Contents
Classification criteria EULAR / ACR 2017 (Reference Lundberg IE, 2017)
The criteria summarize scores based on the following parameters with or without muscle biopsy available
Criteria:
- Age at symptom start between 18 and 40 years
- without biopsy 1,3 points, with biopsy 1,5 points
- Age at symptom start between 40 years or older
- without biopsy 2,1 points, with biopsy 2,2 points
- Unbiased symmetrical, usually progressive in upper arms
- without biopsy 0,7 points, with biopsy 0,7 points
- Unbiased symmetrical, usually progressive in thighs
- without biopsy 0,8 points, with biopsy 0,5 points
- Neck flexor muscles (flexing head) relatively weaker than neck extensors
- without biopsy 1,9 points, with biopsy 1,6 points
- Thigh muscles relatively weaker than calves
- without biopsy 0,9 points, with biopsy 1,2 points
- Heliotrophic rash (around eyes)
- without biopsy 3,1 points, with biopsy 3,2 points
- Gottron's papels (over the fingerjoints, elbows or knees)
- without biopsy 2,1 points, with biopsy 2,7 points
- Gottrons` sign
- without biopsy 3,3 points, with biopsy 3,7 points
- Dysphagia or esophageal dysmotility
- without biopsy 0,7 points, with biopsy 0,6 points
- Anti-Jo-1 antibody
- without biopsy 3,9 points, with biopsy 3,8 points
- Elevated Enzymes (Without Biopsy 1,3 Points, With Biopsy 1,4 Points)
- CK (creatine kinase) or
- LD (lactate dehydrogenase) or
- AST / ALT
- Muscle biopsy with endomyal infiltrations of mononuclear cells surrounding, but not penetrating muscle fibers (muscle cells)
- Biopsy positive 1,7
- Muscle biopsy with perimyseal and / or perivascular infiltration of mononuclear cells
- Biopsy positive 1,2
- Muscle biopsy with perifascular atrophy
- Biopsy positive 1,9
- Muscle biopsy with “Rimmed vacuoles”
- Biopsy positive 3,1
"Definite myositis" (at least 90% definite) if total summed score is at least 7,5 without biopsy or at least 8,7 where biopsy is available
Diagnostic criteria for poly- and dermatomyositis (Bohan and Peter)*
- 1. Symmetrical proximal muscle weakness
- 2. Elevated muscle enzymes in serum (CK, aldolase, AST, ALAT, LD)
- 3. EMG with polyphase MUP, "sharp waves" and repetitive discharges
- 4. Muscle biopsy; Degeneration, regeneration, necrosis, phagocytosis and interstitial mononuclear infiltrates
- Typical exanthema (eg heliotropic exanthema, Gottron's signs / papules)
- In order to diagnose DM, criterion 5 must be present
- Possible PM / DM = 2 criteria
- Probably PM / DM = 3 criteria
- Definitive PM / DM = 4 criteria
* The criteria assume that other causes of myopathy are excluded.
Revised classification criteria for poly / dermatomyositis (Targoff et al.)
- 1. Symmetrical proximal muscle weakness
- 2. Elevated muscle enzymes
- 3. EMG with myopathic "MUP", positive "sharp waves", repetitive discharges.
- 4. Muscle biopsy; Degeneration, regeneration, necrosis, phagocytosis and interstitial mononuclear infiltrates
- 5. One of the myositis specific antibodies
- 6. Typical eczema
Positive MRI examination for inflammation may replace criterion 1 or 2. In order to diagnose DM, criterion 6 must be present.
- Possible PM / DM = 2 criteria
- Probably PM / DM = 3 criteria
- Definitive PM / DM = 4 criteria
Myositis, BINDEVEVSSYKDOMMER.no
This page has had 1 visits today