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Idiopathic Pulmonary Fibrosis
Transcript of Idiopathic Pulmonary Fibrosis
Usual Interstitial Pneumonia
J.C.'s Physical Manifestations
HR: 84bpm regular
Crackles auscultated bilaterally
Bright red hemoptysis mixed with sputum
SaO2 at 93% at 10L/min
J.C. was an 85 y/o male admitted to ED on 9/13 with hemoptysis x 6 weeks and SOB. History of Idiopathic Pulmonary Fibrosis, Coronary Artery Disease, NSTEMI on 8/2012, chronic chest pain, colon cancer & colecectomy in 2005.
What is Idiopathic Pulmonary Fibrosis?
What does idiopathic mean? Why is this disease idiopathic?
Idiopathic Pulmonary Fibrosis
What does a diagnosis of IPF
mean to our patients?
Irritation is believed to occur either through the inhalation of an irritant
or as the result of an endogenous
Idiopathic Pulmonary Fibrosis
chronic non-productive cough
clubbing of fingers
Identification of Usual Interstitial Pneumonia and Honeycombing of alveoli.
Pathophysiology of IPF
MMPs & TIMPs
Alveolar Epithelial Cells Type 2
image source: http://www.studyblue.com/notes/note/n/w-ards--sepsis/deck/1264727
Begins with inflammation
image source: http://en.wikipedia.org/wiki/File:Usual_interstitial_pneumonia_%281%29.JPG#filehistory
image source: http://www.medicalhistology.us/twiki/bin/view/Main/RespiratorySystemAtlas09
Best diagnostic method
Matrix Metalloproteinases (MMPs) are critical mediators of extracellular degradation as directed by Tissue Inhibitors of Metalloproteinases (TIMPs). (Muller-Quernheim, 2011)
image source: http://www.brown.edu/Courses/Digital_Path/systemic_path/pulmonary/honeycomb.html
Decreased Lung Compliance
Decrease in FVC, FEV1, TLC & FRC
image source: http://safetycouncilsemi.org/safety/first-aid-cpr-aed-training/
Chronic Chest Pain
image source: http://www.havi-north.com/angina.html
image source: https://www.meducation.net/resources/10442-Right-Ventricle-Hypertrophy-Gross-Fixed-Tissue-But-Good-Color-Horizontal-Sectio
image source: http://en.wikipedia.org/wiki/File:Right_Ventricular_hypertrophy.svg#file
IPF increases the risk of Alveolar Infection
Likely cause of hemoptysis
image source: http://www.learningradiology.com/notes/chestnotes/hodgkinspage.htm
Possible link to pulmonary infection and...
image source: http://www.mybiologica.com/7450/mesothelioma/mesothelioma-diagnosis.html
Oh great! A mass like consolidation in the left lower lung lobe!
image source: http://www.meddean.luc.edu/lumen/meded/medicine/pulmonar/cxr/atlas/plmass1.htm
Emphysema was also noted in the upper lobes of J.C.'s lungs. The attending physicians suspected that it was a result of J.C.'s prior history of smoking.
"The presence of IPF with secondary pulmonary hypertension negatively affects the patient's prognosis." (Raghu et al., 2011)
The combined impact on J.C.
Rapid desaturation during ambulation.
Constant need for high flow O2 via NC.
"Exercise intolerance and rapid desaturation to 89% SaO2 while performing 6 min. walk test is a strong determinant of poor prognosis." (Nathan, Noble and Tuder, 2007)
CO2: 31 mm Hg, HCO3: 23.6 mmol/L, pH: 7.45
pO2: 81 mm Hg
lactic acid: 4.1 mmol/L (0.5-2.2 mmol/L)
platelet count 91K/mm3 (152-348 K/mm3)
D-Dimer 919 nanograms (35-540 nanograms)
bilirubin 1.9 mg/dL (0.1-1.4 mg/dL) & Aspartate Aminotransferase 106 units/L (16-63 units/L) indicate possible liver damage.
image source: http://blog.cambridgecoaching.com/blog/bid/246608/Advice-from-a-Chemistry-Tutor-Identifying-Unknown-Chemicals-Lab
Treatment of Infection:
cefepime: 1g/100ml IV Q8H
Vancomycin: 500mg Q8H
cefuroxime: 500mg PO BID
Treatment of Angina:
aspirin 81mg PO daily
Clopidogrel (plavix) 75mg PO daily
Treatment of IPF
image source: http://ragraphic.wordpress.com/category/four-week-tasks/
Albuterol 2.5mg/Ipratropium 0.5mg via INH Q4H
methylprednisolone 40mg/1ml IV Q12H
High flow O2
There is no currently FDA approved medication regimen proven effective at treating IPF. Lung transplantation is the only medical intervention that can increase a patient's lifespan.
Cottin, V., Le Pavec, J., Prevot, G., Mal, H., Humbert, M., Simonneau, G., Cordier, J-F., Germ, P. (2009, July 30). Pulmonary Hypertension in Patients with Combined
Pulmonary Fibrosis and Emphysema Syndrome. Retrieved from http:// www.ersj.org.uk/content/35/1/105.full
Godfrey, A., Ouellette, D. (2013, September 20). Idiopathic Pulmonary Fibrosis. Medscape. Retrieved from http://emedicine.medscape.com/article/301226- overview
Gunther, A., Korfei, M., Mahavadi, P., Von Der Beck, D., Ruppert, C., Markart, P. (2012, March 16).
Unravelling the Progressive Pathophysiology of Idiopathic Pulmonary Fibrosis. Retrieved from http://err.ersjournals.com/content/21/124/152.short
Lewis, S. L., Dirksen, S. R., Heitkemper, M. M., Bucher, L & Camera, I. (2011).
Medical surgical nursing: Assessment and management of clinical problems (8th ed.). St. Lewis, MO: Mosby Elsevier.
Meltzer, E., Noble, P. (2008, March 26). Idiopathic Pulmonary Fibrosis. Retrieved from
Muller-Quernheim, J. (2011, July 1). MMP’s are Regulatory Enzymes in Pathways of
Inflammatory Disorders, Tissue Injury, Malignancies and Remodelling of the Lung. Retrieved from http://www.ersj.org.uk/content/38/1/12.full
Nathan, S., Noble, P., Tudor, R. (2007, May 1). Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension: Connecting the Dots. Retrieved from http:// www.atsjournals.org/doi/full/10.1164/rccm.200608-1153CC#.UpPNEm1XjTo
Pagana, K. D., & Pagana, T. J. (2010). Mosby's manual of diagnostic and laboratory tests (4th ed.). St. Louis, Mo: Mosby Elsevier.
Raghu, G., Collard, H., Egan, J., Martinez, F., Behr, J., Brown, K., Colby, T., Cordier, J- F., Flaherty, K., Lasky, J., Lynch, D., Ryu, J., Swigris, J., Wells, A., Ancochea, J., Bouros, D., Carvalho, C., Costabel, U., Ebina, M., Hansell, D., Johkoh, T., Soon Kim, D., King Jr., D., Kondoh, Y., Myers, J., Muller, N., Nicholson, A., Richeldi, L., Selman, M., Dudden, R., Griss, B., Protzko, S., Schunemann, H. (2011, March 15). An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management.
Retrieved from http://www.atsjournals.org/doi/full/10.1164/rccm. 2009-040GL#.Uo7xxG1XjTo
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image source: http://n-equals-one.com/blogs/2010/09/29/immunity-and-bacterial-flora/