(Ref: Harrison 21st ed, p 2780; Harrison 20th ed, p 2555-56) Patients of scleroderma develop NSIP (Non-specific interstitial pneumonitis) and the extent of changes predict ILD progression and mortality. Option a, Cor pulmonale will occur in these patients due to PAH but findings of elevated JVP, Hepatomegaly and pedal edema are not described in the question. In textbook, Echocardiography is mentioned to be done every 6-12 months as follow up investigation and not for extent and subtype of disease. HRCT would be required to demonstrate the exact extent of interstitial changes seen on CXR and decide the site for Lung Biopsy which will confirm presence of NSIP. NSIP has a better prognosis as compared to other forms of ILD. Option c will show restrictive lung disease, but this finding is present in IPF, NSIP, UIP and does not help in differentiation between these subtypes. Option d is ruled out as vascular lesion is not evaluated.