New data has revealed some additional help that is available to patients with scarring diseases of the lung generally called interstitial lung disease or ILD. Patients with this disease may have it for a variety of reasons.

One expert article I reviewed suggested there were more than 200 potential causes for this type of lung disease.

Not all patients with an ILD need to be treated. However, there are some forms of this disease that can be rapidly progressive and result in marked physical limitations and early death.

One contributing cause to early debility and mortality in some patients with an ILD is a severe elevation in the pressure in the blood vessels of the lungs commonly referred to pulmonary hypertension. Pulmonary hypertension should not be confused with the more common systemic hypertension so prevalent in America. Pulmonary hypertension is specific to the lung’s blood vessels and may cause significant stress on the heart worsening the outcome for these patients.

Diagnosing pulmonary hypertension in ILD patients can be challenging and is usually inferred by certain laboratory and cardiac tests.

Ultimately, most patients require a right heart catherization to accurately diagnose this condition. The reason it is important to make this diagnosis is because there is now an approved treatment which can be very helpful. Prior to this this medication, treprostinil , previously had to be given by injection or a complicated oral program. This medication is now available by inhaler and may change the outlook for patients with ILD.