Scleroderma classification: Difference between revisions

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==Overview==
==Overview==
There is no established system for the classification of [disease name].
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|American College of Rheumatology (ACR)]] 1980 preliminary scleroderma criteria. Scleroderma ([[systemic sclerosis]]) is now classified according to the new [[American College of Rheumatology|American College of Rheumatology (ACR)]]/European League Against Rheumatism (EULAR) criteria.


OR
==Classification==
 
Scleroderma ([[systemic sclerosis]]) is classified into 2 main subtypes:<ref name="pmid3361530">{{cite journal |vauthors=LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F |title=Scleroderma (systemic sclerosis): classification, subsets and pathogenesis |journal=J. Rheumatol. |volume=15 |issue=2 |pages=202–5 |date=February 1988 |pmid=3361530 |doi= |url=}}</ref><ref name="pmid25672301">{{cite journal |vauthors=Careta MF, Romiti R |title=Localized scleroderma: clinical spectrum and therapeutic update |journal=An Bras Dermatol |volume=90 |issue=1 |pages=62–73 |date=2015 |pmid=25672301 |pmc=4323700 |doi=10.1590/abd1806-4841.20152890 |url=}}</ref><ref name="pmid17017489">{{cite journal |vauthors=Pogorzelska-Antkowiak A, Antkowiak R |title=[Diagnostic and therapeutic problems of scleroderma] |language=Polish |journal=Wiad. Lek. |volume=59 |issue=5-6 |pages=392–5 |date=2006 |pmid=17017489 |doi= |url=}}</ref>
[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
*Limited cutaneous scleroderma:
 
**[[Fibrosis]] is restricted to [[hands]], arms and [[face]].
OR
**[[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.<ref name="pmid508020">{{cite journal |vauthors=Velayos EE, Masi AT, Stevens MB, Shulman LE |title=The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma) |journal=Arch. Intern. Med. |volume=139 |issue=11 |pages=1240–4 |date=November 1979 |pmid=508020 |doi= |url=}}</ref><ref name="pmid7761133">{{cite journal |vauthors=Bertsch C |title=CREST syndrome: a variant of systemic sclerosis |journal=Orthop Nurs |volume=14 |issue=2 |pages=53–60; quiz 61 |date=1995 |pmid=7761133 |doi= |url=}}</ref>
**[[Morphea]] is a type of localized scleroderma limited to the [[skin]] and [[Subcutaneous tissue|subcutaneous tissues]].<ref name="pmid23948159">{{cite journal |vauthors=Bielsa Marsol I |title=Update on the classification and treatment of localized scleroderma |journal=Actas Dermosifiliogr |volume=104 |issue=8 |pages=654–66 |date=October 2013 |pmid=23948159 |doi=10.1016/j.adengl.2012.10.012 |url=}}</ref>
**Patients with internal organ involvement with out the [[skin]] manifestations are known to have [[systemic sclerosis]] sine scleroderma.
*Diffuse cutaneous scleroderma:
**It progresses rapidly.
**Large area of the [[skin]] is affected.
**One or more internal organs are affected.
**It is associated with Anti-Scl-70 [[antibody]] (Anti-DNA topoisomerase I [[antibody]]).


[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3].
===American College of Rheumatology (ACR) 1980 preliminary scleroderma criteria===
[Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
Scleroderma was previously classified according to [[American College of Rheumatology|American College of Rheumatology (ACR)]] 1980 preliminary scleroderma criteria:<ref name="pmid7378088">{{cite journal |vauthors= |title=Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee |journal=Arthritis Rheum. |volume=23 |issue=5 |pages=581–90 |date=May 1980 |pmid=7378088 |doi= |url=}}</ref>
:*Major criteria:
:**Proximal [[cutaneous]] [[sclerosis]] ([[skin]] thickening-nonpitting) of fingers that also extended proximally to the [[Metacarpophalangeal joints|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.


OR
===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|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) criteria:<ref name="pmid24092682">{{cite journal |vauthors=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 |title=2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative |journal=Ann. Rheum. Dis. |volume=72 |issue=11 |pages=1747–55 |date=November 2013 |pmid=24092682 |doi=10.1136/annrheumdis-2013-204424 |url=}}</ref><ref name="pmid26210125">{{cite journal |vauthors=Pope JE, Johnson SR |title=New Classification Criteria for Systemic Sclerosis (Scleroderma) |journal=Rheum. Dis. Clin. North Am. |volume=41 |issue=3 |pages=383–98 |date=August 2015 |pmid=26210125 |doi=10.1016/j.rdc.2015.04.003 |url=}}</ref>


Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
{| class="wikitable"
!Item
!Sub-item(s)
!Weight/score
|-
|Skin thickening of the fingers of both hands extending proximal to the [[Metacarpophalangeal joint|metacarpophalangeal joints]] (sufficient criterion)
| -
|9
|-
|Skin thickening of the fingers (only count higher score)
|Puffy fingers


OR
[[Sclerodactyly]] of the fingers (distal to the [[Metacarpophalangeal joint|metacarpophalangeal joints]] but proximal to the proximal interphalangeal joints)
|2


If the staging system involves specific and characteristic findings and features:
4
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].
|-
|Fingertip lesions (only count the higher score)
|Digital tip ulcers


OR
Fingertip pitting scars
|2


The staging of [malignancy name] is based on the [staging system].
3
|-
|[[Telangiectasias|Telangiectasia]]
| -
|2
|-
|Abnormal nail fold capillaries
| -
|2
|-
|[[Pulmonary hypertension|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)


OR
Anti-RNA polymerase-3
|3
|}


There is no established system for the staging of [malignancy name].
*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.
==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):


==References==
==References==
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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]

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]

  • 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

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ Pogorzelska-Antkowiak A, Antkowiak R (2006). "[Diagnostic and therapeutic problems of scleroderma]". Wiad. Lek. (in Polish). 59 (5–6): 392–5. PMID 17017489.
  4. ↑ 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.
  5. ↑ Bertsch C (1995). "CREST syndrome: a variant of systemic sclerosis". Orthop Nurs. 14 (2): 53–60, quiz 61. PMID 7761133.
  6. ↑ 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.
  7. ↑ "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.
  8. ↑ 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.
  9. ↑ 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.

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