In 2000, IPF was defined as a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults and limited to the lungs. Usual interstitial pneumonia (UIP) is the histopathological pattern of IPF. IPF is characterized by progressive worsening of dyspnea and lung function and is associated with a poor prognosis. Continue reading
Juvenile idiopathic arthritis is a heterogeneous group of diseases characterised by arthritis of unknown origin with onset before age of 16 years.
Bell’s palsy is a peripheral palsy of the facial nerve that results in muscle weakness on one side of the face.
Exclusion of other known causes of ILD such as certain drug toxicities, environmental exposures, and connective tissue diseases
Abnormal pulmonary function studies that include evidence of restriction (reduced VC, often with an increased FEV1/FVC ratio) and impaired gas exchange [increased P(A–a)O2, decreased PaO2 with rest or exercise or decreased DLCO]
Bibasilar reticular abnormalities with minimal ground glass opacities on HRCT scans
Transbronchial lung biopsy or BAL showing no features to support an alternative diagnosis