Acute interstitial pneumonia (Hamman-Rich syndrome)
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
Acute interstitial pneumonia (AIP) is a rare and fulminant form of diffuse lung injury originally described by Hamman and Rich in 1935 [1,2]. AIP is classified as an idiopathic interstitial pneumonia (IIP), and among the IIPs, it has the most acute onset and rapidly progressive course [1-3].
AIP is similar in presentation to the acute respiratory distress syndrome (ARDS) and probably represents a subset of cases of idiopathic ARDS . (See "Acute respiratory distress syndrome: Clinical features and diagnosis in adults".)
The clinical presentation, diagnosis, treatment, and prognosis of AIP will be reviewed here. The other idiopathic interstitial pneumonias and the approach to patients with interstitial lung disease are discussed separately. (See "Idiopathic interstitial pneumonias: Clinical manifestations and pathology" and "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Approach to the adult with interstitial lung disease: Diagnostic testing".)
AIP generally affects previously healthy individuals without a prior history of lung disease and occurs with roughly equal frequency in men and women [5-8]. It is not associated with cigarette smoking. Most patients are over the age of 40 years, with a mean age of 50 to 55 years [3,5,6,8,9].
AIP has the histopathologic appearance of diffuse alveolar damage (DAD) . DAD is a reaction pattern that occurs in response to a number of known causes of lung injury, but in the case of AIP, it is idiopathic (table 1) . DAD has characteristic temporal phases in its evolution — acute exudative, organizing proliferative, and healed (or fibrotic) phases (table 2) [3,6]. (See "Idiopathic interstitial pneumonias: Clinical manifestations and pathology".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- Suda T, Kaida Y, Nakamura Y, et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med 2009; 103:846.
- Johkoh T, Müller NL, Taniguchi H, et al. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology 1999; 211:859.
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- Lim SY, Suh GY, Choi JC, et al. Usefulness of open lung biopsy in mechanically ventilated patients with undiagnosed diffuse pulmonary infiltrates: influence of comorbidities and organ dysfunction. Crit Care 2007; 11:R93.
- Parambil JG, Myers JL, Ryu JH. Diffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connective tissue diseases. Chest 2006; 130:553.
- Park IN, Kim DS, Shim TS, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest 2007; 132:214.
- Avnon LS, Pikovsky O, Sion-Vardy N, Almog Y. Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations. Anesth Analg 2009; 108:232.
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- Robinson DS, Geddes DM, Hansell DM, et al. Partial resolution of acute interstitial pneumonia in native lung after single lung transplantation. Thorax 1996; 51:1158.
- Ogawa D, Hashimoto H, Wada J, et al. Successful use of cyclosporin A for the treatment of acute interstitial pneumonitis associated with rheumatoid arthritis. Rheumatology (Oxford) 2000; 39:1422.
- CLINICAL FEATURES
- Arterial blood gases
- Microbiologic tests
- Bronchoscopy and BAL
- Lung biopsy
- DIFFERENTIAL DIAGNOSIS
- Supportive care
- Other immunosuppressive therapy
- Lung transplantation
- SUMMARY AND RECOMMENDATIONS