Pulmonary Disease and Critical Care· Topic 16.4 Interstitial lung disease· 16.4.1

Idiopathic pulmonary fibrosis (IPF)

Idiopathic pulmonary fibrosis is a chronic, progressive fibrosing lung disease of unknown cause defined by a usual interstitial pneumonia (UIP) pattern, presenting with progressive dyspnea, dry cough, bibasilar Velcro crackles, and clubbing in older smokers. A definite UIP pattern on high-resolution CT confirms the diagnosis without biopsy; management centers on antifibrotic therapy (pirfenidone or nintedanib), oxygen, and early lung transplant referral, since corticosteroids are not indicated and median survival is 3 to 5 years.

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What this article covers

  • Overview

    IPF is the most common idiopathic interstitial pneumonia: a chronic, progressive fibrosing lung disease of unknown cause, defined by the pattern of usual interstitial pneumonia (UIP), reflecting…

  • Epidemiology

    Age of onset is characteristically older: usually affects men and women older than 50 years, most commonly between ages 60 and 70; diagnosis under age 50 is rare and should prompt reconsideration.

  • Risk factors

    Cigarette smoking: the most consistently identified risk factor; many patients carry a substantial pack-year history.

  • Pathophysiology

    Recurrent alveolar epithelial injury is the initiating event: repeated microinjury (smoking, occult microaspiration, environmental exposures) in a susceptible host triggers an aberrant wound-healing response rather than normal repair.

  • Clinical features

    Progressive exertional dyspnea for more than six months is the hallmark: insidious onset, gradually limiting exercise tolerance, in a patient older than 50 to 60 years without an identified cause.

  • Differential diagnosis

    Hypersensitivity pneumonitis: a UIP-like pattern can develop chronically, but a history of organic antigen exposure (birds, mold, hot tubs), a subacute flu-like onset, mid/upper-lung distribution…

  • Diagnosis

    The diagnosis is established by identifying a UIP pattern (histopathologic and/or radiographic) and excluding all other known causes of UIP; it is a diagnosis of exclusion by multidisciplinary…

  • Management

    First, refer to a pulmonologist or ILD center before starting antifibrotic drug therapy, given the need for correlated diagnostic confirmation and monitoring.

  • Complications

    Acute exacerbation of IPF: rapid worsening with new bilateral ground-glass opacities on background fibrosis; mortality exceeds 50%, approaching 100% with mechanical ventilation, the most feared complication.

  • Prognosis

    Median survival is 3 to 5 years from diagnosis, historically, before antifibrotic therapy; long-term outcomes with antifibrotic treatment are not yet fully defined but the agents slow functional decline.

  • Prevention

    No screening program is established for asymptomatic at-risk individuals; the priority is prompt evaluation of unexplained dyspnea or cough lasting more than six months in a patient older than 50 to 60 years, particularly a smoker.

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