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The Pathogenesis of Dermatomyositis

Robert C. Griggs, MD; George Karpati, MD
Arch Neurol. 1991;48(1):21-22. doi:10.1001/archneur.1991.00530130029013
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Considerable progress has been made in the understanding of dermatomyositis. Banker and Victor1 characterized childhood dermatomyositis as an angiopathy in 1966. Banker then identified distinctive capillary endothelial changes and postulated that these were an early and fundamental lesion in dermatomyositis.2 Subsequently, Carpenter et al3 showed endomysial capillary necrosis and significantly reduced capillary numbers relative to muscle fiber area, providing substantive support for muscle fiber atrophy being a sequel of chronic sublethal ischemia.

See also p 26.

In this issue of the Archives, Kissel et al4 provide still more evidence for the primacy of blood-vessel damage as an early pathologic feature of dermatomyositis. The report by Kissel et al5 followed up on an earlier study from the same group, which showed that membrane attack complex (MAC) was present on muscle biopsy in blood vessels of patients with dermatomyositis. The present study used quantitative histologic methods, combined

REFERENCES

Banker BQ, Victor M.  Dermatomyositis (systemic angiopathy) of childhood . Medicine . 1966;;45:261-289.
Banker BQ.  Dermatomyositis of childhood . J Neuropathol Exp Neurol . 1975;;34:46-75.
Carpenter S, Karpati G, Rothman S, Watters G.  The childhood type of dermatomyositis . Neurology . 1976;;26:952-962.
Kissel JT, Halterman RK, Rammohan KW, Mendell JR.  The relationship of complement-mediated microvasculopathy to the histologic features and clinical duration of disease in dermatomyositis . Arch Neurol . 1991;;48:26-30.
Kissel JT, Mendell JR, Rammohan KW.  Microvascular deposition of complement membrane attack complex in dermatomyositis . N Engl J Med . 1986;;314:329-334.
Carpenter S, Karpati G. Pathology of Skeletal Muscle . New York, NY: Churchill Livingstone Inc; 1984;.
Whitaker JN.  Inflammatory myopathy: a review of etiologic and pathogenetic factors . Muscle Nerve . 1982;;5:573-592.
Whitaker JN, Engel WK.  Vascular deposits of immunoglobulin and complement in idiopathic inflammatory myopathy . N Engl J Med . 1972;;286:333-338.
Kolb WP, Muller-Eberhard HJ.  Neoantigens of the membrane attack complex of human complement . Proc Natl Acad Sci USA . 1975;;72:1687-1689.
Engel AG, Bisecker G.  Complement activation in muscle fiber necrosis: demonstration of the membrane attack complex of complement in necrotic fibers . Ann Neurol . 1982;;12:289-296.
Emslie-Smith AM, Engel AG.  Microvascular changes in early and advanced dermatomyositis: a quantitative study . Ann Neurol . 1990;;27:343-356.
Carry MR, Ringel SP, Starcevich JM.  Distribution of capillaries in normal and diseased human skeletal muscle . Muscle Nerve . 1986;;9:445-454.
Pangburn MK, Muller-Eberhard HJ.  The alternative pathway of complement . Semin Immunopathol . 1984;;7:163-192.
Podack ER, Muller-Eberhard HJ.  Isolation of human S protein, an inhibitor of the membrane attack complex of complement . J Biol Chem . 1979;;254:9908-9914.
Iida K, Mornaghi R, Nussenzweig V.  Complement receptor CR-1 deficiency in erythrocytes from patients with systemic lupus erythematosus . J Exp Med . 1982;;155:1427-1438.
Falk RJ, Dalmasso AP, Kim Y, Lam S, Michael A.  Radioimmunoassay of the attack complex of complement in serum from patients with systemic lupus erythematosus . N Engl J Med . 1985;;312:1594-1599.
Gamboa ET, Eastwood AB, Hays AP, Maxwell J, Penn AS.  Isolation of influenza virus from muscle in myoglobinuric polymyositis . Neurology . 1979;;29:1323-1325.
Rosenberg NL, Rotbart HA, Abzug MJ, Ringel SP, Levin MJ.  Evidence for a novel picornavirus in human dermatomyositis . Ann Neurol . 1989;;26:204-209.
Karpati G, Carpenter S.  Idiopathic inflammatory myopathies . Curr Opin Neurol Neurosurg . 1989;;1:806-814.
Silver RM, Maricq HR.  Childhood dermatomyositis: serial microvascular studies . Pediatrics . 1989;;83:278-283.
Yunis EJ, Samaha FJ.  Inclusion body myositis . Lab Invest . 1971;;25:240-248.
Carpenter S, Karpati G, Heller I, Eisen A.  Inclusion body myositis: a distinct variety of idiopathic inflammatory myopathy . Neurology . 1978;;28:8-17.
Arahata K, Engel AG.  Monoclonal antibody analysis of mononuclear cells in myopathies, IV: cell-mediated cytotoxicity and muscle fiber necrosis . Ann Neurol . 1988;;23:168-173.
Karpati G, Pouliot Y, Carpenter S.  Expression of immunoreactive major histocompatibility complex products in human skeletal muscles . Ann Neurol . 1988;;23:64-72.
Love LA.  Causes of idiopathic inflammatory myopathy. In: Plotz PH, moderator. Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders . Ann Intern Med . 1989;;111:148-150.
Miller FW, Twitty SA, Biswas T, Plotz PH.  Origin and regulation of a disease-specific autoantibody response . J Clin Invest . 1990;;85:468-475.
Cronin ME.  Treatment. In: Plotz PH, moderator. Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders . Ann Intern Med . 1989;;111:153-155.
Mathieson PW, Cobbold SP, Hale G, et al.  Monoclonal antibody therapy in systemic vasculitis . N Engl J Med . 1990;;323:250-254.

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Banker BQ, Victor M.  Dermatomyositis (systemic angiopathy) of childhood . Medicine . 1966;;45:261-289.
Banker BQ.  Dermatomyositis of childhood . J Neuropathol Exp Neurol . 1975;;34:46-75.
Carpenter S, Karpati G, Rothman S, Watters G.  The childhood type of dermatomyositis . Neurology . 1976;;26:952-962.
Kissel JT, Halterman RK, Rammohan KW, Mendell JR.  The relationship of complement-mediated microvasculopathy to the histologic features and clinical duration of disease in dermatomyositis . Arch Neurol . 1991;;48:26-30.
Kissel JT, Mendell JR, Rammohan KW.  Microvascular deposition of complement membrane attack complex in dermatomyositis . N Engl J Med . 1986;;314:329-334.
Carpenter S, Karpati G. Pathology of Skeletal Muscle . New York, NY: Churchill Livingstone Inc; 1984;.
Whitaker JN.  Inflammatory myopathy: a review of etiologic and pathogenetic factors . Muscle Nerve . 1982;;5:573-592.
Whitaker JN, Engel WK.  Vascular deposits of immunoglobulin and complement in idiopathic inflammatory myopathy . N Engl J Med . 1972;;286:333-338.
Kolb WP, Muller-Eberhard HJ.  Neoantigens of the membrane attack complex of human complement . Proc Natl Acad Sci USA . 1975;;72:1687-1689.
Engel AG, Bisecker G.  Complement activation in muscle fiber necrosis: demonstration of the membrane attack complex of complement in necrotic fibers . Ann Neurol . 1982;;12:289-296.
Emslie-Smith AM, Engel AG.  Microvascular changes in early and advanced dermatomyositis: a quantitative study . Ann Neurol . 1990;;27:343-356.
Carry MR, Ringel SP, Starcevich JM.  Distribution of capillaries in normal and diseased human skeletal muscle . Muscle Nerve . 1986;;9:445-454.
Pangburn MK, Muller-Eberhard HJ.  The alternative pathway of complement . Semin Immunopathol . 1984;;7:163-192.
Podack ER, Muller-Eberhard HJ.  Isolation of human S protein, an inhibitor of the membrane attack complex of complement . J Biol Chem . 1979;;254:9908-9914.
Iida K, Mornaghi R, Nussenzweig V.  Complement receptor CR-1 deficiency in erythrocytes from patients with systemic lupus erythematosus . J Exp Med . 1982;;155:1427-1438.
Falk RJ, Dalmasso AP, Kim Y, Lam S, Michael A.  Radioimmunoassay of the attack complex of complement in serum from patients with systemic lupus erythematosus . N Engl J Med . 1985;;312:1594-1599.
Gamboa ET, Eastwood AB, Hays AP, Maxwell J, Penn AS.  Isolation of influenza virus from muscle in myoglobinuric polymyositis . Neurology . 1979;;29:1323-1325.
Rosenberg NL, Rotbart HA, Abzug MJ, Ringel SP, Levin MJ.  Evidence for a novel picornavirus in human dermatomyositis . Ann Neurol . 1989;;26:204-209.
Karpati G, Carpenter S.  Idiopathic inflammatory myopathies . Curr Opin Neurol Neurosurg . 1989;;1:806-814.
Silver RM, Maricq HR.  Childhood dermatomyositis: serial microvascular studies . Pediatrics . 1989;;83:278-283.
Yunis EJ, Samaha FJ.  Inclusion body myositis . Lab Invest . 1971;;25:240-248.
Carpenter S, Karpati G, Heller I, Eisen A.  Inclusion body myositis: a distinct variety of idiopathic inflammatory myopathy . Neurology . 1978;;28:8-17.
Arahata K, Engel AG.  Monoclonal antibody analysis of mononuclear cells in myopathies, IV: cell-mediated cytotoxicity and muscle fiber necrosis . Ann Neurol . 1988;;23:168-173.
Karpati G, Pouliot Y, Carpenter S.  Expression of immunoreactive major histocompatibility complex products in human skeletal muscles . Ann Neurol . 1988;;23:64-72.
Love LA.  Causes of idiopathic inflammatory myopathy. In: Plotz PH, moderator. Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders . Ann Intern Med . 1989;;111:148-150.
Miller FW, Twitty SA, Biswas T, Plotz PH.  Origin and regulation of a disease-specific autoantibody response . J Clin Invest . 1990;;85:468-475.
Cronin ME.  Treatment. In: Plotz PH, moderator. Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders . Ann Intern Med . 1989;;111:153-155.
Mathieson PW, Cobbold SP, Hale G, et al.  Monoclonal antibody therapy in systemic vasculitis . N Engl J Med . 1990;;323:250-254.

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