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
Scleroderma (systemic sclerosis) is classified into 2 subtypes, limited cutaneous scleroderma and diffuse cutaneous scleroderma. Morphea and CREST syndrome are variants of limited cutaneous scleroderma. Scleroderma was previously classified according to American College of Rheumatology (ACR) 1980 preliminary scleroderma criteria. Scleroderma (systemic sclerosis) is now classified according to the new American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) criteria.
Classification
Scleroderma (systemic sclerosis) is classified into 2 main subtypes:[1][2][3]
- Limited cutaneous scleroderma:
- Fibrosis is restricted to hands, arms and face.
- Raynaud's phenomenon is present for many years before the onset of fibrosis.
- Pulmonary hypertension often occurs.
- Anti-centromere antibodies are present in more than half of the patients.
- Anti-centromere antibodies are associated with CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) syndrome.[4][5]
- Morphea is a type of localized scleroderma limited to the skin and subcutaneous tissues.[6]
- Patients with internal organ involvement with out the skin manifestations are known to have systemic sclerosis sine scleroderma.
- Diffuse cutaneous scleroderma:
American College of Rheumatology (ACR) 1980 preliminary scleroderma criteria
Scleroderma was previously classified according to American College of Rheumatology (ACR) 1980 preliminary scleroderma criteria:[7]
- 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:
- Scleroderma is classified if either one major criteria or 2 of 3 minor criteria are present.
American College of Rheumatology (ACR)/ European League Against Rheumatism (EULAR) criteria
Scleroderma (systemic sclerosis) is now classified according to the new American College of Rheumatology (ACR)/ European League Against Rheumatism (EULAR) criteria:[8][9]
| 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 | Anti-centromere antibodies
Anti-topoisomerase I (anti-SCL-70) Anti-RNA polymerase-3 |
3 |
- The total score is determined by adding only the highest weight/score in each category.
- Patients with a total score ≥ 9 are classified as having scleroderma.
References
- ↑ LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F (February 1988). "Scleroderma (systemic sclerosis): classification, subsets and pathogenesis". J. Rheumatol. 15 (2): 202–5. PMID 3361530.
- ↑ Careta MF, Romiti R (2015). "Localized scleroderma: clinical spectrum and therapeutic update". An Bras Dermatol. 90 (1): 62–73. doi:10.1590/abd1806-4841.20152890. PMC 4323700. PMID 25672301.
- ↑ Pogorzelska-Antkowiak A, Antkowiak R (2006). "[Diagnostic and therapeutic problems of scleroderma]". Wiad. Lek. (in Polish). 59 (5–6): 392–5. PMID 17017489.
- ↑ Velayos EE, Masi AT, Stevens MB, Shulman LE (November 1979). "The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma)". Arch. Intern. Med. 139 (11): 1240–4. PMID 508020.
- ↑ Bertsch C (1995). "CREST syndrome: a variant of systemic sclerosis". Orthop Nurs. 14 (2): 53–60, quiz 61. PMID 7761133.
- ↑ Bielsa Marsol I (October 2013). "Update on the classification and treatment of localized scleroderma". Actas Dermosifiliogr. 104 (8): 654–66. doi:10.1016/j.adengl.2012.10.012. PMID 23948159.
- ↑ "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.
- ↑ Pope JE, Johnson SR (August 2015). "New Classification Criteria for Systemic Sclerosis (Scleroderma)". Rheum. Dis. Clin. North Am. 41 (3): 383–98. doi:10.1016/j.rdc.2015.04.003. PMID 26210125.