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Pathology - Polymyositis and Dermatomyositis
illnesses of the system with no recognized etiology, though immune pathways may be involved.
Occur most frequently in women between the ages of 40 and 60.
Polymyositis (P): Associated with necrotic muscle cells, endomysium-containing lymphocytic cells, minimal vascular injury, and CDS+ T-cell damage to myofibers.
The symptoms of dermatomyositis (D) include vascular endothelial fibrosis, atrophic and necrotic muscle fibers, and immune complex deposition in blood vessels with complement activation causing an inflammatory infiltration in perivascular areas and in perimysial connective tissue of muscle fibers.
Clinical Signs and Symptoms
Polymyositis: Interstitial lung disease, arthralgias, late muscular atrophy and contracture, symmetric, gradual proximal muscle weakening.
Skin-muscle inflammation: Pediorbital edema; purplish suffusion over eyelids (heliotrope rash); scaly patches over interphalangeal and MCP joints (Gottron papules); proximal muscle weakness; interstitial lung disease; cardiac arrhythmias or dilated cardiomyopathy; dysphagia; increased risk of underlying malignancy. Characteristic dusky red rash in malar distribution mimjcking SLE.
Research results for every myositide: increased levels of aldolase and creatine kinase, as well as positive ANA, anti-Jo-1, and anti-Mi-2 antibodies.
Handling
IVIG for severe, refractory cases; high-dose corticosteroids; immunosuppression with methotrexate or azathioprine.
illnesses of the system with no recognized etiology, though immune pathways may be involved.
Occur most frequently in women between the ages of 40 and 60.
Polymyositis (P): Associated with necrotic muscle cells, endomysium-containing lymphocytic cells, minimal vascular injury, and CDS+ T-cell damage to myofibers.
The symptoms of dermatomyositis (D) include vascular endothelial fibrosis, atrophic and necrotic muscle fibers, and immune complex deposition in blood vessels with complement activation causing an inflammatory infiltration in perivascular areas and in perimysial connective tissue of muscle fibers.
Clinical Signs and Symptoms
Polymyositis: Interstitial lung disease, arthralgias, late muscular atrophy and contracture, symmetric, gradual proximal muscle weakening.
Skin-muscle inflammation: Pediorbital edema; purplish suffusion over eyelids (heliotrope rash); scaly patches over interphalangeal and MCP joints (Gottron papules); proximal muscle weakness; interstitial lung disease; cardiac arrhythmias or dilated cardiomyopathy; dysphagia; increased risk of underlying malignancy. Characteristic dusky red rash in malar distribution mimjcking SLE.
Research results for every myositide: increased levels of aldolase and creatine kinase, as well as positive ANA, anti-Jo-1, and anti-Mi-2 antibodies.
Handling
IVIG for severe, refractory cases; high-dose corticosteroids; immunosuppression with methotrexate or azathioprine.
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