Related Subjects:
|Ferritin
|CEA
|ESR
|CRP
|ALP
|LDH
|HbA1c
|Alpha Fetoprotein
|Anti-Hu ab
|Anti-Yo ab
|Anti-SCL70 ab
|Biochemical Lab values
๐ About
- โณ Turnaround time for results may be several weeks.
๐งฌ Aetiology
- Progressive Systemic Sclerosis (PSS / systemic sclerosis) is a chronic systemic rheumatic disease affecting skin, GI tract, heart, lungs, and kidneys.
- ๐ฉโ๐ฆฐ More common in females, usually onset between 20โ50 years.
- Patients often have multiple autoantibodies (Scl-70, Sm, centromere, Ro, La, RNP).
- Anti-Scl-70 (Topoisomerase I) is found in ~15โ20% of scleroderma cases, especially diffuse cutaneous systemic sclerosis (dcSSc, ~15โ30%).
- ๐ฌ Difficult to detect by immunofluorescence (gives fine speckled nuclear pattern) โ usually part of the ENA screen.
- ๐ Occurs less frequently in Caucasians compared to some other ethnic groups.
- โ ๏ธ Presence does not correlate with disease activity but is associated with more aggressive systemic disease.
๐ฉบ Clinical
- Seen in systemic sclerosis, particularly diffuse cutaneous disease.
- Often associated with:
- ๐คฒ Sclerodactyly (tight, thickened skin on hands).
- โ๏ธ Raynaudโs phenomenon.
- ๐ซ Pulmonary fibrosis / interstitial lung disease.
- ๐ฆด Subcutaneous calcification.
- ๐ฝ๏ธ Oesophageal dysmotility.
- ๐ง Neurological symptoms (e.g., paraesthesia, brachial plexopathy, optic neuritis).
๐งช 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: โ Negative / โ๏ธ Equivocal / โ
Positive.
๐ References
- UpToDate: Systemic Sclerosis autoantibodies.
- BNF / NICE immunology guidance.
- ๐ Walker-Bone K. *Oxford Handbook of Rheumatology*.