Related Subjects:
|Ferritin
|CEA
|ESR
|CRP
|ALP
|LDH
|HbA1c
|Alpha Fetoprotein
|Anti-Hu ab
|Anti-Yo ab
|Anti-SCL70 ab
|Biochemical Lab values
🔎 About
- ⏳ Turnaround time for results may be several weeks.
🧬 Aetiology
- Progressive Systemic Sclerosis (PSS / systemic sclerosis) is a chronic systemic rheumatic disease affecting skin, GI tract, heart, lungs, and kidneys.
- 👩🦰 More common in females, usually onset between 20–50 years.
- Patients often have multiple autoantibodies (Scl-70, Sm, centromere, Ro, La, RNP).
- Anti-Scl-70 (Topoisomerase I) is found in ~15–20% of scleroderma cases, especially diffuse cutaneous systemic sclerosis (dcSSc, ~15–30%).
- 🔬 Difficult to detect by immunofluorescence (gives fine speckled nuclear pattern) - usually part of the ENA screen.
- 🌍 Occurs less frequently in Caucasians compared to some other ethnic groups.
- ⚠️ Presence does not correlate with disease activity but is associated with more aggressive systemic disease.
🩺 Clinical
- Seen in systemic sclerosis, particularly diffuse cutaneous disease.
- Often associated with:
- 🤲 Sclerodactyly (tight, thickened skin on hands).
- ❄️ Raynaud’s phenomenon.
- 🫁 Pulmonary fibrosis / interstitial lung disease.
- 🦴 Subcutaneous calcification.
- 🍽️ Oesophageal dysmotility.
- 🧠 Neurological symptoms (e.g., paraesthesia, brachial plexopathy, optic neuritis).
🧪 Investigations
- Anti-Scl-70 testing performed when the ENA screen is positive but Ro, La, RNP, Sm, and centromere are negative.
- Results usually reported as: ❌ Negative / ⚖️ Equivocal / ✅ Positive.
📚 References
- UpToDate: Systemic Sclerosis autoantibodies.
- BNF / NICE immunology guidance.
- 📖 Walker-Bone K. *Oxford Handbook of Rheumatology*.