INDIVIDUAL TEST 548
Myositis/ Interstitial Lung Disease (ILD) Screen
Indication
Suspicion of polymyositis (PM), dermatomyositis (DM) or overlap syndrome
Sample material
Serum
- Minim. volume: 0,5 mL
Transport
Within Sweden
- room temperature
International
- room temperature
Method
Immunoblot
Reference interval
Mi-2α, Mi-2β, Ku, SRP, Jo-1, PL-7, PL-12, EJ, OJ, PM-Scl75, PM-Scl100, NXP2, Ro52, och SAE1: ≤10 negative MDA5 och TIF1γ: ≤5 negative (6-10 borderline)
Result
Results are reported as negative, borderline, or positive with reaction intensity.
Interpretation
PL-12
Anti-PL-12 is a type of anti-synthetase antibody and can be detected in <5% of patients with inflammatory myositis. Anti-PL-12 is often (90%) associated with interstitial lung disease that may occur with or without myositis.
PL-7
Anti-PL-7 is a type of anti-synthetase antibody and can be detected in <5% of patients with inflammatory myositis. Anti-PL-7 is often associated with interstitial lung disease that may occur with or without myositis.
Ro52
Anti-Ro52 can often be detected in myositis with or without other myositis-associated antibodies and are associated with an increased risk of interstitial lung disease. Anti-Ro52 is also associated with several other autoimmune conditions such as rheumatic diseases and autoimmune liver disease.
Jo-1
Anti-Jo1 is the most common type of anti-synthetase antibodies and is detected in up to 20-30% of patients with inflammatory myositis. Interstitial lung disease is often also seen.
EJ
Anti-EJ are a type of anti-synthetase antibodies and can be detected in <5% of the patients with inflammatory myositis.
OJ
Anti-OJ are a type of anti-synthetase antibodies and can be detected in <5% of the patients with inflammatory myositis. Anti-OJ is strongly associated with interstitial lung disease.
Ku
Anti-Ku is associated with myositis that clinically overlaps with other rheumatic systemic diseases and can in some cases also be detected in SLE, Sjögren's syndrome, idiopathic pulmonary fibrosis and inflammatory myositis. Anti-Ku is often detected together with antibodies against SSA/Ro52.
PM-Scl75
Antibodies against the PM-Scl complex are associated with myositis that clinically overlaps with other rheumatic systemic diseases. The antibodies are usually associated with positive nucleolar ANA and are usually directed against the 75 kD and 100 kD subunits. Isolated occurrence of antibodies against PM-Scl75, i.e. without the presence of anti-PM-Scl100, has a significantly lower diagnostic value and can also be seen in healthy people.
PM-Scl100
Antibodies against the PM-Scl complex are associated with myositis that clinically overlap with other rheumatic systemic diseases and are often associated with Raynaud's phenomenon, arthritis, muscle involvement and effects on the skin and lungs. Usually, positive ANA with a nucleolar pattern is also seen. The antibodies are usually directed against the subunits 75 kD and 100 kD.
SRP
Anti-SRP is a strong marker for severe and rapidly progressing necrotizing myositis that often responds poorly to treatment. Cardiac involvement may occur. The level of anti-SRP levels correlates to disease activity. Dysphagia may occur. Anti-SRPs are rarely associated with interstitial lung disease or malignancy.
Mi-2α and Mi-2β
Antibodies against Mi-2 are associated with classic dermatomyositis, which often has a mild course and responds well to treatment. Low risk of pulmonary involvement or malignancy. The antibodies can target both the alpha- and beta-chains of Mi-2. Isolated occurrence of antibodies against Mi-2 beta has low diagnostic value and can also be seen in healthy individuals.
TIF1γ (p155/140)
Antibodies against TIF1γ are associated with dermatomyositis and in adults (but rarely in children) strongly associated with malignancy, but not with interstitial lung disease. In children, anti-TIF1γ is linked to dermatomyositis without association with malignancy.
NXP2 (MJ)
Antibodies against NXP2 can be detected in patients with juvenile dermatomyositis and are associated with a severe course of pronounced muscle involvement (atrophy, contractures). Calcinosis, ulcerations and intestinal vasculitis also occur. Antibodies against NXP2 are associated with an increased risk of underlying malignancy in adults.
SAE1 (SUMO-1)
Antibodies against "small ubiquitin-like modifier activating enzyme" is a rare antibody in adults with dermatomyositis where the skin symptoms often precede the development of myositis. Interstitial lung disease can also occur. Anti-SAE is associated with malignancy.
MDA5
Antibodies against melanoma-differentiation-associated gene 5 (anti-MDA5) are strongly associated with severe and rapidly progressing interstitial lung disease in dermatomyositis where muscle weakness may be mild and CK (creatine kinase) normal (amyopathic dermatomyositis). Borderline values may also be of clinical significance. Anti-MDA5 is not associated with malignancy.
References
- Satoh M. et al. Clinic Rev Allerg Immunol. 2017. A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy. PMID: 26424665
- Fujimoto M et al. Curr Opin Rheumatol. 2016. Recent advances in dermatomyositis-specific autoantibodies. PMID: 27533321
- Nakashima R et al. Lupus. 2016. Clinical significance and new detection system of autoantibodies in myositis with interstitial lung disease. PMID: 27252271
- Trallero Arguas E et al. Semin Arthritis Rheum. 2016. Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group. PMID: 27139168
- Allenbach Y, Benveniste O. Journal of Neuromuscular Diseases 2. 2015. Diagnostic Utility of Auto-Antibodies in Inflammatory Muscle Diseases. PMID: 28198709
- Ghirardello A et al. Autoimmun Highlights. 2014. Myositis autoantibodies and clinical phenotypes. PMID: 26000158
- Beneviste et al. Arthritis Rheum. 2011. Correlation of anti-signal recognition particle autoantibody levels with creatine kinase activity in patients with necrotizing myopathy. PMID: 21400483
- Belizna C et al. Autoimmunity Reviews. 2010. Anti-Ku antibodies: Clinical, genetic and diagnostic insights. PMID: 20621654
- Gunawardena H, Betteridge ZE, McHugh NJ. Rheumatology. 2009. Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression. PMID: 19439503
- Mahler M et al. Autoimmunity Reviews. 2007. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. PMID: 17643929
- Lundberg IE et al. Ann Rheum Dis. 2017. European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. PMID: 29079590
- Allenbach Y et al. Neuromuscul Disord. 2018. 224th ENMC International Workshop: Clinico-sero-pathological classification of immune-mediated necrotizing myopathies Zandvoort, The Netherlands, 14–16 October 2016. PMID: 29221629
- Lundberg IE et al. Nat Rev Dis Primers. 2021. Idiopathic inflammatory myopathies. PMID: 34857780
Last updated: 2025-03-18
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ENSKILD ANALYS 548
Myosit/Interstitiell lungsjukdom (ILD) Screen
Indikation
Vid misstanke om polymyosit (PM), dermatomyosit (DM) eller overlap syndrom
Provmaterial
Serum
- Minim. volym: 0,5 mL
Transport
Inom Sverige
- rumstemperatur
Internationellt
- rumstemperatur
Metod
Immunoblot
Referensintervall
Mi-2α, Mi-2β, Ku, SRP, Jo-1, PL-7, PL-12, EJ, OJ, PM-Scl75, PM-Scl100, NXP2, Ro52, och SAE1: ≤10 negativt MDA5 och TIF1γ: ≤5 negativt (6-10 gränsvärde)
Resultat
Resultat anges som negativt, gränsvärde eller positivt med med intensitet på reaktionen.
Tolkning
PL-12
Anti-PL-12 är en typ av anti-syntetasantikroppar och kan påvisas hos <5% av patienter med inflammatorisk myosit. Anti-PL-12 är ofta (90%) associerat med interstitiell lungsjukdom som kan förekomma med eller utan myosit.
PL-7
Anti-PL-7 är en typ av anti-syntetasantikroppar och kan påvisas hos <5% av patienterna med inflammatorisk myosit. Anti-PL-7 är ofta associerade med interstitiell lungsjukdom som kan förekomma med eller utan myosit.
Ro52
Anti-Ro52 kan ofta påvisas vid myosit med eller utan andra myositassocierade antikroppar och är associerad med ökad risk för interstitiell lungsjukdom. Anti-Ro52 är även associerade med ett flertal andra autoimmuna tillstånd som reumatiska sjukdomar och autoimmun leversjukdom.
Jo-1
Anti-Jo1 är den vanligaste typen av anti-syntetasantikroppar och påvisas hos upp till 20-30% av patienter med inflammatorisk myosit. Ofta ses även interstitiell lungsjukdom.
EJ
Anti-EJ är en typ av anti-syntetasantikroppar och kan påvisas hos <5% av patienterna med inflammatorisk myosit.
OJ
Anti-OJ är en typ av anti-syntetasantikroppar och kan påvisas hos <5% av patienter med inflammatorisk myosit. Anti-OJ är starkt associerat med interstitiell lungsjukdom.
Ku
Anti-Ku är associerade med myosit som kliniskt överlappar med andra reumatiska systemsjukdomar och kan i enstaka fall även påvisas vid SLE, Sjögrens syndrom, idiopatisk lungfibros och inflammatorisk myosit. Anti-Ku påvisas ofta tillsammans med antikroppar mot SSA/Ro52.
PM-Scl75
Antikroppar mot PM-Scl-komplexet är associerade med myosit som kliniskt överlappar med andra reumatiska systemsjukdomar. Antikropparna är vanligtvis associerade med positiv nukleolär ANA och är vanligen riktade mot subenheterna 75 kD och 100 kD. Isolerad förekomst av antikroppar mot PM-Scl75, dvs utan förkomst av anti-PM-Scl100, har betydligt lägre diagnostiskt värde och kan även ses hos friska individer.
PM-Scl100
Antikroppar mot PM-Scl-komplexet är associerade med myosit som kliniskt överlappar med andra reumatiska systemsjukdomar och ofta associerade med Raynaud-fenomen, artrit, muskelpåverkan och påverkan på hud och lungor. Vanligtvis ses även positiv ANA med nukleolärt mönster. Antikropparna är vanligen riktade mot subenheterna 75 kD och 100 kD.
SRP
Anti-SRP är en stark markör för svår och snabbt förlöpande nekrotiserande myosit som ofta svarar dåligt på behandling. Hjärtengagemang kan förekomma. Nivån av anti-SRP-nivåerna korrelerar till sjukdomsaktivitet. Dysfagi kan förekomma. Anti-SRP är sällan associerade med interstitiell lungsjukdom eller malignitet.
Mi-2α och Mi-2β
Antikroppar mot Mi-2 är associerade med klassisk dermatomyosit som ofta har ett milt förlopp och svarar bra på behandling. Låg risk för lungengagemang eller malignitet. Antikropparna kan vara riktade mot både alfa- och betakedjan av Mi-2. Isolerad förekomst av antikroppar mot Mi-2 beta har lågt diagnostiskt värde och kan även ses hos friska individer.
TIF1γ (p155/140)
Antikroppar mot TIF1γ är associerade med dermatomyosit och hos vuxna (men sällan hos barn) starkt kopplade till malignitet, men inte till interstitiell lungsjukdom. Hos barn är anti-TIF1γ kopplat till dermatomyosit utan association till malignitet.
NXP2 (MJ)
Antikroppar mot NXP2 kan påvisas hos patienter med juvenil dermatomyosit och är kopplade till ett allvarligt förlopp med uttalad muskelpåverkan (atrofi, kontrakturer). Även kalcinos, ulcerationer och intestinal vaskulit förekommer. Antikroppar mot NXP2 är hos vuxna associerade med ökad risk för bakomliggande malignitet.
SAE1 (SUMO-1)
Antikroppar mot ”small ubiquitin-like modifier activating enzyme” är en ovanlig antikropp hos vuxna med dermatomyosit där hudsymptomen ofta föregår utveckling av myosit. Även interstitiell lungsjukdom kan förekomma. Anti-SAE är associerad med malignitet.
MDA5
Antikroppar mot melanoma-differentiation-associated gene 5 (anti-MDA5) är starkt associerade med allvarlig och snabbt progredierande interstitiell lungsjukdom vid dermatomyosit där muskelsvagheten kan vara mild och CK (creatine kinase) normalt (amyopatisk dermatomyosit). Även gränsvärden kan vara av klinisk betydelse. Anti-MDA5 är inte associerade med malignitet.
Referenser
- Satoh M. et al. Clinic Rev Allerg Immunol. 2017. A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy. PMID: 26424665
- Fujimoto M et al. Curr Opin Rheumatol. 2016. Recent advances in dermatomyositis-specific autoantibodies. PMID: 27533321
- Nakashima R et al. Lupus. 2016. Clinical significance and new detection system of autoantibodies in myositis with interstitial lung disease. PMID: 27252271
- Trallero Arguas E et al. Semin Arthritis Rheum. 2016. Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group. PMID: 27139168
- Allenbach Y, Benveniste O. Journal of Neuromuscular Diseases 2. 2015. Diagnostic Utility of Auto-Antibodies in Inflammatory Muscle Diseases. PMID: 28198709
- Ghirardello A et al. Autoimmun Highlights. 2014. Myositis autoantibodies and clinical phenotypes. PMID: 26000158
- Beneviste et al. Arthritis Rheum. 2011. Correlation of anti-signal recognition particle autoantibody levels with creatine kinase activity in patients with necrotizing myopathy. PMID: 21400483
- Belizna C et al. Autoimmunity Reviews. 2010. Anti-Ku antibodies: Clinical, genetic and diagnostic insights. PMID: 20621654
- Gunawardena H, Betteridge ZE, McHugh NJ. Rheumatology. 2009. Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression. PMID: 19439503
- Mahler M et al. Autoimmunity Reviews. 2007. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. PMID: 17643929
- Lundberg IE et al. Ann Rheum Dis. 2017. European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. PMID: 29079590
- Allenbach Y et al. Neuromuscul Disord. 2018. 224th ENMC International Workshop: Clinico-sero-pathological classification of immune-mediated necrotizing myopathies Zandvoort, The Netherlands, 14–16 October 2016. PMID: 29221629
- Lundberg IE et al. Nat Rev Dis Primers. 2021. Idiopathic inflammatory myopathies. PMID: 34857780
Senast uppdaterat: 2025-03-18