Mixed Connective-Tissue Disease Differential Diagnoses

Updated: Sep 29, 2017
  • Author: Eric L Greidinger, MD; Chief Editor: Herbert S Diamond, MD  more...
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DDx

Diagnostic Considerations

Overlapping criteria for diagnosis of mixed connective tissue disease (MCTD) have been published by Sharp, Alarcón-Segovia, Kasukawa, and Kahn. A comparison study determined that MCTD was best identified with the Alarcón-Segovia criteria, which have 62.5% sensitivity and 86.2% specificity, and the Kahn criteria. [22]  

The Alarcón-Segovia diagnostic criteria consist of a positive anti–U1 RNP titer (>1:1600) and at least three of the following five clinical findings:

  • Hand edema
  • Synovitis
  • Biologically or histologically proven myositis
  • Raynaud phenomenon
  • Acrosclerosis with or without proximal systemic sclerosis

Other conditions to consider in the differential diagnosis of MCTD include the following:

  • Pleuritis
  • Respiratory distress syndrome
  • Stroke

Differential Diagnoses