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Question

Which of the following features are associated with poor prognosis in Diffuse Cutaneous Systemic Sclerosis?
1. Older age
2. A high gas transfer factor for carbon monoxide (TLCO)
3. Proteinuria
4. Diffuse skin disease
Select the correct answer using the code given below:

The correct answer is
1, 3 and 4

Key Features of Poor Prognosis in Diffuse Cutaneous Systemic Sclerosis

Identifying factors associated with a poor prognosis in Diffuse Cutaneous Systemic Sclerosis (dcSSc) is crucial for patient management. Let's analyze the given features:

  • 1. Older age: Advanced age at diagnosis is frequently linked to more severe disease progression and poorer outcomes in various systemic conditions, including dcSSc.
  • 2. High gas transfer factor for carbon monoxide (TLCO): A high TLCO is generally indicative of preserved or even enhanced lung function. Conversely, a low TLCO is often associated with lung fibrosis, a common and serious complication in dcSSc, pointing towards a worse prognosis. Therefore, a high TLCO is typically not considered a feature of poor prognosis.
  • 3. Proteinuria: The presence of protein in the urine (proteinuria) signifies kidney involvement, often indicative of scleroderma renal crisis. Renal complications are a major cause of mortality and morbidity in dcSSc, making proteinuria a significant negative prognostic marker.
  • 4. Diffuse skin disease: The classification Diffuse Cutaneous Systemic Sclerosis itself implies widespread skin thickening. Compared to limited cutaneous scleroderma, the diffuse subtype is associated with earlier onset of skin thickening, a higher likelihood of internal organ involvement (including lungs, heart, and kidneys), and generally a more severe disease course and poorer prognosis.

Conclusion on Prognostic Factors

Based on the analysis, the features associated with poor prognosis in Diffuse Cutaneous Systemic Sclerosis are:

  • Older age (1)
  • Proteinuria (3)
  • Diffuse skin disease (4)

The high TLCO (2) is not typically associated with a poor prognosis.

Therefore, the correct combination is 1, 3, and 4.

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