familial: 1.6%, sporadic: 0.025%)
- Etiology unknown; ?triggers: eg CMV, solvents, silica, ...
- Autoantibodies [2]
- AutoAb in diffuse disease: U3RNP=U3 ribonuclear protein, Topo=Topoisomerase, Pol-III=polymerase III
- AutoAb in limited disease: ACA=anticentromere Ab, Th/To=....?, U1RNP=U1 ribonuclear protein
- Therapy: ...
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