Differentiating Scleroderma from other diseases: Difference between revisions

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! colspan="2" rowspan="4" |Diseases
! colspan="2" rowspan="4" |Diseases
| colspan="7" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
| colspan="7" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
|
! colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
! colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Additional findings
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Additional findings
|-
|-
| colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
! colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
! colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
|-
|-
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
|-  
|-  
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! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Raynaud phenomenon
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Raynaud phenomenon
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Heart burn
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Heart burn
!Dyspnea
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Edema (swelling)
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Edema (swelling)
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sclerodactyly
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sclerodactyly
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! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Autoantibodies
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Autoantibodies
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Blood indices
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Blood indices
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Nailfold video capillaroscopy
|-
|-
| rowspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma<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="pmid8340733">{{cite journal |vauthors=Black CM |title=Scleroderma--clinical aspects |journal=J. Intern. Med. |volume=234 |issue=2 |pages=115–8 |date=August 1993 |pmid=8340733 |doi= |url=}}</ref>
| rowspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma<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="pmid8340733">{{cite journal |vauthors=Black CM |title=Scleroderma--clinical aspects |journal=J. Intern. Med. |volume=234 |issue=2 |pages=115–8 |date=August 1993 |pmid=8340733 |doi= |url=}}</ref>
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* Pulmonary hypertension
* Pulmonary hypertension
* Calcinosis
* Calcinosis
* Dyspnea
|-
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Diffuse cutaneous systemic sclerosis
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Diffuse cutaneous systemic sclerosis
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Revision as of 13:48, 20 April 2018

Scleroderma Microchapters

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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

[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].

OR

[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].

Differentiating X from other Diseases

  • [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
  • [Disease name] must be differentiated from [differential dx1], [differential dx2], and [differential dx3].
  • As [disease name] manifests in a variety of clinical forms, differentiation must be established in accordance with the particular subtype. [Subtype name 1] must be differentiated from other diseases that cause [clinical feature 1], such as [differential dx1] and [differential dx2]. In contrast, [subtype name 2] must be differentiated from other diseases that cause [clinical feature 2], such as [differential dx3] and [differential dx4].

Preferred Table

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Skin thickening Raynaud phenomenon Heart burn Edema (swelling) Sclerodactyly Telangiectasia Impaired mobility Autoantibodies Blood indices
Scleroderma[1][2] Limited cutaneous systemic sclerosis (CREST syndrome) +

(induration)

+ + +/- + + +/-
  • Anti-centromere antibody
  • Antinuclear antibody (ANA)
  • Anemia
  • Elevated creatinine level
  • Nailfold microvascular changes
  • Chest CT showing evidence of pulmonary fibrosis
  • Nailfold microvascular changes
  • Dilated capillary loops
  • Microhemmorhages
  • Interstitial lung disease
  • Pulmonary hypertension
  • Calcinosis
  • Dyspnea
Diffuse cutaneous systemic sclerosis +

(induration)

+ + +/- + + +/-
  • Anti-topoisomerase-I (Scl-70) antibody
  • Antinuclear antibody (ANA)
  • Anti-RNA polymerase III antibody
  • Anemia
  • Elevated creatinine level
  • Nailfold microvascular changes
  • Nailfold microvascular changes
  • Dilated capillary loops
  • Microhemmorhages
  • Interstitial lung disease
  • Pulmonary hypertension
  • Scleroderma renal crisis
Systemic diseases Scleredema

(Buschke's disease)[3]

+ - - + - - + - - -
  • Normal epidermis
  • Thickened dermis with swelling of collagen
  • Fibroblast proliferation is absent in the dermis
  • symmetrical skin thickening
  • trunk, shoulders, upper back, face
  • fingers are spared
  • Mobility of shoulders and chest
  • Internal organ involvement rare
  • Associated with viral URTI and Diabetes mellitus type 1, monoclonal gammopathy
Scleromyxedema

(lichen myxedematosus)

Fibroblast proliferation in the dermis
Amyloidosis
Eosinophilic fasciitis + Eosinophilia (peripheral blood smear) Skin changes present on trunk sparing extremities
Chronic graft-versus-host disease +

(induration)

-
  • Antinuclear antibody (ANA)
  • Anti-mitochondrial antibody
 Occurs after allogeneic hematopoietic transplantation
Drug induced scleroderma
Scleroderma overlap syndromes[4][5][6][7] Systemic lupus erythematosus +

(rash)

+ + +/- + + +/-
  • Anti-centromere antibody (ACA)
  • Anti-topoisomerase-I (Scl-70) antibody
  • Anti-RNA polymerase III antibody
  • Antinuclear antibody (ANA)
  • Anti-Smith antibody
  • Anti-dsDNA antibody
  • Antiphospholipid antibody
  • Anti-Ro52
  • Decreased C3, C4 and CH50
  • Anemia
 Inflammation at the dermal-epidermal junction
  • Malar (butterfly) rash
  • Arthritis
Polymyositis +

(rash)

+ + +/- + + +/-
  • Anti-centromere antibody (ACA)
  • Anti-topoisomerase-I (Scl-70) antibody
  • Anti-RNA polymerase III antibody
  • Antinuclear antibody (ANA)
  • Anti-Jo-1 antibody
  • Anti-SRP antibody
  • Anti-Mi-2 antibody
  • Anti-Ro52
  • Elevated creatinine kinase
  • Aldolase
  • Anemia
  • Inflammation at the dermal-epidermal junction
  • Atrophy of the epidermis
  • Perivascular infiltrate in the dermis
 Symmetric proximal muscle weakness
Rheumatoid arthritis +

(rash)

+ + +/- + + +/-
  • Anti-centromere antibody (ACA)
  • Anti-topoisomerase-I (Scl-70) antibody
  • Anti-RNA polymerase III antibody
  • Antinuclear antibody (ANA)
  • Antinuclear antibody (ANA)
  • Rheumatoid factor (RA) +ve
  • Anti-CCP antibody
  • Anti-Ro52
  • Elevated ESR
  • Anemia
 Inflammation at the dermal-epidermal junction Clinical findings that suggest rheumatoid arthritis (RA) but do not fulfill the ACR criteria for RA
Endocrine disorders Diabetes mellitus (diabetic cheiroarthropathy)[8][9] +

(waxy skin)

- - - + - + - - Long standing type I diabetes
Myxedema due to hypothyroidism[10] +

(coarse skin)

- - + - - -
  • Anti-TPO antibody
  • Anti-Tg antibody
  • Anemia
  • Serum TSH
  • Atrophied epidermis
  • Hyperkeratosis
 Hypothyroidism
Renal diseases Nephrogenic systemic fibrosis[11] +

(induration)

- - + + - - -
  • Elevated C-reactive protein
  • Elevated ESR
  • Decreased serum albumin
  • Decreased creatinine clearance
  • Expansion and fibrosis of dermis
  • Accumulation of CD34 positive fibroblasts
  • Skin changes on extremities and trunk
  • Affects patients with advanced deals dependent renal failure
  • Gadolinium containing contrast exposure is a risk factor

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. ↑ Black CM (August 1993). "Scleroderma--clinical aspects". J. Intern. Med. 234 (2): 115–8. PMID 8340733.
  3. ↑ Rongioletti F, Kaiser F, Cinotti E, Metze D, Battistella M, Calzavara-Pinton PG, Damevska K, Girolomoni G, André J, Perrot JL, Kempf W, Cavelier-Balloy B (December 2015). "Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients". J Eur Acad Dermatol Venereol. 29 (12): 2399–404. doi:10.1111/jdv.13272. PMID 26304054.
  4. ↑ Satoh M, Chan EK, Sobel ES, Kimpel DL, Yamasaki Y, Narain S, Mansoor R, Reeves WH (September 2007). "Clinical implication of autoantibodies in patients with systemic rheumatic diseases". Expert Rev Clin Immunol. 3 (5): 721–38. doi:10.1586/1744666X.3.5.721. PMID 20477023.
  5. ↑ Moinzadeh P, Aberer E, Ahmadi-Simab K, Blank N, Distler JH, Fierlbeck G, Genth E, Guenther C, Hein R, Henes J, Herich L, Herrgott I, Koetter I, Kreuter A, Krieg T, Kuhr K, Lorenz HM, Meier F, Melchers I, Mensing H, Mueller-Ladner U, Pfeiffer C, Riemekasten G, Sárdy M, Schmalzing M, Sunderkoetter C, Susok L, Tarner IH, Vaith P, Worm M, Wozel G, Zeidler G, Hunzelmann N (April 2015). "Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis". Ann. Rheum. Dis. 74 (4): 730–7. doi:10.1136/annrheumdis-2013-204487. PMC 4392314. PMID 24389298.
  6. ↑ Foocharoen C, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R (September 2016). "Clinical characteristics of scleroderma overlap syndromes: comparisons with pure scleroderma". Int J Rheum Dis. 19 (9): 913–23. doi:10.1111/1756-185X.12884. PMID 27126733.
  7. ↑ Pakozdi A, Nihtyanova S, Moinzadeh P, Ong VH, Black CM, Denton CP (November 2011). "Clinical and serological hallmarks of systemic sclerosis overlap syndromes". J. Rheumatol. 38 (11): 2406–9. doi:10.3899/jrheum.101248. PMID 21844148.
  8. ↑ Seibold JR (November 1982). "Digital sclerosis in children with insulin-dependent diabetes mellitus". Arthritis Rheum. 25 (11): 1357–61. PMID 6753855.
  9. ↑ Jelinek JE (April 1993). "The skin in diabetes". Diabet. Med. 10 (3): 201–13. PMID 8485952.
  10. ↑ Heymann WR (June 1992). "Cutaneous manifestations of thyroid disease". J. Am. Acad. Dermatol. 26 (6): 885–902. PMID 1607406.
  11. ↑ Galan A, Cowper SE, Bucala R (November 2006). "Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy)". Curr Opin Rheumatol. 18 (6): 614–7. doi:10.1097/01.bor.0000245725.94887.8d. PMID 17053507.

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