CTD-Systemic sclerosis Flashcards
systemic sclerosis
Excessive collagen deposition causes skin and internal organ changes.
Characteristics of systemic sclerosis (2)
vasomotor disturbances (Raynauds)
fibrosis and subsequent atrophy of the skin subcutaneous tissue.
presentation (5)
CREST
Calcinosis
Raynuads
oEsophageal distility
Sclerodactyly
Tengalesia
what are the features divided into? (2)
major and minor
organ involvement (9)
Pulmonary hypertension, pulmonary fibrosis and accelerated hypertension leading to renal crisis are important organ manifestations.
Gut involvement may lead to dysphagia, malabsorption and bacterial overgrowth of the small bowel.
Inflammatory arthritis and myositis may be seen.
classification (2)
divided into limited and diffuse forms.
investigations (5)
limited- anti-centromere antibody
diffuse- anti-Scl-70.
Organ screening
echo
monitoring of renal function.
management (5)
no one overall treatment for SSc.
Raynauds/digital ulcers: calcium channel blockers, Iloprost, bosentan
Renal involvement: ACE inhibitors
GI involvement: proton pump inhibitors for reflux
Interstitial lung disease: immunosuppression, usually with cyclophosphamide.
Diffuse systemic sclerosis (2)
skin changes develop more rapidly and can involve the trunk.
Early significant organ involvement
Limited systemic sclerosis (2)
skin involved tends to be confined to face, hands and forearms and feet.
Organ involvement tends to occur later
Limited SS antibody
Anti-centromere antibody association
Diffuse SS antibody
Anti-Scl-70 antibody association
Major
Major: includes centrally located skin sclerosis that affects the arms, face, and/or neck
Minor
Minor: includes sclerodactyly and atrophy of the fingertips and bilateral lung fibrosis.
