The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months.

[7] Thirty to 40% of those with interstitial lung disease eventually develop pulmonary fibrosis which has a median survival of 2.5-3.5 years.

[8] Pulmonary function tests usually show a restrictive defect with decreased diffusion capacity of carbon monoxide (DLCO) indicating reduced alveolar to blood capillary transport.

[8] With advances in computed tomography, CT scans of the chest have supplanted lung biopsy as the preferred diagnostic test for ILD.

A bronchoscopic transbronchial cryobiopsy, in which a camera is introduced into the airways followed by rapid freezing of an area of lung tissue prior to biopsy is associated a lower complication rate and a much lower mortality rate compared to VATS or surgical biopsy with near comparable diagnostic accuracy.

[17][19] Radiologic appearance alone, however, is not adequate and should be interpreted in the clinical context, keeping in mind the temporal profile of the disease process.

For a limited number of cases, this is a definite advantage, as a precise molecular diagnosis can be done; frequently then there is no need for a lung biopsy.

[8] Long-term oxygen therapy can be beneficial to people with ILD and hypoxemia to enhance gas exchange, lessen dyspnea, and increase physical activity.

[21] Pulmonary rehabilitation appears to be useful with the benefits being sustainable longer term with improvements in exercise capacity (as measured by a six minute walking test), dyspnea, and quality of life.

[8] The antifibrotics pirfenidone and nintedanib have been shown to slow the decline in lung function (as measured by forced vital capacity [FVC]) in those with ILD compared to placebo.

[8] The immunomodulator tocilizumab has a benefit in scleroderma associated ILD by helping to preserve lung function (as measured by FVC) at 48 weeks.

[8] The immunomodulators cyclophosphamide, mycophenolate mofetil and rituximab all showed improved lung function (as measured by % predicted FVC) compared to placebo in systemic sclerosis or scleroderma associated ILD.

[8] The inhaled vasodilator treprostinil (a synthetic prostacyclin which acts as a prostaglandin I2 analogue) is indicated in the treatment of pulmonary hypertension secondary to interstitial lung disease and is associated with improved exercise capacity as measured by a 6-minute walk test.

The opiate agonist-antagonist nalbuphine and morphine are also known to improve coughing in those with ILD and other end stage lung diseases.