Type:
Educational Exhibit
Keywords:
Lung, CT-High Resolution, Education, Education and training
Authors:
C. Botía González1, L. Martinez Encarnacion1, Y. Martínez Paredes1, I. M. González Moreno1, A. barceló cárceles1, M. Lozano Ros1, E. M. M. Núñez Peynado2, I. Vicente Zapata1, A. García Chiclano1; 1Murcia/ES, 2Murcia, Murcia/ES
DOI:
10.26044/ecr2019/C-0354
Background
Idiopathic Pulmonary Fibrosis is a chronic,
progressive,
fibrotic interstitial lung disease of unknown cause.
If mainly affects older adults,
with male predominance,
and higher incidence North America and Europe (3 to 9 cases per 100,000 person-years) than in South America and East Asia (fewer than 4 cases per 100,000 person-years).
IPF is associated with the histopathologic or radiologic pattern of UIP.
Several environmental exposures that target the lung epithelium increase the risk of IPF,
with the most consistent evidence being for cigarette smoking.
An increased risk has also been linked to occupational exposures such as agriculture and farming,
livestock,
wood dust,
metal dust,
stone dust and silica.
Microaspirations of gastric content have also been suggested as another potential cause of injury to the lung epithelium in IPF.
In support of this notion,
the prevalence of gastro-oesophageal reflux is elevated in those with IPF.
The clinical presentation of these patients is unespecific,
but chronic exertional dyspnea and velcro-like crackles are almost universal.
Other signs and symptoms include chronic cough without purulence,
fatigue and clubbing.
The prognosis of this entity is por,
with a median survival of less than 5 years,
even though survival is quite variable from patient to patient .
In general,
older age,
male sex,
worse dyspnoea and greater physiological abnormality have been associated with impaired prognosis.
An early recongition and diagnosis of this condition are likely to improve outcomes through avoidance of potentially harmful therapies (e.g.,
glucocorticoids) and prompt initiation of new therapies that slow the progression of the disease.