Scleroderma Flashcards
1
Q
What is scleroderma?
A
Multisystem autoimmune disease Characterised by -functional and structural abnormalities of small blood vessels -fibrosis of skin and internal organs -production of autoantibodies
2
Q
What are the types of scleroderma?
A
Limited- better prognosis, less organ involvement
Diffuse
3
Q
What are the risk factors for scleroderma?
A
FH
Immune dysregulation
Female
4
Q
What is the presentation of limited scleroderma?
A
CREST Calcinosis Raynaud's Oesophageal dysmotility Sclerodactyly Talangiectasia
5
Q
What is the presentation of diffuse scleroderma?
A
CREST Digital pos and ulcers, skin thickening Bloating Myalgia, arthralgia, fatigue Dyspnoea, dry cough
6
Q
What investigations are done for scleroderma?
A
Autoantibodies -anti-scl 70= diffuse -ANA -anticentromere= limited WCC, urea and creatinine CXR and pulmonary function tests
7
Q
What is the management of scleroderma?
A
Symptomatic relief- e.g. analgesia, akin emollients, CCBs for Raynaud’s
Immunosuppression esp cyclophosphamide
8
Q
What are the complications of scleroderma?
A
Renal crisis
Pulmonary fibrosis and hypertension
Bacterial overgrowth of the gut
