Clinical Specialist Services Care Group Department of ...
[Pages:2]Clinical Specialist Services Care Group Department of Pathology - Immunology
MYOSITIS SCREEN Screen includes:
- Mi-2, Ku, PM-Scl100, PM-Scl75, Jo-1, SRP, PL-7, PL-12, EJ, OJ, Ro-52
Specific Information
Laboratory
Immunology
Specimen type
Serum
Container
- Standard - Paediatric - Neonates
4.0 mL White top yellow ring Vacuette 2.5 mL Yellow top white ring Reduced draw 0.8 mL Ochre top MiniCollect
Clinical information Special considerations and limitations Reference ranges Clinical significance of test
Please supply sufficient clinical information necessary for test interpretation Myositis is rare and antibodies are found in about half of patients.
Normal result = Negative Mi-2 antibodies are typically found in patients with steroid responsive dermatomyositis. They are rare in polymyositis. Mi-2 antibodies recognise nuclear helicase and homogenous staining on Hep2 slides is usually seen. Mi-2 antibodies are invariably of high titre and show no variation during the course of the disease or treatment.
Antibodies to PM-Scl75 and PM-Scl100 antigen are found in 50-70% of patients with the polymyositis/scleroderma overlap syndrome. PM-Scl75 is seen in 8% of patients with myositis and 3% of patients with systemic sclerosis but 25% of patients with scleroderma/myositis overlap syndrome. PM-Scl100 is not as closely associated with systemic sclerosis as PM-Scl75. Strong nucleolar staining with weak, fine speckled, nucleoplasmic staining can be seen on Hep2 immunofluorescence.
Ku antibodies are seen in a variety of diseases including systemic lupus erythematosus, mixed connective tissue disease, scleroderma and the polymysitis/scleroderma overlap syndrome. They are also seen in patients with pulmonary hypertension. They show fine speckled nuclear and nucleolar staining on Hep2 immunofluorescence and are of little diagnostic value.
Jo-1 (Histidyl tRNA synthetase) antibodies are found in 20-40% of patients with aggressive polymyositis, usually in association with interstitial lung disease and arthralgia. Whilst the majority of Jo-1 antibodies will demonstrate a fine granular cytoplasmic pattern on IIF with Hep 2 cell substrate, up to 40% may be IIF negative.
PL-7 (Threonyl tRNA synthetase ) and PL-12 (Alanyl tRNA synthetase) belong to the same family of aminoacyl-tRNA synthetases (ARS) as Jo-1. Antibodies against ARS are associated with polymyositis and dermatomyositis, they are also seen in antisynthetase syndrome (ASS) recognized as a spectrum of myositis, interstitial pneumonia, non-erosive arthritis, fever and Raynaud's phenomena. PL-12 may be associated with lung disease in the absence of clinically apparent myositis
Anti-Ro52 are the most common ENA specificity amongst autoimmune diseases. They can be seen in Sj?gren's syndrome, SLE, cutaneous lupus erythematosus, neonatal lupus and primary biliary cirrhosis.
Signal recognition peptide (SRP) antibodies can be found in approximately 5% of polymyositis and dermatomyositis cases. They are also markers for necrotising myopathy which shows similar skin changes to dermatomyositis but has more acute symptoms including muscle pain/weakness and interstitial lung disease.
EJ (glycyl) and OJ (Isoleucyl) are markers for polymyositis and may be found in
Index no: QF/ND/IM/HB.028 Editor: Rebecca Haycox
Page 1 of 2
Version no. 1 Date of issue: May 2017
Clinical Specialist Services Care Group Department of Pathology - Immunology
Availability of testing Turnaround time
interstitial lung fibrosis, in overlap syndrome, arthritis and Raynaud's syndrome. EJ may also be observed in SLE. Routine referred: Protein Reference Unit, Sheffield
Approx. 21 working days
General Information
Optimal time and
Collect using aseptic technique
method of
collection
Volume / quantity
Minimum 1 mL or one full neonatal container
requirements
Transportation
Please see "Safe Working Practice for Courier/Taxi Drivers Carrying Pathological Specimens" (SF/PA/GP/004) and "Use and Maintenance of the Pneumatic Air Tube
System (PTS)" (LP/PA/GR/021)
Storage
Specimens that cannot be transported or processed immediately should be refrigerated (2-8?C).
Consent information
The trust consent policy requires that the patient has been informed of the possible use of their specimens by the pathology laboratories for quality control, education or research as part of the quality management system. If the patient declines use other
than for the tests specifically requested, please indicate by marking the box provided
on the front of the request form.
Information for
Please see NHS Choices website
patients
Index no: QF/ND/IM/HB.028 Editor: Rebecca Haycox
Page 2 of 2
Version no. 1 Date of issue: May 2017
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