Idiopathic interstitial pneumonia 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: Ahmed Zaghw, M.D. [2]
Overview
Natural history and Complications
Major Idiopathic Interstitial Pneumonias
Chronic Fibrosing IIPs
- Idiopathic Pulmonary Fibrosis
- The most common and the worst prognosis of chronic IIPs.
- survival time depends on the definition of the diagnosis ( reticular background on conventional X-ray or onset of the symptoms which usually is 2-3 years after the radiological findings) [1]
- 5-yr mortality 80%, median survival 2-3 yr
- Idiopathic nonspecific interstitial pneumonia
- Better outcome than IPF
- Cellular type is more common than Fibrotic type
- The cellular NSIP is better than fibrosing type of NSIP
- Larger studies are needed to understand the clinical course of NSIP
- Median survival for cellular NSIP > 10 yr and 5-yr mortality <10%, however the median survival for fibrotic NSIP is 6-8 yr, and 5-yr mortality >10% [2]
- other studies showed that 10-yr survival of fibrotic NSIP is 35%,[3] 5-Yr survival rate is 43%, [4] 25% deterioration of lung function despite treatment.[5]
Acute/Subacute IIPs
- Cryptogenic organizing pneumonia
- 5-yr mortality < 5% [2]
- Acute interstitial pneumonia (Hamman-Rich Syndrome)
- Respiratory bronchiolitis-interstitial lung disease
- No death reported, 5-yr mortality < 5% [2]
- Desquamative interstitial pneumonia
- No death reported, 5-yr mortality < 5% [2]
Rare Idiopathic Interstitial Pneumonias
- Idiopathic lymphoid interstitial pneumonia
- Idiopathic pleuroparenchymal fibroelastosis
Biomarkers
The functional lung capacity, pulmonary functions and mortality have been linked to some biomarkers. For example some studies showed that rapidly deteriorating lung function and higher mortality have been associated with high serum level of epithelial or macrophage-related proteins such as SP-A, SP-D, KL-6 (Krebs von den Lungen-6), CCL18 (chemokine ligand-18), and MMP-7 (matrix metalloproteinase-7) [6] [7] [8]
References
- ↑ 1.0 1.1 Kim, DS.; Collard, HR.; King, TE. (2006). "Classification and natural history of the idiopathic interstitial pneumonias". Proc Am Thorac Soc. 3 (4): 285–92. doi:10.1513/pats.200601-005TK. PMID 16738191. Unknown parameter
|month=ignored (help) - ↑ 2.0 2.1 2.2 2.3 2.4 King, TE. (2005). "Clinical advances in the diagnosis and therapy of the interstitial lung diseases". Am J Respir Crit Care Med. 172 (3): 268–79. doi:10.1164/rccm.200503-483OE. PMID 15879420. Unknown parameter
|month=ignored (help) - ↑ Travis, WD.; Matsui, K.; Moss, J.; Ferrans, VJ. (2000). "Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia". Am J Surg Pathol. 24 (1): 19–33. PMID 10632484. Unknown parameter
|month=ignored (help) - ↑ Nicholson, AG.; Colby, TV.; du Bois, RM.; Hansell, DM.; Wells, AU. (2000). "The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis". Am J Respir Crit Care Med. 162 (6): 2213–7. doi:10.1164/ajrccm.162.6.2003049. PMID 11112140. Unknown parameter
|month=ignored (help) - ↑ Kondoh, Y.; Taniguchi, H.; Yokoi, T.; Nishiyama, O.; Ohishi, T.; Kato, T.; Suzuki, K.; Suzuki, R. (2005). "Cyclophosphamide and low-dose prednisolone in idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia". Eur Respir J. 25 (3): 528–33. doi:10.1183/09031936.05.00071004. PMID 15738299. Unknown parameter
|month=ignored (help) - ↑ Satoh, H.; Kurishima, K.; Ishikawa, H.; Ohtsuka, M. (2006). "Increased levels of KL-6 and subsequent mortality in patients with interstitial lung diseases". J Intern Med. 260 (5): 429–34. doi:10.1111/j.1365-2796.2006.01704.x. PMID 17040248. Unknown parameter
|month=ignored (help) - ↑ Prasse, A.; Probst, C.; Bargagli, E.; Zissel, G.; Toews, GB.; Flaherty, KR.; Olschewski, M.; Rottoli, P.; Müller-Quernheim, J. (2009). "Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis". Am J Respir Crit Care Med. 179 (8): 717–23. doi:10.1164/rccm.200808-1201OC. PMID 19179488. Unknown parameter
|month=ignored (help) - ↑ Ishii, H.; Mukae, H.; Kadota, J.; Kaida, H.; Nagata, T.; Abe, K.; Matsukura, S.; Kohno, S. (2003). "High serum concentrations of surfactant protein A in usual interstitial pneumonia compared with non-specific interstitial pneumonia". Thorax. 58 (1): 52–7. PMID 12511721. Unknown parameter
|month=ignored (help)