Question

Difficulty: HardRheumatology and Immunology

Match each patient's clinical presentation with the most likely characteristic finding on muscle biopsy.

  • A 65-year-old man with insidious, asymmetric grip weakness, frequent tripping, and mild creatine kinase elevation.Endomysial inflammation with rimmed vacuoles containing amyloid-like material
  • A 45-year-old woman with progressive symmetric proximal weakness, a violaceous periorbital rash, and marked creatine kinase elevation.Perifascicular atrophy and perimysial inflammation with CD4+ T cells
  • A 50-year-old man with symmetric proximal muscle weakness, interstitial lung disease, and no cutaneous manifestations; anti-Jo-1 antibody is positive.Endomysial inflammation with CD8+ T cells invading non-necrotic muscle fibers
  • A 55-year-old woman with severe proximal muscle weakness persisting despite statin cessation; anti-HMG-CoA reductase antibodies are positive.Scattered necrotic and regenerating muscle fibers with minimal to absent inflammatory cell infiltrate

Answer

The correct matches align Inclusion Body Myositis with rimmed vacuoles, Dermatomyositis with perifascicular atrophy, Polymyositis with CD8+ T cell endomysial invasion, and Immune-Mediated Necrotizing Myopathy with necrosis lacking significant inflammation.
Each idiopathic inflammatory myopathy possesses distinct clinical and histopathologic features. Dermatomyositis is characterized by humoral-mediated perivascular inflammation resulting in perifascicular atrophy. Polymyositis involves direct T-cell cytotoxicity against muscle fibers, appearing as endomysial CD8+ cells. Inclusion body myositis uniquely presents in older males with asymmetric distal weakness and shows rimmed vacuoles. Immune-mediated necrotizing myopathy features profound myonecrosis with scant inflammation.

Step-by-Step Solution

1
Analyze the demographic and clinical features of each patient to identify the specific inflammatory myopathy.
Diagnoses established: left_1 is Inclusion Body Myositis (IBM), left_2 is Dermatomyositis (DM), left_3 is Polymyositis (PM), left_4 is Immune-Mediated Necrotizing Myopathy (IMNM).
Age, symmetry, distal vs. proximal involvement, and the presence or absence of skin findings/autoantibodies reliably differentiate the idiopathic inflammatory myopathies clinically.
2
Correlate each identified myopathy with its primary underlying immunopathologic mechanism.
DM is a humoral microangiopathy (perimysial), PM/IBM are cytotoxic T-cell processes (endomysial), and IMNM is an antibody-mediated necrotizing process.
Understanding the pathophysiology is essential to predicting the histologic appearance on biopsy.
3
Select the histologic description that matches the pathophysiology of the condition.
IBM matches rimmed vacuoles; DM matches perifascicular atrophy; PM matches CD8+ endomysial invasion; IMNM matches necrosis without inflammation.
Muscle biopsy remains the definitive gold standard for confirming specific subtypes of inflammatory myopathies.

Key Concept

Clinicopathologic correlation of idiopathic inflammatory myopathies
Estimated Time:2m 0s
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