Scleroderma classification: Difference between revisions

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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>
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>
*Limited cutaneous scleroderma:
*Limited cutaneous scleroderma:
**[[Fibrosis]] is restricted to [[hands]], arms and [[face]]
**[[Fibrosis]] is restricted to [[hands]], arms and [[face]].
**[[Raynaud's phenomenon]] present for many years before [[fibrosis]] appears
**[[Raynaud's phenomenon]] is present for many years before the onset of [[fibrosis]].
**[[Pulmonary hypertension]] often occurs
**[[Pulmonary hypertension]] often occurs.
**[[Anti-centromere antibodies]] are present in more than half of the patients
**[[Anti-centromere antibodies]] are present in more than half of the patients.
**[[Anti-centromere antibodies]] 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>  
**[[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>
**[[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
**Patients with internal organ involvement with out the [[skin]] manifestations are known to have [[systemic sclerosis]] sine scleroderma.
*Diffuse cutaneous scleroderma:
*Diffuse cutaneous scleroderma:
**Progresses rapidly
**It progresses rapidly.
**Large area of the [[skin]] is affected
**Large area of the [[skin]] is affected.
**One or more internal organs are affected
**One or more internal organs are affected.
**Associated with Anti-Scl-70 [[antibody]] (Anti-DNA topoisomerase I [[antibody]])
**It is associated with Anti-Scl-70 [[antibody]] (Anti-DNA topoisomerase I [[antibody]]).


===American College of Rheumatology (ACR) 1980 preliminary scleroderma criteria===
===American College of Rheumatology (ACR) 1980 preliminary scleroderma criteria===
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:**Digital pitting scars of fingertips or loss of substance of the [[distal]] finger pad (digital tuft resorption)
:**Digital pitting scars of fingertips or loss of substance of the [[distal]] finger pad (digital tuft resorption)
:**Bibasilar [[pulmonary fibrosis]]
:**Bibasilar [[pulmonary fibrosis]]
*If major criteria was present that was sufficient otherwise 2 of 3 minor criteria had to be present.
*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===
===American College of Rheumatology (ACR)/ European League Against Rheumatism (EULAR) criteria===
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!Item
!Item
!Sub-item(s)
!Sub-item(s)
!Weight/Score
!Weight/score
|-
|-
|Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (sufficient criterion)
|Skin thickening of the fingers of both hands extending proximal to the [[Metacarpophalangeal joint|metacarpophalangeal joints]] (sufficient criterion)
| -
| -
|9
|9
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|Puffy fingers  
|Puffy fingers  


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


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3
3
|-
|-
|Telangiectasia
|[[Telangiectasias|Telangiectasia]]
| -
| -
|2
|2
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|2
|2
|-
|-
|Pulmonary arterial hypertension and/or Interstitial lung disease
|[[Pulmonary hypertension|Pulmonary arterial hypertension]] and/or [[interstitial lung disease]]
|
|
|2
|2
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|3
|3
|-
|-
|Any of the Scleroderma related autoantibodies
|Any of the scleroderma related [[autoantibodies]]
|Anti-centromere
|[[Anti-centromere antibodies]]
Anti-topoisomerase I (anti-SCL-70)
Anti-topoisomerase I (anti-SCL-70)


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|}
|}


*The total score is determined by adding only the highest weight/score in each category
*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
*Patients with a total score ≥ 9 are classified as having scleroderma.


==References==
==References==
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{{WH}}
{{WH}}
{{WS}}
{{WS}}
[[Category:Up-To-Date]]
[[Category:Up-To-Date]]
[[Category:Primary care]]
[[Category:Medicine]]
[[Category:Medicine]]
[[Category:Dermatology]]
[[Category:Dermatology]]
[[Category:Rheumatology]]
[[Category:Rheumatology]]

Latest revision as of 00:05, 30 July 2020

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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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