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Plain Films. Radiographs. PA and Lateral Views. CT Chest. Polymyositis Overlap Syndromes, Anti-Synthetase Syndrome. Epidemiology. DM/PM Syndrome affects mostly adults with a female:male ratio of 3:1

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Epidemiology
Epidemiology

  • DM/PM Syndrome affects mostly adults with a female:male ratio of 3:1

  • Relative prevalence exhibits regional latitude in Europe increasing from North (Iceland; RP:0.08) to South (Greece; RP:0.56) (Hengstman, van Venrooij et al. Ann. Rheum. Dis. ; 2000)

  • Jo-1 antibodies are correlated with HLA-DR3 in Caucasians

  • Prevalence of antisynthetase antibodies is 20-40% in patients with DM/PM


Pathophysiology
Pathophysiology

  • Thought to have precedent viral exposure leading to persistent presence of auto-antigens (molecular mimicry)

  • UV-B is thought to play a role given prevalence in European countries along latitude.

    • UV-B stimulates dermal expression of TNF-alpha and other type-1 cytokines

  • Spectrum includes both humoral mediated (DM) and cell mediated (CD8+ cytotoxic cells in PM) mechanisms

  • Due to these different mechanisms, both DM, PM, and the overlap syndromes have differing responses to anti-inflammatory agents


Clinical presentation
Clinical Presentation

  • Features

    • Raynaud’s

    • Proximal muscle weakness vs. combined proximal and distal weakness in inclusion body myositis

    • Photodermatitis

    • Mechanic’s hands

    • Elevated inflammatory markers, variable elevations in CK

    • Capillary dilation with periungal inflammation

    • May have amyopathic disease

    • Subcutaneous calcinosis (anti-synthetase syndrome)

    • Interstitial Lung Disease (anti-synthetase syndrome)

    • Symmetric, deforming (but not erosive) polyarthritis (anti-synthetase syndrome)


Physical exam findings
Physical Exam Findings

Adapted from Targoff et al


Physical exam findings1
Physical Exam Findings

Adapted from Targoff et al


Physical exam findings2
Physical Exam Findings

Adapted from Targoff et al


Autoantibodies
Autoantibodies

  • Note that 40% of these DM/PM/Overlap patients have detectable antibodies. Clinical presentation and muscle biopsy then becomes paramount.

  • + ANA in 80% of cases, don’t discount a negative ANA


Ana patterns
ANA Patterns

  • + ANA, Speckled pattern. DM/PM (anti-Mi2 and SRP antibodies)

  • + ANA, nucleolar pattern. Overlap of PM with Scleroderma (anti-PM-Scl)

  • Negative ANA, cytoplasmic pattern. Anti-synthetase syndromes


Anti synthetase syndrome
Anti-Synthetase Syndrome

  • Anti-Synthetase antibodies

    • These autoantibodies are directed towards aminoacyl-tRNA synthetases (responsible for transfer of AA to conjugate transfer RNAs)

    • These are found in the cytoplasm, therefore ANA stains are cytoplasmic and are thus reported as negative (20% of all inflammatory myopathies)


Anti synthetase syndrome1
Anti-Synthetase Syndrome

  • Clinical Manifestation

    • Patients with antisynthetase syndrome typically present with two or more components of myositis, ILD, and joint involvement

    • ILD may occur in absence of myositis, particularly associated with PL-12 autoantibodies (Friedman, Targoff, Arnett. Semin Arthritis Rheum; 1996)


Treatment
Treatment

  • Physical therapy and an active exercise regimen

  • Corticosteroids 1mg/kg/day with good success (remission rates of 25-68% achieved)

  • Non-steroidals (methotrexate .3mg/kg/week, azathioprine 2.5mg/kg/day, cyclophosphamide 3mg/kg/day) used with mild-moderate success

  • Use IVIG in refractory cases

  • Case reports describe use of Rituximab in refractory cases


Considerations
Considerations

  • In older patients, consider occult malignancy workup

  • Non-Hodgkin’s Lymphoma associated with DM/PM

  • May order DEXA scan given prolonged course of corticosteroids

  • Complications can include steroid induced myopathy (progressive weakness with improved CK)

  • Most common cause of mortality = complications from ILD leading to pulmonary HTN and hypoxia



References
References

  • Hengstman GJD, van Venrooij WJ, Vencovsky J, Moutsopoulos HM, van Engelen BGM. The relative prevalence of dermatomyositis and polymyositis in Europe exhibits a latitudinal gradient. Ann. Rheum. Dis. 59:141-142; 2000.

  • Plotz PH, Targoff I. Myositis associated antigens. Aminoacyl-tRNA synthetases. In : Manual of Biological Markers of Disease. Eds,WJ Van venrooij and R Maini. Kluwer Academic Publications. The Netherlands. pp B6.1: 1-18; 1994.

  • Love LA, Leff RL, Fraser DD, Targoff IN, Dalakas M, Plotz PH, Miller FW. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine (Baltimore) 70:360-74; 1991.

  • Friedman AW, Targoff IN, Arnett FC. Interstitial lung disease with autoantibodies against aminoacyl-tRNA synthetases in the absence of clinically apparent myositis. Semin Arthritis Rheum 26:459-67; 1996.

  • Grathwohl KW, Thompson JW, Riordan KK, Roth BJ, Dillard TA. Digital clubbing associated with polymyositis and interstitial lung disease. Chest  108:1751-2; 1995.

  • Kalenian M, Zweiman B Inflammatory myopathy, bronchiolitis obliterans/organizing pneumonia, and anti-Jo-1 antibodies: an interesting association. Clin Diagn Lab Immunol 4:236-40;1997.

  • Targoff IN, Arnett FC Clinical manifestations in patients with antibody to PL-12 antigen (alanyl-tRNA synthetase). Am J Med 88:241-51; 1990.

  • Lee W, Zimmermann B 3rd, Lally EV.Relapse of polymyositis after prolonged remission. J.Rheumatol 24:1641-4; 1997.

  • Dalakas MC. Current treatment of the inflammatory myopathies. Curr Opin Rheumatol  6:595-601; 1994.


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