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Rheumatology

Scleroderma

Classification

2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative

Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints

Skin thickening or hardening not due to scarring after injury, trauma etc.
This field is required
Puffy fingers

Swollen digits - a diffuse, usually nonpitting increase in soft tissue mass of the digits extending beyond the normal confines of the joint capsule. Normal digits are tapered distally with the tissues following the contours of the digital bone and joint structures. Swelling of the digits obliterates these contours. Not due to other reasons such as inflammatory dactylitis.
This field is required
Sclerodactyly of the fingers

Swollen digits - a diffuse, usually nonpitting increase in soft tissue mass of the digits extending beyond the normal confines of the joint capsule. Normal digits are tapered distally with the tissues following the contours of the digital bone and joint structures. Swelling of the digits obliterates these contours. Not due to other reasons such as inflammatory dactylitis.
This field is required
Digital tip ulcers

Ulcers or scars distal to or at the proximal interphalangeal joint not thought to be due to trauma.

This field is required
Fingertip pitting scars

Digital pitting scars are depressed areas at digital tips as a result of ischemia, rather than trauma or exogenous causes.
This field is required
Telangiectasia

Telangiectasiae are visible macular dilated superficial blood vessels, which collapse upon pressure and fill slowly when pressure is released. Telangiectasiae in a scleroderma-like pattern are round and well demarcated and found on hands, lips, inside of the mouth, and/or are large mat-like telangiectasiae. Distinguishable from rapidly filling spider angiomas with central arteriole and from dilated superficial vessels.
This field is required
Abnormal nail fold capillaries

Enlarged capillaries and/or capillary loss with or without pericapillary hemorrhages at the nailfold. May also be seen on the cuticle.
This field is required
Pulmonary arterial hypertension

Pulmonary arterial hypertension diagnosed by right-sided heart catheterization according to standard definitions.
This field is required
Interstitial lung disease

Pulmonary fibrosis seen on high-resolution computed tomography or chest radiography, most pronounced in the basilar portions of the lungs, or occurrence of “Velcro” crackles on auscultation, not due to another cause such as congestive heart failure
This field is required
Raynaud's phenomenon

Self-reported or reported by a physician, with at least a 2-phase color change in finger(s) and often toe(s) consisting of pallor, cyanosis, and/or reactive hyperemia in response to cold exposure or emotion; usually one phase is pallor.
This field is required
Anticentromere antibodies

Positive according to local laboratory standards.
This field is required
Anti-topoisomerasel [anti-ScL 70]

Positive according to local laboratory standards.
This field is required
Anti-RNA polymerase III

Positive according to local laboratory standards.
This field is required

Statistics

Sensitivity: 91%

Specificity: 92%

Considerations

The tool is for educational use only, is not meant to be a substitute for professional advice and should not be used for medical diagnosis and/or medical treatment.

  • Alternate diagnoses mimicking scleroderma should be excluded prior to applying the criteria
Reference

van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, 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. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424. PMID: 24092682.

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