Acute interstitial pneumonitis (AIP), also known as Hamman-Rich syndrome is a rapidly progressive non-infectious interstitial lung disease of unknown aetiology. It is considered the only acute process among the idiopathic interstitial pneumonias.
Truly idiopathic AIP tends to occur in those without pre-existing lung disease and typically affects middle-aged adults (mean ~ 50 years 5). However, in certain conditions such as leflunomide-induced acute interstitial pneumonia, patients have pre-existing lung disease.
Clinical features are varied. Patients often have a history of an antecedent illness such as a viral upper respiratory infection. Common initial symptoms include myalgia, arthralgia, pyrexia, chills, and malaise. Severe exertional dyspnoea develops over a matter of days to weeks 13.
AIP is characterised histologically by diffuse alveolar damage (DAD) 2. The alveolar damage comprises three phases:
- an acute exudative phase
- a subsequent organising phase
- a final fibrotic phase
Histological features are very similar to those of adult respiratory distress syndrome (ARDS).
The clinical context is vital for correct image interpretation.
Nonspecific and often shows bilateral patchy airspace opacification.
During the initial stages, AIP can have features similar to adult respiratory distress syndrome (ARDS), which include:
- areas with ground-glass attenuation: generally tend to be bilateral and symmetrical 10
- traction bronchiectasis: can be seen in ~80% of cases during the course of the disease 4 and correlates with disease duration 2
- parenchymal architectural distortion of the lung
- air space consolidation: may have a slight predilection towards the dependent portions 5
Treatment and prognosis
The condition usually progresses to respiratory failure that requires mechanical ventilation and corticosteroid therapy. Even despite mechanical ventilation, it often carries a grave prognosis with > 70% mortality at ~ 3 months 1.
History and etymology
- clinical features first described by L Hamman and A Rich in 1935 8
- pathological processes first described by A L Katzenstein et al. in 1986 3
Considerations in early stages include:
- adult respiratory distress syndrome (ARDS): can involve other organs 9
- infectious multifocal pneumonia
Other considerations include:
- an acute interstitial pneumonitis process triggered by certain medications, e.g. leflunomide-induced acute interstitial pneumonia
For a more general differential, consider:
- 1. Bouros D, Nicholson AC, Polychronopoulos V et-al. Acute interstitial pneumonia. Eur. Respir. J. 2000;15 (2): 412-8. Eur. Respir. J. (link) - Pubmed citation
- 2. Johkoh T, Müller NL, Taniguchi H et-al. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology. 1999;211 (3): 859-63. Radiology (full text) - Pubmed citation
- 3. Katzenstein AL, Myers JL, Mazur MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and cell kinetic study. Am. J. Surg. Pathol. 1986;10 (4): 256-67. - Pubmed citation
- 4. Tomiyama N, Müller NL, Johkoh T et-al. Acute parenchymal lung disease in immunocompetent patients: diagnostic accuracy of high-resolution CT. AJR Am J Roentgenol. 2000;174 (6): 1745-50. AJR Am J Roentgenol (full text) - Pubmed citation
- 5. Wittram C, Mark EJ, Mcloud TC. CT-histologic correlation of the ATS/ERS 2002 classification of idiopathic interstitial pneumonias. Radiographics. 23 (5): 1057-71. doi:10.1148/rg.235035702 - Pubmed citation
- 6. Silva CI, Müller NL. Idiopathic interstitial pneumonias. J Thorac Imaging. 2009;24 (4): 260-73. doi:10.1097/RTI.0b013e3181c1a9eb - Pubmed citation
- 7. Vourlekis JS, Brown KK, Cool CD et-al. Acute interstitial pneumonitis. Case series and review of the literature. Medicine (Baltimore). 2000;79 (6): 369-78. Medicine (Baltimore) (link) - Pubmed citation
- 8 Hamman, L, Rich, A Fulminating diffuse interstitial fibrosis of the lungs. Trans Am Clin Clinatol Assoc 1935;51,154-163
- 9. Quefatieh A, Stone CH, Digiovine B et-al. Low hospital mortality in patients with acute interstitial pneumonia. Chest. 2003;124 (2): 554-9. doi:10.1378/chest.124.2.554 - Pubmed citation
- 10.Primack SL, Hartman TE, Ikezoe J et-al. Acute interstitial pneumonia: radiographic and CT findings in nine patients. Radiology. 1993;188 (3): 817-20. Radiology (abstract) - Pubmed citation
- 11. Mueller-mang C, Grosse C, Schmid K et-al. What every radiologist should know about idiopathic interstitial pneumonias. Radiographics. 27 (3): 595-615. doi:10.1148/rg.273065130 - Pubmed citation
- 12. Ichikado K, Suga M, Müller NL et-al. Acute interstitial pneumonia: comparison of high-resolution computed tomography findings between survivors and nonsurvivors. Am. J. Respir. Crit. Care Med. 2002;165 (11): 1551-6. doi:10.1164/rccm.2106157 - Pubmed citation
- 13. Bonaccorsi A, Cancellieri A, Chilosi M et-al. Acute interstitial pneumonia: report of a series. Eur. Respir. J. 2003;21 (1): 187-91. doi:10.1183/09031936.03.00297002 - Pubmed citation
- 14. Avnon LS, Pikovsky O, Sion-Vardy N et-al. Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations. Anesth. Analg. 2009;108 (1): 232-7. doi:10.1213/ane.0b013e318188af7a - Pubmed citation
Interstitial lung disease
interstitial lung disease
- drug-induced interstitial lung disease
- hypersensitivity pneumonitis
idiopathic interstitial pneumonia (mnemonic)
- acute interstitial pneumonia (AIP)
- cryptogenic organising pneumonia (COP)
- desquamative interstitial pneumonia (DIP)
- idiopathic non-specific interstitial pneumonia (NSIP)
- idiopathic pleuroparenchymal fibroelastosis
- lymphoid interstitial pneumonia (LIP)
- respiratory bronchiolitis–associated interstitial lung disease (RB-ILD)
- usual interstitial pneumonia / idiopathic pulmonary fibrosis (UIP/IPF)