Polymyositis and dermatomyositis laboratory findings: Difference between revisions

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==Overview==
==Overview==
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
Elevated [[Sarcoplasmic reticulum|sarcoplasmic]] enzymes are consistent with the diagnosis of polymyositis and dermatomyositis which include [[Creatine kinase|creatine phosphokinase]], [[aldolase]], [[Transaminase|transaminases]], [[Lactate dehydrogenase|lactic dehydrogenase]], and [[myoglobin]]. High [[White blood cells|white blood cell]] counts, low [[Lymphocyte|lymphocytes]], and low [[hematocrit]] levels might be detected on [[Complete blood count|CBC]]. Low [[albumin]] levels, high [[Erythrocyte sedimentation rate|ESR]] and high [[Immunoglobulin M|IgM]] and [[Immunoglobulin G|IgG]] levels could be seen in patients with polymyositis and dermatomyositis. 20 myositis-specific autoantibodies are identified in patients with polymyositis and dermatomyositis. Anti-Jo-1 antibody is the most frequent myositis-specific autoantibodies which causes antisynthetase syndrome. Anti Mi-2 might be seen frequently in patients with dermatomyositis. Anti-SRP antibody is associated with very poor prognosis. Some of these myositis-specific autoantibodies are associated with [[Cancer|malignancy]]. Anti-HMGCR antibody might be elevated in patients with statin-associated necrotizing autoimmune myopathy (SANAM).  


OR
==Laboratory Findings==
 
*Different laboratory tests may be used to diagnose polymyositis and dermatomyositis and exclude other myopathies which include:<ref name="pmid29579414">{{cite journal |vauthors=Adler BL, Christopher-Stine L |title=Triggers of inflammatory myopathy: insights into pathogenesis |journal=Discov Med |volume=25 |issue=136 |pages=75–83 |date=February 2018 |pmid=29579414 |doi= |url=}}</ref><ref name="pmid25182203">{{cite journal |vauthors=Bodoki L, Nagy-Vincze M, Griger Z, Betteridge Z, Szöllősi L, Dankó K |title=Four dermatomyositis-specific autoantibodies-anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5-in adult and juvenile patients with idiopathic inflammatory myopathies in a Hungarian cohort |journal=Autoimmun Rev |volume=13 |issue=12 |pages=1211–9 |date=December 2014 |pmid=25182203 |doi=10.1016/j.autrev.2014.08.011 |url=}}</ref><ref name="KhanChristopher-Stine2011">{{cite journal|last1=Khan|first1=Sabiha|last2=Christopher-Stine|first2=Lisa|title=Polymyositis, Dermatomyositis, and Autoimmune Necrotizing Myopathy: Clinical Features|journal=Rheumatic Disease Clinics of North America|volume=37|issue=2|year=2011|pages=143–158|issn=0889857X|doi=10.1016/j.rdc.2011.01.001}}</ref><ref name="DoblougGaren2015">{{cite journal|last1=Dobloug|first1=Cecilie|last2=Garen|first2=Torhild|last3=Bitter|first3=Helle|last4=Stjärne|first4=Johan|last5=Stenseth|first5=Guri|last6=Grøvle|first6=Lars|last7=Sem|first7=Marthe|last8=Gran|first8=Jan Tore|last9=Molberg|first9=Øyvind|title=Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort|journal=Annals of the Rheumatic Diseases|volume=74|issue=8|year=2015|pages=1551–1556|issn=0003-4967|doi=10.1136/annrheumdis-2013-205127}}</ref><ref name="ChinoyFertig2007">{{cite journal|last1=Chinoy|first1=H.|last2=Fertig|first2=N.|last3=Oddis|first3=C. V|last4=Ollier|first4=W. E R|last5=Cooper|first5=R. G|title=The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis|journal=Annals of the Rheumatic Diseases|volume=66|issue=10|year=2007|pages=1345–1349|issn=0003-4967|doi=10.1136/ard.2006.068502}}</ref><ref name="DalakasHohlfeld2003">{{cite journal|last1=Dalakas|first1=Marinos C|last2=Hohlfeld|first2=Reinhard|title=Polymyositis and dermatomyositis|journal=The Lancet|volume=362|issue=9388|year=2003|pages=971–982|issn=01406736|doi=10.1016/S0140-6736(03)14368-1}}</ref><ref name="DouglasTazelaar2001">{{cite journal|last1=Douglas|first1=William W.|last2=Tazelaar|first2=Henry D.|last3=Hartman|first3=Thomas E.|last4=Hartman|first4=Robert P.|last5=Decker|first5=Paul A.|last6=Schroeder|first6=Darrell R.|last7=Ryu|first7=Jay H.|title=Polymyositis–Dermatomyositis-associated Interstitial Lung Disease|journal=American Journal of Respiratory and Critical Care Medicine|volume=164|issue=7|year=2001|pages=1182–1185|issn=1073-449X|doi=10.1164/ajrccm.164.7.2103110}}</ref><ref name="Miller1993">{{cite journal|last1=Miller|first1=Frederick W.|title=Myositis-Specific Autoantibodies|journal=JAMA|volume=270|issue=15|year=1993|pages=1846|issn=0098-7484|doi=10.1001/jama.1993.03510150080034}}</ref>
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
**Routine tests<ref name="Volochayev2012">{{cite journal|last1=Volochayev|first1=Rita|title=Laboratory Test Abnormalities are Common in Polymyositis and Dermatomyositis and Differ Among Clinical and Demographic Groups|journal=The Open Rheumatology Journal|volume=6|issue=1|year=2012|pages=54–63|issn=18743129|doi=10.2174/1874312901206010054}}</ref>
 
**[[Endoplasmic reticulum|Sarcoplasmic]] enzymes
OR
**Myositis-specific autoantibodies<ref name="Targoff2006">{{cite journal|last1=Targoff|first1=Ira N.|title=Myositis specific autoantibodies|journal=Current Rheumatology Reports|volume=8|issue=3|year=2006|pages=196–203|issn=1523-3774|doi=10.1007/s11926-996-0025-3}}</ref><ref name="GunawardenaBetteridge2009">{{cite journal|last1=Gunawardena|first1=H.|last2=Betteridge|first2=Z. E.|last3=McHugh|first3=N. J.|title=Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression|journal=Rheumatology|volume=48|issue=6|year=2009|pages=607–612|issn=1462-0324|doi=10.1093/rheumatology/kep078}}</ref>
=== Routine test ===
* Abnormal test results include:
** [[Complete blood count|CBC]]
*** High [[White blood cells|white blood cell]] counts
*** Low [[lymphocyte]] counts
*** Low [[hematocrit]] levels
** Low [[albumin]] levels
** High [[erythrocyte sedimentation rate]]
** High [[Immunoglobulin M|IgM]] and [[Immunoglobulin G|IgG]] levels


[Test] is usually normal for patients with [disease name].
=== Sarcoplasmic enzymes ===
* [[Endoplasmic reticulum|Sarcoplasmic]] enzymes consistent with the diagnosis of polymyositis and dermatomyositis include:<ref name="BohanPeter1975">{{cite journal|last1=Bohan|first1=Anthony|last2=Peter|first2=James B.|title=Polymyositis and Dermatomyositis|journal=New England Journal of Medicine|volume=292|issue=8|year=1975|pages=403–407|issn=0028-4793|doi=10.1056/NEJM197502202920807}}</ref>
** [[Creatine kinase|Creatine phosphokinase]] including:
*** [[Creatine kinase|CK-MM]]
*** [[Creatine kinase|CK-MB]]
** [[Aldolase]]
** [[Transaminase|Transaminases]]
*** [[Alanine transaminase|Alanine aminotransferase]]
*** [[Aspartate transaminase|Aspartate aminotransferase]] 
** [[Lactate dehydrogenase|Lactic dehydrogenase]]
** [[Myoglobin]]


OR
=== Myositis-specific autoantibodies ===
{|
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Autoantibody
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Antigen
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Frequency
|-
| rowspan="9" align="left" style="background:#F5F5F5;" + |Antisynthetase
! align="left" style="background:#F5F5F5;" + |Anti-Jo-1
| align="left" style="background:#F5F5F5;" + |Histidyl-tRNA synthetase
| align="left" style="background:#F5F5F5;" + |
* Antisynthetase syndrome
** Arthritis
** Interstitial lung disease
** Fever
** Mechanic's hands
** Raynaud's phenomenon
* Flare up in spring
* Moderate response to treatment
* Poor prognosis
* Associated with epstein-Barr virus (EBV), adenovirus, and influenza infection
| align="left" style="background:#F5F5F5;" + |20-25%
|-
! align="left" style="background:#F5F5F5;" + |Anti-PL-7
| align="left" style="background:#F5F5F5;" + |Threonyl-tRNA synthetase
| align="left" style="background:#F5F5F5;" + |
* Myositis, mechanic's hands, Gottron's papules, arthritis
| align="left" style="background:#F5F5F5;" + |5-10%
|-
! rowspan="2" align="left" style="background:#F5F5F5;" + |Anti PL-12
| align="left" style="background:#F5F5F5;" + |Alanyl-tRNA synthetase
| rowspan="2" align="left" style="background:#F5F5F5;" + |
* Fever, Raynaud's phenomenon, high frequency of ILD
* Epstein-Barr virus (EBV), adenovirus, and influenza infection
| rowspan="2" align="left" style="background:#F5F5F5;" + |<5%
|-
| align="left" style="background:#F5F5F5;" + |Alanyl-tRNA
|-
! align="left" style="background:#F5F5F5;" + |Anti-EJ
| align="left" style="background:#F5F5F5;" + |Glycyl-tRNA synthetase 
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |5-10%
|-
! align="left" style="background:#F5F5F5;" + |Anti-OJ
| align="left" style="background:#F5F5F5;" + |Isoleucyl-tRNA synthetase
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |<5%
|-
! align="left" style="background:#F5F5F5;" + |Anti-KS
| align="left" style="background:#F5F5F5;" + |Asparaginyl-tRNA synthetase
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |<5%
|-
! align="left" style="background:#F5F5F5;" + |Anti-Ha
| align="left" style="background:#F5F5F5;" + |Tyrosyl-tRNA synthetase
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |<1%
|-
! align="left" style="background:#F5F5F5;" + |Anti-Zo
| align="left" style="background:#F5F5F5;" + |Phenylalanyl-tRNA synthetase
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |<1%
|-
| rowspan="12" align="left" style="background:#F5F5F5;" + |Others
! align="left" style="background:#F5F5F5;" + |Anti Mi-2
| align="left" style="background:#F5F5F5;" + |Chromodomain helicase DNA binding protein 4
| align="left" style="background:#F5F5F5;" + |
* Dermatomyositis rash without significant lung involvement
* Acute but mild onset
* Good prognosis
| align="left" style="background:#F5F5F5;" + |5-10%
|-
! align="left" style="background:#F5F5F5;" + |Anti-SRP
| align="left" style="background:#F5F5F5;" + |Signal recognition particle
| align="left" style="background:#F5F5F5;" + |
* Cardiac involvement
* Necrotizing myopathy
* Flare up in fall
* Very acute and severe onset
* Poor response to treatment
* Very poor prognosis
| align="left" style="background:#F5F5F5;" + |<5%
|-
! align="left" style="background:#F5F5F5;" + |Anti-p155/140
| align="left" style="background:#F5F5F5;" + |Transcriptional intermediary factor 1-gamma
| align="left" style="background:#F5F5F5;" + |
* Cancer-associated myositis
* Juvenile dermatomyositis
* Severe cutaneous disease
| align="left" style="background:#F5F5F5;" + |13-21% in IIM


Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
23-29% in JDM
 
|-
OR
! align="left" style="background:#F5F5F5;" + |Anti-MJ
 
| align="left" style="background:#F5F5F5;" + |Nuclear matrix protein (NXP-2)
There are no diagnostic laboratory findings associated with [disease name].
| align="left" style="background:#F5F5F5;" + |
 
* Cancer-associated myositis
==Laboratory Findings==
* Juvenile dermatomyositis
*Laboratory findings consistent with the diagnosis of polymyositis and dermatomyositis include:<ref name="pmid29579414">{{cite journal |vauthors=Adler BL, Christopher-Stine L |title=Triggers of inflammatory myopathy: insights into pathogenesis |journal=Discov Med |volume=25 |issue=136 |pages=75–83 |date=February 2018 |pmid=29579414 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" + |23% in JDM
** Myositis-specific autoantibodies targeting:
|-
***Histidyl-tRNA synthetase (anti-Jo-1)
! align="left" style="background:#F5F5F5;" + |Anti-SAE
***Chromodomain helicase DNA binding protein 4 (Mi-2)  
| align="left" style="background:#F5F5F5;" + |Small ubiquitin-like modifier activating enzyme
***Signal recognition particle (anti-SRP)
| align="left" style="background:#F5F5F5;" + |
***Alanyl-tRNA synthetase (PL-12)
* Cancer-associated myositis
***Transcriptional intermediary factor 1-gamma (TIF-1γ)
| align="left" style="background:#F5F5F5;" + |5%
***Nuclear matrix protein (NXP-2)
|-
***Anti-small ubiquitin-like modifier activating enzyme (anti-SAE)
! align="left" style="background:#F5F5F5;" + |Anti-caDM-140
***Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR)
| align="left" style="background:#F5F5F5;" + |Intracytoplasmic MDA5
| align="left" style="background:#F5F5F5;" + |
* Dermatomyositis and rapidly progressive ILD
| align="left" style="background:#F5F5F5;" + |50% (C-ADM)
|-
! align="left" style="background:#F5F5F5;" + |Anti-PMS1
| align="left" style="background:#F5F5F5;" + |DNA mismatch repair enzyme
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |7.5%
|-
! align="left" style="background:#F5F5F5;" + |Anti-HMGCR
| align="left" style="background:#F5F5F5;" + |3-hydroxy-3-methylglutaryl-coenzyme A reductase
| align="left" style="background:#F5F5F5;" + |
* Statin-associated necrotizing autoimmune myopathy (SANAM)
| align="left" style="background:#F5F5F5;" + |6%
|-
! align="left" style="background:#F5F5F5;" + |Anti-FER
| align="left" style="background:#F5F5F5;" + |Elongation factor 1 alpha
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
|-
! align="left" style="background:#F5F5F5;" + |Anti-KJ
| align="left" style="background:#F5F5F5;" + |Unidentified cytoplasmic protein 
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
|-
! align="left" style="background:#F5F5F5;" + |Anti-MAS
| align="left" style="background:#F5F5F5;" + |Unidentified cytoplasmic RNA
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
|-
! align="left" style="background:#F5F5F5;" + |Anti–PM-Scl
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* Overlap of polymyositis and Scleroderma
| align="left" style="background:#F5F5F5;" + |25%
|}


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 13:20, 4 June 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Overview

Elevated sarcoplasmic enzymes are consistent with the diagnosis of polymyositis and dermatomyositis which include creatine phosphokinase, aldolase, transaminases, lactic dehydrogenase, and myoglobin. High white blood cell counts, low lymphocytes, and low hematocrit levels might be detected on CBC. Low albumin levels, high ESR and high IgM and IgG levels could be seen in patients with polymyositis and dermatomyositis. 20 myositis-specific autoantibodies are identified in patients with polymyositis and dermatomyositis. Anti-Jo-1 antibody is the most frequent myositis-specific autoantibodies which causes antisynthetase syndrome. Anti Mi-2 might be seen frequently in patients with dermatomyositis. Anti-SRP antibody is associated with very poor prognosis. Some of these myositis-specific autoantibodies are associated with malignancy. Anti-HMGCR antibody might be elevated in patients with statin-associated necrotizing autoimmune myopathy (SANAM).

Laboratory Findings

Routine test

Sarcoplasmic enzymes

Myositis-specific autoantibodies

Autoantibody Antigen Features Frequency
Antisynthetase Anti-Jo-1 Histidyl-tRNA synthetase
  • Antisynthetase syndrome
    • Arthritis
    • Interstitial lung disease
    • Fever
    • Mechanic's hands
    • Raynaud's phenomenon
  • Flare up in spring
  • Moderate response to treatment
  • Poor prognosis
  • Associated with epstein-Barr virus (EBV), adenovirus, and influenza infection
20-25%
Anti-PL-7 Threonyl-tRNA synthetase
  • Myositis, mechanic's hands, Gottron's papules, arthritis
5-10%
Anti PL-12 Alanyl-tRNA synthetase
  • Fever, Raynaud's phenomenon, high frequency of ILD
  • Epstein-Barr virus (EBV), adenovirus, and influenza infection
<5%
Alanyl-tRNA
Anti-EJ Glycyl-tRNA synthetase  5-10%
Anti-OJ Isoleucyl-tRNA synthetase <5%
Anti-KS Asparaginyl-tRNA synthetase <5%
Anti-Ha Tyrosyl-tRNA synthetase <1%
Anti-Zo Phenylalanyl-tRNA synthetase <1%
Others Anti Mi-2 Chromodomain helicase DNA binding protein 4
  • Dermatomyositis rash without significant lung involvement
  • Acute but mild onset
  • Good prognosis
5-10%
Anti-SRP Signal recognition particle
  • Cardiac involvement
  • Necrotizing myopathy
  • Flare up in fall
  • Very acute and severe onset
  • Poor response to treatment
  • Very poor prognosis
<5%
Anti-p155/140 Transcriptional intermediary factor 1-gamma
  • Cancer-associated myositis
  • Juvenile dermatomyositis
  • Severe cutaneous disease
13-21% in IIM

23-29% in JDM

Anti-MJ Nuclear matrix protein (NXP-2)
  • Cancer-associated myositis
  • Juvenile dermatomyositis
23% in JDM
Anti-SAE Small ubiquitin-like modifier activating enzyme
  • Cancer-associated myositis
5%
Anti-caDM-140 Intracytoplasmic MDA5
  • Dermatomyositis and rapidly progressive ILD
50% (C-ADM)
Anti-PMS1 DNA mismatch repair enzyme 7.5%
Anti-HMGCR 3-hydroxy-3-methylglutaryl-coenzyme A reductase
  • Statin-associated necrotizing autoimmune myopathy (SANAM)
6%
Anti-FER Elongation factor 1 alpha
Anti-KJ Unidentified cytoplasmic protein 
Anti-MAS Unidentified cytoplasmic RNA
Anti–PM-Scl
  • Overlap of polymyositis and Scleroderma
25%

References

  1. Adler BL, Christopher-Stine L (February 2018). "Triggers of inflammatory myopathy: insights into pathogenesis". Discov Med. 25 (136): 75–83. PMID 29579414.
  2. Bodoki L, Nagy-Vincze M, Griger Z, Betteridge Z, Szöllősi L, Dankó K (December 2014). "Four dermatomyositis-specific autoantibodies-anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5-in adult and juvenile patients with idiopathic inflammatory myopathies in a Hungarian cohort". Autoimmun Rev. 13 (12): 1211–9. doi:10.1016/j.autrev.2014.08.011. PMID 25182203.
  3. Khan, Sabiha; Christopher-Stine, Lisa (2011). "Polymyositis, Dermatomyositis, and Autoimmune Necrotizing Myopathy: Clinical Features". Rheumatic Disease Clinics of North America. 37 (2): 143–158. doi:10.1016/j.rdc.2011.01.001. ISSN 0889-857X.
  4. Dobloug, Cecilie; Garen, Torhild; Bitter, Helle; Stjärne, Johan; Stenseth, Guri; Grøvle, Lars; Sem, Marthe; Gran, Jan Tore; Molberg, Øyvind (2015). "Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort". Annals of the Rheumatic Diseases. 74 (8): 1551–1556. doi:10.1136/annrheumdis-2013-205127. ISSN 0003-4967.
  5. Chinoy, H.; Fertig, N.; Oddis, C. V; Ollier, W. E R; Cooper, R. G (2007). "The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis". Annals of the Rheumatic Diseases. 66 (10): 1345–1349. doi:10.1136/ard.2006.068502. ISSN 0003-4967.
  6. Dalakas, Marinos C; Hohlfeld, Reinhard (2003). "Polymyositis and dermatomyositis". The Lancet. 362 (9388): 971–982. doi:10.1016/S0140-6736(03)14368-1. ISSN 0140-6736.
  7. Douglas, William W.; Tazelaar, Henry D.; Hartman, Thomas E.; Hartman, Robert P.; Decker, Paul A.; Schroeder, Darrell R.; Ryu, Jay H. (2001). "Polymyositis–Dermatomyositis-associated Interstitial Lung Disease". American Journal of Respiratory and Critical Care Medicine. 164 (7): 1182–1185. doi:10.1164/ajrccm.164.7.2103110. ISSN 1073-449X.
  8. Miller, Frederick W. (1993). "Myositis-Specific Autoantibodies". JAMA. 270 (15): 1846. doi:10.1001/jama.1993.03510150080034. ISSN 0098-7484.
  9. Volochayev, Rita (2012). "Laboratory Test Abnormalities are Common in Polymyositis and Dermatomyositis and Differ Among Clinical and Demographic Groups". The Open Rheumatology Journal. 6 (1): 54–63. doi:10.2174/1874312901206010054. ISSN 1874-3129.
  10. Targoff, Ira N. (2006). "Myositis specific autoantibodies". Current Rheumatology Reports. 8 (3): 196–203. doi:10.1007/s11926-996-0025-3. ISSN 1523-3774.
  11. Gunawardena, H.; Betteridge, Z. E.; McHugh, N. J. (2009). "Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression". Rheumatology. 48 (6): 607–612. doi:10.1093/rheumatology/kep078. ISSN 1462-0324.
  12. Bohan, Anthony; Peter, James B. (1975). "Polymyositis and Dermatomyositis". New England Journal of Medicine. 292 (8): 403–407. doi:10.1056/NEJM197502202920807. ISSN 0028-4793.