Inflammatory myopathy: Difference between revisions
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<nowiki>*</nowiki>Skin manifestations include Heliotrope rash, | <nowiki>*</nowiki>Skin manifestations include Heliotrope rash, Gottron's papules, or Gottron's sign | ||
<nowiki>**</nowiki>Clinical features include | <nowiki>**</nowiki>Clinical features include finger flexor weakness, or no treatment response | ||
<nowiki>***</nowiki>Muscle biopsy includes rimmed vacuoles | <nowiki>***</nowiki>[[Muscle biopsy]] includes rimmed vacuoles | ||
<nowiki>****</nowiki>Muscle weakness manifested as | <nowiki>****</nowiki>[[Muscle weakness]] manifested as objective progressive symmetric [[muscle weakness]] of proximal upper or lower extremities, [[neck]] flexors relatively weaker than extensors, or in the legs proximal muscles relatively weaker than distal | ||
==Differentiating Inflammatory Myopathy from other Diseases== | ==Differentiating Inflammatory Myopathy from other Diseases== | ||
Revision as of 22:15, 6 April 2018
|
Inflammatory Myopathy |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]
Overview
Classification
- The 2017 European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) classified idiopathic inflammatory myopathy into 6 subtypes:[1][2]
- Polymyositis
- Dermatomyositis
- Inclusion body myositis
- Immune-mediated necrotizing myopathies (IMNM)
- Amyopathic dermatomyositis
- Juvenile dermatomyositis
| The 2017 EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathy | Score | |
|---|---|---|
| Variable | No muscle biopsy | With muscle biopsy |
| Age of onset ≥ 18 and < 40 years | 1.3 | 1.5 |
| Age of onset ≥ 40 years | 2.1 | 2.2 |
| Objective symmetric weakness, usually progressive, of the proximal upper extremities | 0.7 | 0.7 |
| Objective symmetric weakness, usually progressive, of the proximal lower extremities | 0.8 | 0.5 |
| Neck flexors are relatively weaker than neck extensors | 1.9 | 1.6 |
| In the legs, proximal muscles are relatively weaker than distal muscles | 0.9 | 1.2 |
| Heliotrope rash | 3.1 | 3.2 |
| Gottron’s papules | 2.1 | 2.7 |
| Gottron’s sign | 3.3 | 3.7 |
| Dysphagia or esophageal dysmotility | 0.7 | 0.6 |
| Anti-Jo1 autoantibody present | 3.9 | 3.8 |
| Elevated serum levels of CK or LDH* or ASAT/AST/SGOT* or ALAT/ALT/SGPT* | 1.3 | 1.4 |
| Endomysial infiltration of mononuclear cells surrounding, but not invading, myofibers on muscle biopsy | 1.7 | |
| Perifascicular atrophy | 1.9 | |
| Rimmed vacuoles | 3.1 | |
Probability of IIM including muscle biopsy=1/[1+exponential (5.33–score)]
Probability of IIM without muscle biopsy=1/[1+exponential (6.49–score)]
To use the calculator to assess the probability of IIM, click here.
Classification algorithm for subgroups of IIM
Abbreviation: ACR, American College of Rheumatology; ADM, amyopathic dermatomyositis; DM, dermatomyositis; EULAR, European League Against Rheumatism; IBM, inclusion body myositis; IIM, idiopathic inflammatory myopathies; IMNM, immune-mediated necrotizing myopathy; JDM, juvenile dermatomyositis; PM, polymyositis
| A patient meets the EULAR/ACR classification criteria for IIM (probability of IIM ≥55%) | |||||||||||||||||||||||||||||||||||||||||||||
| Age of onset 0-17 years old | |||||||||||||||||||||||||||||||||||||||||||||
| No | Yes | ||||||||||||||||||||||||||||||||||||||||||||
| Skin manifestations* | Skin manifestations* | ||||||||||||||||||||||||||||||||||||||||||||
| No | Yes | No | Yes | ||||||||||||||||||||||||||||||||||||||||||
| Clinical features** or muscle biopsy*** | Muscle weakness**** | Juvenile myositis other than JDM | JDM | ||||||||||||||||||||||||||||||||||||||||||
| No | Yes | No | Yes | ||||||||||||||||||||||||||||||||||||||||||
| PM | IBM | ADM | DM | ||||||||||||||||||||||||||||||||||||||||||
*Skin manifestations include Heliotrope rash, Gottron's papules, or Gottron's sign
**Clinical features include finger flexor weakness, or no treatment response
***Muscle biopsy includes rimmed vacuoles
****Muscle weakness manifested as objective progressive symmetric muscle weakness of proximal upper or lower extremities, neck flexors relatively weaker than extensors, or in the legs proximal muscles relatively weaker than distal
Differentiating Inflammatory Myopathy from other Diseases
References
- ↑ Leclair V, Lundberg IE (March 2018). "New Myositis Classification Criteria-What We Have Learned Since Bohan and Peter". Curr Rheumatol Rep. 20 (4): 18. doi:10.1007/s11926-018-0726-4. PMC 5857275. PMID 29550929.
- ↑ Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Dankó K, Dimachkie MM, Feldman BM, Torre IG, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinska M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Song YW, Vencovsky J, Ytterberg SR, Miller FW, Rider LG (December 2017). "2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups". Ann. Rheum. Dis. 76 (12): 1955–1964. doi:10.1136/annrheumdis-2017-211468. PMID 29079590.