Hamman-Rich syndrome natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

If left untreated, patients with acute interstitial pneumonitis have high fatality rate and die because of severe respiratory failure. Most of the survivors after initial hospitalization may develop recurrent disease or chronic lung fibrosis. Acute interstitial pneumonitis usually has a very poor prognosis.

• Acute interstitial pneumonitis has an acute and severe onset, progresses very rapidly and have a high mortality rate. If left untreated, patients die because of respiratory failure. Survivors after initial hospitalization may develop recurrent disease or chronic lung fibrosis.^[1]

• Common complications of acute interstitial pneumonitis include:^[2]
  • Respiratory failure and its complications
    • Pulmonary emboli
    • Barotrauma
    • Pneumonia
    • Gastrointestinal hemorrhage
    • Renal failure
  • Progressive pulmonary fibrosis

• Prognosis of acute interstitial pneumonitis is poor.^[3][4]
• Most patients die because of acute respiratory failure or its complications.
• Mortality rate is very high, more than half of the patients die within 2 months. Patients who survive the initial hospitalization die within six months of presentation of disease.
• Recurrence of the disease and progression to chronic interstitial lung disease can occur among survivors.

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