Scleroderma diagnostic study of choice

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 single diagnostic study of choice for the diagnosis of scleroderma. Scleroderma is mainly diagnosed based on clinical presentation, though scleroderma (systemic sclerosis) maybe diagnosed based on the new American College of Rheumatology (ACR)/ European League Against Rheumatism (EULAR) classification criteria. The new criteria is more sensitive and is better for detecting disease earlier, so that the fatal renal and pulmonary complications can be screened for and prevented.

Diagnostic Study of Choice

Study of choice

There is no single diagnostic study of choice for the diagnosis of scleroderma. Scleroderma is mainly diagnosed based on clinical presentation.

Diagnostic Criteria

There is no single diagnostic study of choice for scleroderma, though scleroderma (systemic sclerosis) maybe diagnosed based on the new American College of Rheumatology (ACR)/ European League Against Rheumatism (EULAR) classification criteria:^[1]^[2]^[3]

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 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.
The new criteria is more sensitive and is better for detecting disease earlier, so that the fatal renal and pulmonary complications can be screened for and prevented.

Scleroderma was previously classified according to American College of Rheumatology (ACR) 1980 preliminary scleroderma criteria:^[4]

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
Scleroderma is classified if either one major criteria or 2 of 3 minor criteria are present.

References

↑ 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.
↑ Romanowska-Próchnicka K, Walczyk M, Olesińska M (2016). “Recognizing systemic sclerosis: comparative analysis of various sets of classification criteria”. Reumatologia. 54 (6): 296–305. doi:10.5114/reum.2016.64906. PMC 5241366. PMID 28115780.
↑ “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.

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[pmid24092682-1] 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.

[pmid26210125-2] 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.

[pmid28115780-3] Romanowska-Próchnicka K, Walczyk M, Olesińska M (2016). “Recognizing systemic sclerosis: comparative analysis of various sets of classification criteria”. Reumatologia. 54 (6): 296–305. doi:10.5114/reum.2016.64906. PMC 5241366. PMID 28115780.

[pmid7378088-4] “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.

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