Systemic sclerosis Flashcards
What is systemic sclerosis (scleroderma)?
Systemic sclerosis is an autoimmune connective tissue disease involving inflammation and fibrosis (hardening or scarring) of the skin and internal organs. The exact cause is unclear.
What are the two main patterns of systemic sclerosis?
- Limited cutaneous systemic sclerosis (previously known as CREST syndrome)
- Diffuse cutaneous systemic sclerosis
What features are included in the CREST mnemonic for limited cutaneous systemic sclerosis?
- C – Calcinosis
- R – Raynaud’s phenomenon
- E – Esophageal dysmotility
- S – Sclerodactyly
- T – Telangiectasia
What internal organs are affected in diffuse cutaneous systemic sclerosis?
Diffuse cutaneous systemic sclerosis affects internal organs such as the heart (cardiovascular problems), lungs (pulmonary hypertension and pulmonary fibrosis), and kidneys (scleroderma renal crisis).
What is sclerodactyly in systemic sclerosis?
Sclerodactyly refers to skin tightening around the joints of the hands, leading to restricted range of motion and functional loss, with the fat pads on the fingers being lost. The skin may also break and ulcerate.
What is Raynaud’s phenomenon and how is it related to systemic sclerosis?
Raynaud’s phenomenon is a condition where the fingertips change color in response to cold. In systemic sclerosis, it follows a typical color pattern: white (vasoconstriction), blue (cyanosis), and red (reperfusion).
What are the common complications of systemic sclerosis?
- Pulmonary fibrosis (dry cough, shortness of breath)
- Scleroderma renal crisis (severe hypertension and renal failure)
- Cardiovascular issues (hypertension, coronary artery disease)
- Raynaud’s phenomenon
- Gastrointestinal issues (e.g., esophageal dysmotility, acid reflux)
What is used to manage Raynaud’s phenomenon in systemic sclerosis?
- Keep hands warm (gloves, hand warmers)
- Calcium channel blockers (e.g., nifedipine)
- Specialist drugs (e.g., losartan, ACE inhibitors, sildenafil, fluoxetine)
- Avoid cold triggers
Which autoantibodies are associated with systemic sclerosis?
- Anti-centromere antibodies: Associated with limited cutaneous systemic sclerosis
- Anti-Scl-70 antibodies: Associated with diffuse cutaneous systemic sclerosis and more severe disease
- ANA (anti-nuclear antibodies): Positive in most patients, but non-specific
What is the diagnostic method for systemic sclerosis?
Diagnosis is based on the ACR/EULAR classification criteria from 2013, including clinical features, autoantibodies, and nailfold capillaroscopy.
What is the first-line treatment for Raynaud’s phenomenon in systemic sclerosis?
Nifedipine is usually the first-line treatment for Raynaud’s phenomenon.
What other medications are used in the management of systemic sclerosis?
- Proton pump inhibitors for acid reflux
- Prokinetic medications (e.g., metoclopramide) for gastrointestinal symptoms
- ACE inhibitors for hypertension and scleroderma renal crisis
- Endothelin receptor antagonists (e.g., bosentan) for pulmonary hypertension and digital ulcers
- Sildenafil for pulmonary hypertension
- Oxygen for pulmonary fibrosis
What are non-medical management strategies for systemic sclerosis?
- Avoid smoking
- Gentle skin stretching to maintain joint mobility
- Regular use of emollients
- Physiotherapy and occupational therapy
- Avoid cold triggers for Raynaud’s phenomenon
What is a potential treatment for severe, rapidly progressing systemic sclerosis?
Stem cell transplantation may be considered in rapidly progressing severe disease, but it carries significant risks.
