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Zorluk: ZorRheumatology and Immunology

In the evaluation of patients with suspected idiopathic inflammatory myopathies, specific autoantibody profiles offer critical prognostic information regarding disease progression, expected complications, and treatment response. Match each myositis-specific autoantibody to its most characteristic clinical phenotype.

  • Anti-Jo-1 (anti-histidyl-tRNA synthetase) antibodiesFever, non-erosive polyarthritis, interstitial lung disease, and hyperkeratotic fissured skin on the lateral aspects of the fingers
  • Anti-MDA5 (anti-melanoma differentiation-associated gene 5) antibodiesClinically amyopathic dermatomyositis with cutaneous ulcerations and rapidly progressive, potentially fatal interstitial lung disease
  • Anti-TIF1-gamma (anti-transcriptional intermediary factor 1-gamma) antibodiesClassic cutaneous signs of dermatomyositis with a significantly elevated risk of synchronous or metachronous internal malignancy
  • Anti-SRP (anti-signal recognition particle) antibodiesSevere, acute-onset immune-mediated necrotizing myopathy with profoundly elevated creatine kinase and frequent cardiac involvement

Cevap

Anti-Jo-1 pairs with antisynthetase syndrome (fever, arthritis, ILD, mechanic's hands). Anti-MDA5 pairs with amyopathic dermatomyositis and rapidly progressive ILD. Anti-TIF1-gamma pairs with malignancy-associated dermatomyositis. Anti-SRP pairs with severe immune-mediated necrotizing myopathy.
Each myositis-specific autoantibody is strongly linked to a distinct clinical phenotype that guides prognosis and management. Recognizing these associations is critical: Anti-Jo-1 defines antisynthetase syndrome; Anti-MDA5 flags life-threatening rapidly progressive ILD; Anti-TIF1-gamma warns of underlying malignancy; and Anti-SRP indicates severe immune-mediated necrotizing myopathy.

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1
Evaluate Anti-Jo-1
Matched to the phenotype with non-erosive polyarthritis, interstitial lung disease, and hyperkeratotic skin.
Anti-Jo-1 is the prototypical antibody for antisynthetase syndrome, classically presenting with 'mechanic's hands' and pulmonary involvement.
2
Evaluate Anti-MDA5
Matched to amyopathic dermatomyositis with rapid interstitial lung disease.
Anti-MDA5 is notorious for causing minimal muscle involvement but devastating, rapidly progressive interstitial lung disease and deep skin ulcers.
3
Evaluate Anti-TIF1-gamma
Matched to classic dermatomyositis with an elevated risk of internal malignancy.
The presence of Anti-TIF1-gamma is a major red flag for paraneoplastic dermatomyositis in adults, requiring extensive cancer screening.
4
Evaluate Anti-SRP
Matched to severe, acute-onset necrotizing myopathy.
Anti-SRP signifies a necrotizing process rather than classical inflammation, leading to massive CK elevations, severe weakness, and potential cardiac involvement.

Anahtar Kavram

Myositis-specific autoantibodies and their defining clinical phenotypes
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Konular

Rheumatology and Immunology

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