Diagnostic approach of fibrosing interstitial lung diseases of unknown origin

Interstitial lung diseases encompass a broad range of numerous individual conditions, some of them characterized histologically by fibrosis, especially idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, chronic hypersensitivity pneumonia, interstitial lung disease associated with connective tissue diseases, and unclassifiable interstitial lung disease. The diagnostic approach relies mainly on the clinical evaluation, especially assessment of the patient's demographics, history, smoking habits, occupational or domestic exposures, use of drugs, and on interpretation of high-quality HRCT of the chest. Imaging is key to the initial diagnostic approach, and often can confirm a definite diagnosis, particularly a diagnosis of idiopathic pulmonary fibrosis when showing a pattern of usual interstitial pneumonia in the appropriate context. In other cases, chest HRCT may orientate toward an alternative diagnosis and appropriate investigations to confirm the suspected diagnosis. Autoimmune serology helps diagnosing connective disease. Indications for bronchoalveolar lavage and for lung biopsy progressively become more restrictive, with better considerations for their discriminate value, of the potential risk associated with the procedure, and of the anticipated impact on management. Innovative techniques and genetics are beginning to contribute to diagnosing interstitial lung disease and to be implemented routinely in the clinic. Multidisciplinary discussion, enabling interaction between pulmonologists, chest radiologists, pathologists and often other healthcare providers, allows integration of all information available. It increases the accuracy of diagnosis and prognosis prediction, proposes a first-choice diagnosis, may suggest additional investigations, and often informs the management. The concept of working diagnosis, which can be revised upon additional information being made available especially longitudinal disease behaviour, helps dealing with diagnostic uncertainty inherent to interstitial lung diseases and facilitates management decisions. Above all, the clinical approach and how thoroughly the patient's history and possible exposures are assessed determine the possibility of an accurate diagnosis.