Hamman-Rich syndrome natural history, complications and prognosis

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

Overview

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
  • The symptoms of (disease name) typically develop ___ years after exposure to ___.
  • If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

  • Common complications of acute interstitial pneumonitis include:[1]
    • Respiratory failure and its complications
      • Pulmonary emboli
      • Barotrauma
      • Pneumonia
      • Gastrointestinal hemorrhage
      • Renal failure
    • Progressive pulmonary fibrosis

Prognosis

  • Prognosis of acute interstitial pneumonitis is poor.[2][3]
  • 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.

References

  1. Matthay MA, Zimmerman GA (October 2005). "Acute lung injury and the acute respiratory distress syndrome: four decades of inquiry into pathogenesis and rational management". Am. J. Respir. Cell Mol. Biol. 33 (4): 319–27. doi:10.1165/rcmb.F305. PMC 2715340. PMID 16172252.
  2. Avnon LS, Pikovsky O, Sion-Vardy N, Almog Y (January 2009). "Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations". Anesth. Analg. 108 (1): 232–7. doi:10.1213/ane.0b013e318188af7a. PMID 19095855.
  3. Parambil JG, Myers JL, Aubry MC, Ryu JH (July 2007). "Causes and prognosis of diffuse alveolar damage diagnosed on surgical lung biopsy". Chest. 132 (1): 50–7. doi:10.1378/chest.07-0104. PMID 17475632.

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