Orphan Lung Diseases pp 349-362 | Cite as
Non-specific, Unclassifiable, and Rare Idiopathic Interstitial Pneumonia: Acute Interstitial Pneumonia, Respiratory Bronchiolitis Interstitial Pneumonia, Desquamative Interstitial Pneumonia, Nonspecific Interstitial Pneumonia
Abstract
Acute interstitial pneumonia (AIP), also called Hamman-Rich syndrome, is a rapidly progressive diffuse parenchymal lung disease of unknown etiology resulting in hypoxemic respiratory failure which is often fatal. Respiratory bronchiolitis interstitial lung disease (RB-ILD) and desquamative interstitial pneumonia (DIP) are believed to be on the same spectrum of disease, and share tobacco use as a common denominator; however there is a significant difference between the morbidity and mortality of the two conditions. Non-specific interstitial pneumonia (NSIP) is a pathologic description of a chronic interstitial pneumonia that lacks the histopathologic features typical of other idiopathic interstitial pneumonias, despite many similarities in clinical and radiographic presentation. This section is gives information regarding the history, epidemiology, definition, clinical presentation, and treatment of acute interstitial pneumonia (AIP), respiratory bronchiolitis interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), and non-specific interstitial pneumonia (NSIP).
Keywords
Nonspecific Hamman-Rich Bronchiolitis Desquamative Pneumonia Idiopathic UnclassifiableAbbreviations
- AIP
Acute Interstitial Pneumonia
- ARDS
Acute Respiratory Distress Syndrome
- BAL
Broncho-Alveolar Lavage
- DIP
Desquamative Interstitial Pneumonia
- GGO
Ground Glass Opacification
- HRCT
High Resolution Computed Tomography
- ILD
Interstitial Lung Disease
- NSIP
Non-Specific Interstitial Pneumonia
- RB
Respiratory Bronchiolitis
- RB-ILD
Respiratory Bronchiolitis Interstitial Lung Disease
- TPMT
Thiopurine Methyltransferase
- UCTD
Undifferentiated Connective Tissue Disease
- UIP
Usual Interstitial Pneumonia
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