A patient with limited systemic sclerosis for the past 10 years complaints of shoness of breath for the past one year. His pulmonary function tests are as follows – PFT OBSERVED PREDICTED FVC 2.63 2.82 FEV 1 88% 80% DLCO 5.2 16.3 Which among the following is the probable diagnosis
Correct Answer: Pulmonary hypeension
Description: Pulmonary aery hypeension The forced vital capacity (FVC) is not decreased in this case. The forced vital capacity in the question is FVC 93% is normal. - (Any pulmonary. function test which is 80-100% of the predicted value is considered normal) The FEV I is also mentioned as normal. So, the only pulmonary function abnormality is decrease in DLCO. This strongly suggests pulmonary aery hypeension. - Systemic sclerosis is associated with both pulmonary aery hypeension and interstitial lung disease. - They are quite difficult to distinguish clinically because pulmonary, function test are almost similar. - Both presents with restrictive pattern of disease and decrease in DLCO. Features of pulmonary function test which ours pulmonary aery hypeension are : -- Decrease in DLCO in the absence of any other abnormality on pulmonary. test i.e., isolated decrease in DLCO. - Decrease in DLCO is out of propoion to the decrease in FVC. If FVC/DLCO ratio > 1.5 ---> Pulmonary aery hypeension < 1.5 --> Interstitial lung disease. The definitive diagnosis of : ? Pulmonary aery hypeension --> Pulmonary aery catheterization. Interstitial lung disease --> Helical CT scan In the question the patient shows decrease in DLCO in the absence of any other abnormality. This strongly suggests pulmonary aery hypeension.
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