Scleroderma classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]
Overview
There is no established system for the classification of [disease name].
OR
[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
OR
[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
OR
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
OR
If the staging system involves specific and characteristic findings and features: According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
OR
The staging of [malignancy name] is based on the [staging system].
OR
There is no established system for the staging of [malignancy name].
Classification
- Scleroderma was previously classified according to American College of Rheumatology (ACR) 1980 preliminary scleroderma criteria:[1]
- Major criteria:
- Proximal cutaneous sclerosis (skin thickening-nonpitting) of fingers that also extended proximally to the metacarpophalangeal joints (MCPs)
- Minor criteria:
- Sclerodactyly
- Digital pitting scars of fingertips or loss of substance of the distal finger pad (digital tuft resorption)
- Bibasilar pulmonary fibrosis
- Major criteria:
- If major criteria was present that was sufficient otherwise 2 of 3 minor criteria had to be present.
- Scleroderma (systemic sclerosis) is now classified according to the new American College of Rheumatology (ACR)/ European League Against Rheumatism (EULAR):[2]
| Item | Sub-item(s) | Weight/Score |
|---|---|---|
| Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (sufficient criterion) | - | 9 |
| Skin thickening of the fingers (only count higher score) | Puffy fingers
Sclerodactyly of the fingers (distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints) |
2
4 |
| Fingertip lesions (only count the higher score) | Digital tip ulcers
Fingertip pitting scars |
2
3 |
| Telangiectasia | - | 2 |
| Abnormal nail fold capillaries | - | 2 |
| Pulmonary arterial hypertension and/or Interstitial lung disease | 2 | |
| Raynaud's phenomenon (RP) | - | 3 |
| Any of the Scleroderma related autoantibodies | Anticentromere
Anti-topoisomerase I (anti-SCL-70) Anti-RNA polymerase-3 |
3 |
- The total score is determined by adding the only the highest weight/score in each category
- Patients with a total score ≥ 9 are classified as having scleroderma
References
- ↑ "Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee". Arthritis Rheum. 23 (5): 581–90. May 1980. PMID 7378088.
- ↑ van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE (November 2013). "2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative". Ann. Rheum. Dis. 72 (11): 1747–55. doi:10.1136/annrheumdis-2013-204424. PMID 24092682.