Pulmonary Fibrosis, Idiopathic

Present in ∼42.7–63:100,000 and incidence is ∼16.3:100,000 in USA; more common with increasing age, with majority >55 y.

Occurrence higher in males than females; risk factors include smoking, exposures, increasing age, family history, chronic reflux, environmental, viral, and genetics.

Dependent on degree of underlying lung disease and associated comorbidities.

Pulm Htn, H/O PE, OSA, CKD, CAD, and NYHA class 2 or above are all associated with increasing periop risk for pulmonary failure.

Obesity (which further reduces lung volumes and worsens ventilation-perfusion mismatch), hypercarbia, and respiratory failure.

Preop functional status and exercise capacity; risks include albumin <3.5 mg/dL, smoking, COPD, asthma, and FEV ₁ <80%.

Neurologic impairment and immunosuppressive therapy.

ABG (PaO ₂ /FiO ₂ , 225 mm Hg).

Type of surgery and location (proximity to the diaphragm), especially thoracic surgery for cancer or lung biopsy, along with anesthetic type and surgical duration (>3–4 h).

Progressive respiratory failure, pulm Htn, and potential RV failure

Postop pneumonia and respiratory failure; prolonged ventilation

Increased morbidity and mortality, especially in high-risk pts undergoing thoracic procedures

IPF, also known as cryptogenic fibrosing alveolitis, is a chronic and progressive fibrosing interstitial pneumonia of unclear etiology.

Natural history reveals untreated IPF to have an unpredictable course with an insidious progressive nature and deterioration in physical function and capacity, along with a decline in FVC.

Pts may also present with acute decompensation.

Prognosis is poor and associated with ∼25% survival at 5 y from time of diagnosis and median survival ∼3 y from time of diagnosis.

Morbidity and mortality can occur in approximately ∼3–4% following lobectomy and ∼11.6% following pneumonectomy.

Unclear, but possible association with smoking, chronic aspiration, and viral infection.

Important to rule out common misdiagnoses of interstitial lung diseases (e.g., infectious, drug related, exposures [specifically asbestos], hypersensitivity pneumonitis, rheumatoid arthritis, and systemic sclerosis, and usual interstitial pneumonia).

Clinical course, presentation, and severity variable from pt to pt.