Presentation
Mild honeycombing, usually affecting < 10% of the lung, may be present. Routine laboratory tests are nonspecific and generally not helpful. [msdmanuals.com]
Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018; 27 (150): 180076. [ Links ] 76. Disayabutr S, Calfee CS, Collard HR, Wolters PJ. [scielo.cl]
Treatment
Treatment aims to keep the person alive until the disorder resolves. Mechanical ventilation is needed if there is respiratory failure. Corticosteroids are generally used, but it is not clear whether they are effective. [msdmanuals.com]
Rituximab for the treatment of connective tissue disease-associated interstitial lung disease. Sarcoidosis, Vasc Diffus lung Dis 2016; 32 (4): 296-304. [ Links ] 67. [scielo.cl]
Prognosis
Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage. Can Respir J 2018; 2018: 6043053. [ Links ] 14. Beasley MB, Franks TJ, Galvin JR, Gochuico B, Travis WD. [scielo.cl]
Epidemiology
The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev 2018; 27 (150): 180077. [ Links ] 19. Hutchinson J, Fogarty A, Hubbard R, McKeever T. [scielo.cl]