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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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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

  • 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

  • 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

  • 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

Adapted from Targoff et al


Physical Exam Findings

Adapted from Targoff et al


Physical Exam Findings

Adapted from Targoff et al


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, 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 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 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

  • 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

  • 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


Gratuitous photos


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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