Keywords for Inquiry, Referral, Journal Writing at Antisynthetics Syndrome 5/5 (4)

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The diagnosis is based on

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

Clinical examination

  • 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 

  • 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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