Scleroderma Flashcards
Typical presentation
Woman, presenting with Raynaud’s syndrome
Pathophysiology
Autoimmune systemic condition
Increased fibroblast activity
Abnormal connective tissue growth
Vascular damage & fibrosis
Subtypes
Limited SSC
Diffuse SSC
Diffuse SSC features
Less common
Increased mortality
Sudden onset skin changes
Proximal joint involvement. (Knees, Elbows)
HTN -> Renal Failure
Limited SSC Features
More common
Calcinosis Cutis
Raynaud’s Syndrome
Esophageal dysmotility
Sclerodactyly
Telangiectasia
Pulmonary HTN
Investigations Limited SSC
ANA Positive
Anti Centromere Antibody
Investigations diffuse SSC
Topoisomerase antibody
Anti RNA Polymerase III Antibody
General investigations
CXR - Pulmonary disease
Joint XR - Calcinosis Cutis
Echo/ ECG - Arrhythmia, HF, Myocarditis
CRP/ESR/PV - Normal
Treatment
NO CURE
- Psychosocial
- PDE 5 (Sildenafil)
- Ace inhibitors (Blood pressure, Renal protection)
- Methotrexate, Mycophenolate Mofetil (Skin Thickening)
- Prednisolone (Acute flares)