How are PFTs and thoracic HRCT scan useful in the evaluation of patients with Systemic Sclerosis

How are PFTs and thoracic HRCT scan useful in the evaluation of patients with Systemic Sclerosis?

Serial FVC and DLco allow for objective quantification of ventilatory capacity and gas exchange, respectively. These parameters are useful in assessing the degree of respiratory impairment due to ILD and may provide clues about coexistent PAH as well (based on disproportionate or isolated reduction in the DLco). They are especially helpful when trying to assess for disease progression and response to therapy. Patients who decline ≥10% of predicted FVC or ≥15% of predicted DLco are considered to have progressive disease by PFT. In patients with SSc-ILD, pulmonary physiology is a stronger predictor of survival than underlying histopathologic pattern (FVC < 70%: 5-year survival 60%–65%).

Important information relevant to SSc-ILD can also be obtained by HRCT imaging, including the pattern and extent of disease, and on serial scanning, an assessment of disease progression. Patients with >20% of their lung affected by ILD on HRCT have a poor prognosis (5-year survival 60%).

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