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:
  • 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
• 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):

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