Contents
The diagnosis is based on
- Medical history of symptoms or proven pathology
- Lungs
- Muscles
- Arthritis (Arthritis)
- Skin
- Antibodies
- Pulmonary abnormalities
- Myositis
- Antibodies
- Exclude another reason (Differential diagnosis)
Disease history
- Debut (time) for onset of symptoms: dry cough, dyspnea at physical stress
-
Lungs
- Dry cough
- Dyspnea at physical stress
- Quickly progressive symptoms from lungs (increase from week to week)?
-
Muscles (Myositis)
- Pain
- Weakness
- Dysphagia (esophagus affection)
- Proximal (classical) and / or distal affection
Immunomodulatory treatment to date
- Methyl prednisolone (SoluMedrol)
- Prednisone (Maximum and actual dose)
- Cyclophosphamide (Sendoxan; treatment duration and dose), Rituximab (Rixathon, MabThera), Mycophenolate (CellCept), Azathioprine (Azathioprine), methotrexate, others)
Clinical examination
-
Lungs
- Crepitations over lungs by auscultation?
-
Skin
- Mechanic's Hands (Cracked dry skin on hands)
- Gottron's sign or papules (red-violet color over MCP joints)
- V-shaped sign (chest and throat) (Red-blue color)
- Shawl-shaped characters (neck and back) (Red-blue color)
- Heliotropic rash (eyelid)
- Raynaud's phenomena (Three-phasic)
-
Joint
- 90-90 deformity in thumb
- Rheumatoid arthritis like courses occur
-
Muscles
- Muscular atrophy and reduced force (rising from chair / squat without support?)
- Weaker muscle strength (proximal) in the thighs, arms or neck?
- Reduced walking function?
Laboratory tests
- Creatine kinase (CK) is usually moderately elevated
- AST and LD (usually elevated)
- Antibodies ( "Myositis-specific)
- Jo-1, P1-7 or P1-12 (+ some very rare) are usually present
- ANA (antinuclear antibody) of 50%
- SSA (Ro 52) is often positive in low titer without at the same time Sjögren's syndrome eller SLE may be present
Supplementary Medical Examination
- Lung function tests (including DLCO)
-
Radiological
- HRCT examinations of lungs
- X-ray examination may be normal even in case of major lung changes
- MRI examination (with contrast) of proximal muscles, most of both thighs
- MRI locates muscle groups with the most manifestations. Localization for biopsy (see below)
- X-ray or manometry of esophagus (swallowing)
-
Laboratory
- Rutine (Hb, Erythrocyte Sedimentation Rate (ESR), CRP, leukocytes, platelets, liver enzymes, K, Na, Cl, Ca, Creatinine, TSH, T4, creatine kinaseCK)
- Myositite specific autoantibodies (a-Jo-1 in 80-90%, Pl-7 and Pl-12 and others are less frequent)
- Anti-SSA (Ro) antibody (as by Sjögren's syndrome og SLE) is not uncommon
-
Capillaroscopy
- Pathological findings are somewhat less frequent than at Systemic sclerosis
-
Muscle Biopsy
- Will be performed if increased CK, edema on MRI, or positive EMG (electromyography).
Malignancy
- Malignancy investigations should be considered (especially in adult dermatomyositis)
- Gynecological examination, CT abdominal examination, electrophoresis urine / serum, stool of blood test, colonoscopy, other investigations if clinical suspicion
- Alternatively: PET / CT
Differential diagnosis
Myositis (BINDEVEVSSYKDOMMER.no)
Antisynthetase syndrome, BINDEVEVSSYKDOMMER.no
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