Do not apply them when skin thickening spares the fingers or another disorder explains the findings better. Mimics include generalized morphea, eosinophilic fasciitis, nephrogenic systemic fibrosis, scleredema, scleromyxedema, porphyria, lichen sclerosus, erythromelalgia, graft-versus-host disease and diabetic cheiroarthropathy.
Systemic sclerosis
Scleroderma assessment.
Systemic sclerosis can affect the skin, circulation and internal organs. These criteria concern systemic sclerosis, not localized scleroderma (morphea).
2013 ACR/EULAR classification criteria. Complete this with your clinician using confirmed findings. These are classification criteria, not a self-diagnosis tool.
When to seek care: Sudden breathlessness, chest pain, a severe headache with high blood pressure, reduced urine or a persistently blue/black painful finger requires urgent care.
These criteria use examination findings and specific test definitions. Do not infer them from symptoms alone; use not sure when a finding has not been assessed.
A clinician must confirm that thickening extends proximal to the metacarpophalangeal (MCP) joints. This alone supplies 9 points; do not add the other categories to it.
Choose the highest finding only. Sclerodactyly means thickening distal to the MCP joints but extending proximal to the finger middle (PIP) joints.
Only ischemic lesions count, not injuries. Ulcers and pitting scars are alternatives; use the highest score.
A clinician must identify the typical systemic sclerosis pattern of dilated small surface vessels.
Enlarged capillaries and/or loss of capillaries in a pattern consistent with systemic sclerosis.
Pulmonary arterial hypertension requires right-heart catheterization. Interstitial lung disease requires qualifying imaging or characteristic basal crackles without another cause. Both together still score 2.
Cold or emotion-related finger/toe colour changes with at least two phases involving pallor, blue colour or redness.
Anti-centromere (or centromere ANA pattern), anti-topoisomerase I/Scl-70 or anti-RNA polymerase III. More than one positive antibody does not add extra points.
Symptoms to discuss
- Cold-triggered finger colour changes, puffy fingers, tight skin or fingertip sores.
- Reflux, swallowing problems, breathlessness or reduced exercise tolerance may need investigation.
Tests and care
- Skin and nailfold examination plus targeted antibody tests.
- Lung-function tests, chest imaging, heart assessment, blood pressure and kidney checks as indicated.
Treatment depends on the affected organs. Protecting fingers from cold, skin care and regular organ monitoring are useful alongside specialist-directed medicines.
Classification is not diagnosis. A low score does not rule out disease. Do not arrange every listed test or start, stop or change medicines based on this result; your clinician decides what is needed.
Clinical references: 2013 ACR/EULAR criteria and definitions · ACR patient guide: scleroderma