Systemic sclerosis Flashcards
What is systemic sclerosis?
autoimmune inflammatory and fibrotic connective tissue disease
Scleroderma?
Is the localised hardening of the skin and only affects the skin
What are the two patterns of disease in systemic sclerosis?
Limited cutaneous SS
Diffuse cutaneous SS
What are the features of limited cutaneous?
Skin changes limited up to just the elbow Calcinosis Raynauds oEsophageal dysmotility Sclerodactyly Telengiectasia
What are the features of diffuse cutaneous SS?
"Malignant scleroderma" CREST + Microstomia Tethering of skin renal failure secondary to severe HTN Pulmonary fibrosis - restrictive ventilatory defect + pulmonary HTN Oesophageal atony --> reflux & aspiration Cardiovascular --> HTN + CAD
3 key questions in the history?
- Do you hands change colour in the cold - white, blue or red?
- Do you get breathless?
- Do you get indigestion or heartburn?
Features of clinical presentation in systemic sclerosis?
Scleroderma - tight waxy skin without normal folds Sclerodactyly - reduces ROM Microstomia Beaking - tethering of skin around nose Telengiectasia ---> small dilated blood vessels in the skin Raynaud's Oesophageal dysmotility Systemic & pulmonary HTN Pulmonary fibrosis Renal failure + HTN --> renal crisis
Telengiectasia - on face,
What to look for in examination?
Scleroderma & dactyly Palpate for temp Skin tethering Calcifications on extensor aspects of hands Check hand function - prayer sign Three finger test for microstomia
Investigations
ANA
Anti-centromere for Limited
Anti-Scl70 for diffuse
Nail fold capillaroscopy
Diagnosis
Based on ACR and ELAR classification criteria –> clinical aspects, antibodies & nailfold capillaroscopy
Management
No formal proven tx for SS
Steroids & immunosuppressants mainstay
Conservative management
Physiotherapy to maintain ROM
Regular stretching to maintain ROM
Emollients for scleroderma
Avoiding cold triggers for raynauds
Medical management
of symptoms Nifedipine for Raynauds Omeprazole for indigestion Metoclopramide for GI --> pro-motility Analgesia for joint pain Antihypertensives for HTN Pulmonary artery HTN - sildenafil or bosentan Supportive management for pulmonary fibrosis
