Interstitial lung disorders are a group of respiratory diseases characterized by interstitial compartment infiltration, varying degrees of infiltration, and fibrosis, with or without small airway involvement. Although some are idiopathic (e.g., idiopathic pulmonary fibrosis, idiopathic interstitial pneumonias, and sarcoidosis), the great majority have an underlying etiology, such as systemic autoimmune rheumatic disease (SARD, also called Connective Tissue Diseases or CTD), inhalational exposure to organic matter, medications, and rarely, genetic disorders. This review focuses on diagnostic approaches in interstitial lung diseases associated with SARDs. To make an accurate diagnosis, a multidisciplinary, personalized approach is required, with input from various specialties, including pulmonary, rheumatology, radiology, and pathology, to reach a consensus. In a minority of patients, a definitive diagnosis cannot be established. Their clinical presentations and prognosis can be variable even within subsets of SARDs.
Keyphrases
- idiopathic pulmonary fibrosis
- end stage renal disease
- organic matter
- chronic kidney disease
- rheumatoid arthritis
- ejection fraction
- pulmonary hypertension
- gene expression
- high resolution
- squamous cell carcinoma
- clinical practice
- prognostic factors
- peritoneal dialysis
- mass spectrometry
- copy number
- systemic lupus erythematosus
- systemic sclerosis
- patient reported outcomes
- interstitial lung disease
- rectal cancer