Systemic Sclerosis Flashcards
What is responsible for death in systemic sclerosis ?
Renal and lung changes
Pulmonary hypertension
Describe the common early findings of systemic sclerosis?
Raynauds phenomenon
What are the three phases of cutaneous involvement in systemic sclerosis?
1) Oedematous
2) Indurative
3) Atrophic
How are cutaneous features divided in systemic sclerosis?
Major = centrally located skin sclerosis that affects arms, face and/or neck
Minor = includes sclerodactyly and atrophy of fingertips and bilateral lung fibrosis
When is a diagnosis of systemic sclerosis made?
When patient has 1 major and 2 minor features
What are common facial features of systemic sclerosis ?
Beaking of nose
Tightening of skin around mouth
Telangectasia
What can be seen in the digits in systemic sclerosis?
Calcinosis (subcutaneous deposits of calcium)
What are the important organ manifestations of systemic sclerosis?
Pulmonary hypertension
Pulmonary fibrosis
Accelerated hypertension
Renal crisis
Describe gut involvement in systemic sclerosis?
Lead to dysphagia, malabsorption and SIBO
What MSK manifestation of systemic sclerosis are there?
inflammatory arthritis
Myositis
Describe limited systemic sclerosis?
Skin involved tends to be confined to face, hands and forearms and feet. Organ involvement occurs later. Anti-centromere antibody.
Describe diffuse systemic sclerosis?
Skin changes develop more rapidly and can involve the trunk. Early significant organ involvement. Anti-Scl-70 antibody
How is systemic sclerosis investigated?
Anti-centromere antibody
Anti-Scl-70
Organ screening
- pulmonary function testing
- echo
- monitoring of renal function
How is raynauds/digital ulcers managed?
Calcium channel blockers, iloprost, bosentan
How is renal involvement managed?
ACE inhibitors
