Idiopathic interstitial pneumonia natural history, complications and prognosis: Difference between revisions
Jump to navigation
Jump to search
Ahmed Zaghw (talk | contribs) No edit summary |
Ahmed Zaghw (talk | contribs) No edit summary |
||
| Line 18: | Line 18: | ||
*Controversial debate among the patient age and the baseline pulmonary function, as some studies found that may have predictive significance however other studies found that inconsistent with any prognostic value. | *Controversial debate among the patient age and the baseline pulmonary function, as some studies found that may have predictive significance however other studies found that inconsistent with any prognostic value. | ||
*HRCT has a prognostic value as consistent HRCT findings are associated with worst survival with median survival of 2.08, however inconsistent HRCT findings are associated with less mortality with more median survival 5.79. <ref name="Flaherty-2003">{{Cite journal | last1 = Flaherty | first1 = KR. | last2 = Thwaite | first2 = EL. | last3 = Kazerooni | first3 = EA. | last4 = Gross | first4 = BH. | last5 = Toews | first5 = GB. | last6 = Colby | first6 = TV. | last7 = Travis | first7 = WD. | last8 = Mumford | first8 = JA. | last9 = Murray | first9 = S. | title = Radiological versus histological diagnosis in UIP and NSIP: survival implications. | journal = Thorax | volume = 58 | issue = 2 | pages = 143-8 | month = Feb | year = 2003 | doi = | PMID = 12554898 }}</ref> | *Histological and radiological parameters were considered over the past decade as an estimate for the patient outcome. | ||
*Conventional radiological findings as mild ground glass attenuation predicts physiologic improvement after steroid treatment. <ref name="Wells-1993">{{Cite journal | last1 = Wells | first1 = AU. | last2 = Rubens | first2 = MB. | last3 = du Bois | first3 = RM. | last4 = Hansell | first4 = DM. | title = Serial CT in fibrosing alveolitis: prognostic significance of the initial pattern. | journal = AJR Am J Roentgenol | volume = 161 | issue = 6 | pages = 1159-65 | month = Dec | year = 1993 |doi = 10.2214/ajr.161.6.8249719 | PMID = 8249719 }}</ref> As mild ground glass opacity on CT often regresses on treatment in patients who have histological DIP, but may not decrease as readily in those with histologic UIP. <ref name="Hartman-1996">{{Cite journal | last1 = Hartman | first1 = TE. | last2 = Primack | first2 = SL. | last3 = Kang | first3 = EY. | last4 = Swensen | first4 = SJ. | last5 = Hansell | first5 = DM. | last6 = McGuinness | first6 = G. |last7 = Müller | first7 = NL. | title = Disease progression in usual interstitial pneumonia compared with desquamative interstitial pneumonia. Assessment with serial CT. | journal = Chest | volume = 110 | issue = 2 | pages = 378-82 | month = Aug | year = 1996 | doi = | PMID = 8697837 }}</ref> However some areas of ground glass opacity not only regress with treatment, but it may progress to reticular opacity or honeycombing on follow-up evaluation <ref name="Terriff-1992">{{Cite journal | last1 = Terriff | first1 = BA. | last2 = Kwan | first2 = SY. | last3 = Chan-Yeung | first3 = MM. | last4 = Müller |first4 = NL. | title = Fibrosing alveolitis: chest radiography and CT as predictors of clinical and functional impairment at follow-up in 26 patients. |journal = Radiology | volume = 184 | issue = 2 | pages = 445-9 | month = Aug | year = 1992 | doi = 10.1148/radiology.184.2.1620845 | PMID = 1620845 }}</ref> Predominant reticular opacity or honeycombing usually progress despite treatment. The extent of lung fibrosis on CT is an important predictor of survival. <ref name="Gay-1998">{{Cite journal | last1 = Gay | first1 = SE. | last2 = Kazerooni | first2 = EA. | last3 = Toews | first3 = GB. | last4 = Lynch | first4 = JP. | last5 = Gross | first5 = BH. | last6 = Cascade | first6 = PN. | last7 = Spizarny | first7 = DL. | last8 = Flint | first8 = A. |last9 = Schork | first9 = MA. | title = Idiopathic pulmonary fibrosis: predicting response to therapy and survival. | journal = Am J Respir Crit Care Med |volume = 157 | issue = 4 Pt 1 | pages = 1063-72 | month = Apr | year = 1998 | doi = 10.1164/ajrccm.157.4.9703022 | PMID = 9563720 }}</ref> | |||
*HRCT has a prognostic value as consistent HRCT findings are associated with the worst survival with median survival of 2.08, however inconsistent HRCT findings are associated with less mortality with more median survival 5.79. <ref name="Flaherty-2003">{{Cite journal | last1 = Flaherty | first1 = KR. | last2 = Thwaite | first2 = EL. | last3 = Kazerooni | first3 = EA. | last4 = Gross | first4 = BH. | last5 = Toews | first5 = GB. | last6 = Colby | first6 = TV. | last7 = Travis | first7 = WD. | last8 = Mumford | first8 = JA. | last9 = Murray | first9 = S. | title = Radiological versus histological diagnosis in UIP and NSIP: survival implications. | journal = Thorax | volume = 58 | issue = 2 | pages = 143-8 | month = Feb | year = 2003 | doi = | PMID = 12554898 }}</ref> | |||
* Composite index increase the prognostic power as multiple parameters are more accurate than single parameter. | * Composite index increase the prognostic power as multiple parameters are more accurate than single parameter. | ||
| Line 24: | Line 28: | ||
* Composite physiologic index (CPI) from simple spirometry and DlCO and demonstrated that CPI was linked to mortality more closely than the individual pulmonary function test values. <ref name="Flaherty-2003">{{Cite journal | last1 = Flaherty | first1 = KR. | last2 = Thwaite | first2 = EL. | last3 = Kazerooni | first3 = EA. | last4 = Gross | first4 = BH. | last5 = Toews | first5 = GB. | last6 = Colby | first6 = TV. | last7 = Travis | first7 = WD. | last8 = Mumford | first8 = JA. | last9 = Murray | first9 = S. | title = Radiological versus histological diagnosis in UIP and NSIP: survival implications. | journal = Thorax | volume = 58 | issue = 2 | pages = 143-8 | month = Feb | year = 2003 | doi = | PMID = 12554898 }}</ref> | * Composite physiologic index (CPI) from simple spirometry and DlCO and demonstrated that CPI was linked to mortality more closely than the individual pulmonary function test values. <ref name="Flaherty-2003">{{Cite journal | last1 = Flaherty | first1 = KR. | last2 = Thwaite | first2 = EL. | last3 = Kazerooni | first3 = EA. | last4 = Gross | first4 = BH. | last5 = Toews | first5 = GB. | last6 = Colby | first6 = TV. | last7 = Travis | first7 = WD. | last8 = Mumford | first8 = JA. | last9 = Murray | first9 = S. | title = Radiological versus histological diagnosis in UIP and NSIP: survival implications. | journal = Thorax | volume = 58 | issue = 2 | pages = 143-8 | month = Feb | year = 2003 | doi = | PMID = 12554898 }}</ref> | ||
* The CPI score is easier to generate because no radiographic scoring or exercise data are required. But still wide validation studies for CPI are required | * The CPI score is easier to generate because no radiographic scoring or exercise data are required. But still wide validation studies for CPI are required | ||
==Major Idiopathic Interstitial Pneumonias== | ==Major Idiopathic Interstitial Pneumonias== | ||
Revision as of 16:55, 19 November 2013
|
Idiopathic Interstitial Pneumonia Microchapters |
|
Differentiating Idiopathic interstitial pneumonia from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Idiopathic interstitial pneumonia natural history, complications and prognosis On the Web |
|
American Roentgen Ray Society Images of Idiopathic interstitial pneumonia natural history, complications and prognosis |
|
FDA onIdiopathic interstitial pneumonia natural history, complications and prognosis |
|
CDC on Idiopathic interstitial pneumonia natural history, complications and prognosis |
|
Idiopathic interstitial pneumonia natural history, complications and prognosisin the news |
|
on Idiopathic interstitial pneumonia natural history, complications and prognosis |
|
Directions to Hospitals Treating Idiopathic interstitial pneumonia |
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
- Definition of acute exacerbation of IPF require evidence of deterioration and exclusion of common causes, such as infection, pulmonary embolism, heart failure, and pneumothorax. Usually it is associated with a histopathologic pattern of diffuse alveolar damage may be a common terminal event. [1]
- Some retrospective analysis showed that 50% of the mortality cause for IPF patients followed acute deterioration in pulmonary function associated with acute exacerbation. [4]
Predictors for survival
- Controversial debate among the patient age and the baseline pulmonary function, as some studies found that may have predictive significance however other studies found that inconsistent with any prognostic value.
- Histological and radiological parameters were considered over the past decade as an estimate for the patient outcome.
- Conventional radiological findings as mild ground glass attenuation predicts physiologic improvement after steroid treatment. [5] As mild ground glass opacity on CT often regresses on treatment in patients who have histological DIP, but may not decrease as readily in those with histologic UIP. [6] However some areas of ground glass opacity not only regress with treatment, but it may progress to reticular opacity or honeycombing on follow-up evaluation [7] Predominant reticular opacity or honeycombing usually progress despite treatment. The extent of lung fibrosis on CT is an important predictor of survival. [8]
- HRCT has a prognostic value as consistent HRCT findings are associated with the worst survival with median survival of 2.08, however inconsistent HRCT findings are associated with less mortality with more median survival 5.79. [9]
- Composite index increase the prognostic power as multiple parameters are more accurate than single parameter.
- Composite physiologic index (CPI) from simple spirometry and DlCO and demonstrated that CPI was linked to mortality more closely than the individual pulmonary function test values. [9]
- The CPI score is easier to generate because no radiographic scoring or exercise data are required. But still wide validation studies for CPI are required
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) [3]
- 5-yr mortality 80%, median survival 2-3 yr
- Mortality is most frequently due to respiratory failure (38.7%); other causes of death include heart failure (14.4%), bronchogenic carcinoma (10.4%), ischemic heart disease (9.5%), infection (6.5%), and pulmonary embolism (3.4%) [10]
- Nonfatal disease-associated complications include pneumothorax, corticosteroid-induced metabolic side effects and myopathy, and therapy-related immunosuppression.
- 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% [11]
- other studies showed that 10-yr survival of fibrotic NSIP is 35%,[12] 5-Yr survival rate is 43%, [13] 25% deterioration of lung function despite treatment.[14]
Acute/Subacute IIPs
- Cryptogenic Organizing Pneumonia
- 5-yr mortality < 5% [11]
- Acute Interstitial Pneumonia (Hamman-Rich Syndrome)
- Respiratory Bronchiolitis-Interstitial Lung Disease
- No death reported, 5-yr mortality < 5% [11]
- Desquamative Interstitial Pneumonia
- No death reported, 5-yr mortality < 5% [11]
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) [15] [16] [17]
References
- ↑ Rice, AJ.; Wells, AU.; Bouros, D.; du Bois, RM.; Hansell, DM.; Polychronopoulos, V.; Vassilakis, D.; Kerr, JR.; Evans, TW. (2003). "Terminal diffuse alveolar damage in relation to interstitial pneumonias. An autopsy study". Am J Clin Pathol. 119 (5): 709–14. doi:10.1309/UVAR-MDY8-FE9F-JDKU. PMID 12760290. Unknown parameter
|month=ignored (help) - ↑ Azuma, A.; Nukiwa, T.; Tsuboi, E.; Suga, M.; Abe, S.; Nakata, K.; Taguchi, Y.; Nagai, S.; Itoh, H. (2005). "Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis". Am J Respir Crit Care Med. 171 (9): 1040–7. doi:10.1164/rccm.200404-571OC. PMID 15665326. Unknown parameter
|month=ignored (help) - ↑ 3.0 3.1 3.2 Kim, DS.; Park, JH.; Park, BK.; Lee, JS.; Nicholson, AG.; Colby, T. (2006). "Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features". Eur Respir J. 27 (1): 143–50. doi:10.1183/09031936.06.00114004. PMID 16387947. Unknown parameter
|month=ignored (help) - ↑ Martinez, FJ.; Safrin, S.; Weycker, D.; Starko, KM.; Bradford, WZ.; King, TE.; Flaherty, KR.; Schwartz, DA.; Noble, PW. (2005). "The clinical course of patients with idiopathic pulmonary fibrosis". Ann Intern Med. 142 (12 Pt 1): 963–7. PMID 15968010. Unknown parameter
|month=ignored (help) - ↑ Wells, AU.; Rubens, MB.; du Bois, RM.; Hansell, DM. (1993). "Serial CT in fibrosing alveolitis: prognostic significance of the initial pattern". AJR Am J Roentgenol. 161 (6): 1159–65. doi:10.2214/ajr.161.6.8249719. PMID 8249719. Unknown parameter
|month=ignored (help) - ↑ Hartman, TE.; Primack, SL.; Kang, EY.; Swensen, SJ.; Hansell, DM.; McGuinness, G.; Müller, NL. (1996). "Disease progression in usual interstitial pneumonia compared with desquamative interstitial pneumonia. Assessment with serial CT". Chest. 110 (2): 378–82. PMID 8697837. Unknown parameter
|month=ignored (help) - ↑ Terriff, BA.; Kwan, SY.; Chan-Yeung, MM.; Müller, NL. (1992). "Fibrosing alveolitis: chest radiography and CT as predictors of clinical and functional impairment at follow-up in 26 patients". Radiology. 184 (2): 445–9. doi:10.1148/radiology.184.2.1620845. PMID 1620845. Unknown parameter
|month=ignored (help) - ↑ Gay, SE.; Kazerooni, EA.; Toews, GB.; Lynch, JP.; Gross, BH.; Cascade, PN.; Spizarny, DL.; Flint, A.; Schork, MA. (1998). "Idiopathic pulmonary fibrosis: predicting response to therapy and survival". Am J Respir Crit Care Med. 157 (4 Pt 1): 1063–72. doi:10.1164/ajrccm.157.4.9703022. PMID 9563720. Unknown parameter
|month=ignored (help) - ↑ 9.0 9.1 Flaherty, KR.; Thwaite, EL.; Kazerooni, EA.; Gross, BH.; Toews, GB.; Colby, TV.; Travis, WD.; Mumford, JA.; Murray, S. (2003). "Radiological versus histological diagnosis in UIP and NSIP: survival implications". Thorax. 58 (2): 143–8. PMID 12554898. Unknown parameter
|month=ignored (help) - ↑ Panos, RJ.; Mortenson, RL.; Niccoli, SA.; King, TE. (1990). "Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment". Am J Med. 88 (4): 396–404. PMID 2183601. Unknown parameter
|month=ignored (help) - ↑ 11.0 11.1 11.2 11.3 11.4 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)