This document provides an overview of idiopathic pulmonary fibrosis (IPF). It describes IPF as a progressive, irreversible scarring of the lungs of unknown cause. Key points include: IPF results in excessive fibrotic tissue in the lungs; incidence is increasing and median survival is 2-4 years; high-resolution CT is required for diagnosis; no effective pharmacological treatments exist though lung transplantation may increase survival. The pathophysiology involves abnormal epithelial cell behavior leading to fibrosis. Patient education focuses on management, prognosis, medications, and palliative/end-of-life care planning.

2. Introduction
 A progressive, irreversible, devastating interstitial lung
disease
 Etiology unknown (duBois, Weycker, Albera, Bradford, & Costabel ,2011)
 Disease of the basal and peripheral lungs that progresses
centrally and toward apices of the lungs over time (Leslie, 2012)
 Lungs contain excessive amount of fibrous or connective
tissue
 Fibrotic process causes lungs to become stiff and difficult
to ventilate (McCance & Heuther, 2010)

3. X-ray of fibrotic lung evidencing excessive amount of
fibrotic tissue
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4. Incidence and Prevalence of IPF
• Incidence
• No differentiation found among ethnicities
• Rising
• Estimated to be between 4.6 and 16.3 per 100,000
• Median survival post diagnosis is 2 to 4 years
• Prevalence
• More predominant in men than women (1.7:1)
• Frequency increases with age
• Occurs in middle aged and elderly adults (median age at
diagnosis-66 years old, range 55-75) (King, Pardo, Selman, 2011)

5. Assessment
Probable Causes: Exposure to inhaled harmful substances
(toxic fumes, organic/inorganic dusts, smoking) (McCance & Huether,
2010)
• Signs & Symptoms
• Slow progressive breathlessness, especially with exertion
• Non-productive cough
• Decreased oxygen saturation with exercise
• Diffuse inspiratory crackles (Leslie, 2012)
• Clubbing of fingers (King et al., 2011)
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6. Assessment (continued)
 Diagnostics
 Lab studies—reveal mild non-specific elevation of
antinuclear antibodies
 Pulmonary Function Test
 Decreased lung capacity
 Decreased forced vital capacity
 Diffusing capacity for CO2
 Arterial Blood Gas
 Decreased oxygen (pO2) levels
 Increased carbon dioxide (pCO2) levels (Leslie, 2012)

7. Assessment (continued)
 Chest X-Ray
 Will demonstrate fibrotic patches
 Computed Tomography more definitive
 High Resolution Computed Axial Tomography
(HRCT)
 Patchy, coarse, subpleural reticulation
 Distortion of lung architecture
 Presence of pleural-based cysts (required feature
for a confident diagnosis)
 Subpleural “honeycombing” at bases (Leslie, 2012)

8. Assessment (continued)
 Lung Biopsy
 Partially or completely scarred lobules devoid of
alveolar spaces
 Coarse peripheral lobar fibrosis
 Scar tissue demonstrates small cysts lined by
respiratory epithelium
 Fibroblast foci exist at the interface between fibrosis
and uninvolved lung tissue
 Microscopic “honeycombing” nearly always present
(Leslie, 2012)
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9. Pathophysiology of IPF
 A heterogenous disease
 The result of abnormal behavior of alveolar epithelial
cells that:
 Provoke migration, proliferation, and activation of
mesenchymal cells
 Initiate formation of fibroblast and myofibroblast foci
 Activated fibroblasts secrete exaggerated amounts
of extracellular matrix molecules
 Subsequent destruction of lung architecture with
alveolar collapse (King, Pardo, & Selman, 2011)

10. Pathophysiology
(continued)
 Gene expression of CCNA2 and {alpha}-Defensins up-regulated
in patients with exacerbation of IPF,
localized in the alveolar epithelium
 {Alpha}-Defensin and ST2 protein levels in serum found
to be elevated (Bhatti, Girdhar, Usman, & Abubakr, 2013)

11. Pathological Process of IPF & Activation of
Coagulation Cascade and Procoagulant
Signaling
 Tissue factor-Factor VIIa-Factor X complex assembles
on alveolar epithelium
 Factor X activation stimulates fibroblasts within
underlying fibrotic regions
 Thrombin and activated Factor X induce differentiation
of lung fibroblasts to myofibroblasts via the proteinase-activated
receptor (King, Pardo, & Selman, 2011)

12. Proposed Pathological
Sequence (Leslie, 2012)
1. Stretch injury to
epithelial-mesenchymal
transition
2. Formation of the
Fibroblastic Reticulum-Type 2
cells proliferate over tear and
reconstitute the alveolar interface with
air
3. Local alveolar
collapse
4. Collagen
deposition
5. Vascular
6. “Simplification” growth
of lobules-devoid of
alveoli, consist only of
terminal airways and
dilate over time
7. Honeycomb
lung
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13. Genetic/Genomic Implications for
Care & Treatment
 Genetics/Genomics
 Genetic transmission occurs in approximately 0.5-3.7% of
patients with IPF
 Effected families have autosomal dominant vertical
transmission patters of inheritance with reduced
penetrance
 In some familial cases, alterations in unfolded protein
response occur with mutations in surfactant protein C– a
hydrophobic protein expressed exclusively by AEC type II
(King, Pardo, Selman, 2011)

14. Genetic/Genomic Implications for
Care & Treatment (continued)
 A genome wide scan of several families with familial
IPF identified shared haplotype on chromosome 4g31
that harbored ELMOD2—a gene expressed in the lung
 ELMOD2 expressed slightly less in IPF lung when
compared to healthy lung
 ELMOD2 essential for cellular process
 Mutations of telomerase also implicated in familial IPF
(King, Pardo,& Selman, 2011)
 50% of asymptomatic members have evidence of
alveolar inflammation—a possible precursor to IPF (Doyle,
Hunninghake, & Rosas, 2012)

15. Genetic/Genomic Implications for
Care & Treatment (continued)
 Some suggest that increased levels of matrix
metalloproteinase-7 (MMP7) predict disease
progression and mortality
 Biomarker serum CC-chemokine ligand 18 as well as
CXCL9 & CXCL10 have shown to be a predictive value
in IPF
 Others suggest further study of biomarkers
neutrophilelastase, KL-6, and lactate dehydrogenase
for disease determinant (Doyle, Hunninghake,& Rosas, 2012)

16. Care & Treatment of Patients
with IPF
 Pharmacological
 Corticosteroids (Methylprednisolone, Prednisolone)
 Immuno-suppressants (Cyclosporin A,
Cyclophosphamide)
 Antifibrotic compounds (Pirfenidone—not yet
available in the United States for Rx)
 Efficacy unknown
 Antioxidant
 Amino Acid/Mucolytic (Acetylcysteine) (Lee, McLaughlin, & Collard,
2011)

17. Care & Treatment of Patients
with IPF
 Non-pharmacological
 Non-invasive ventilation (NIV)
 High-flow oxygen for patients with resting hypoxia
 Continuous positive airway pressure (CPAP)
 Mechanical Ventilation
 Once patient advances to mechanical ventilation,
probability of ventilator removal is poor, as is prognosis
 <15% of patients requiring mechanical ventilation survive
to hospital discharge (Lee, McLaughlin, & Collard, 2011)

18. Care & Treatment of Patients
with IPF
 Surgical – Lung Transplantation
 Only therapy proven to increase long-term survival
 Problems:
 Not all patients qualify for transplant
 Few hospitals have the capability for transplantation
 Donor lungs not readily available (Bharri et al., 2012)

19. Patient Education
Goal: Maintain maximal level of wellness and quality of life
 Disease Management
 Initial Teaching
 Disease Pathophysiology
 Types of Diagnostic testing, indications
 Prognosis
 Disease- and symptom-centered management
 Oxygen therapy
 Medications (indications, actions, possible complications/side
effects) (Lee, McLaughlin, & Collard, 2011)

20. Patient Education
 Supplemental teaching
 Advanced Care Planning
 Goal set within context of patient’s values and
preferences
 Initiated at a non-critical time (when death is
imminent)
 Palliative care/End-of-Life care
 Symptom control
 Relief of suffering (Lee, McLaughlin, & Collard, 2011)
 Continual Reassessment

21. Patient Education
 Cultural
 Teaching specific to language of patient
 Utilizing language-appropriate materials and
interpretive modalities
 AT&T language line
 Language Services Associates (LSA) video
communicator
 Providing care according to cultural beliefs (Lever, 2011)

22. Patient Education
 Spiritual Considerations
 Significant when dealing with advanced planning and
end-of-life care
 Encourage support of church family (if affiliated with a
church/religious organization)
 Provide pastoral care if requested
 Allow patient to express concerns and initiate
interdisciplinary modalities
NOTE: All education will utilize teach-back method to
enhance/confirm understanding.

23. References
Bhatti, H., Girdhar, A., Usman, F., Cury, J. Bajwa, A. (2013). Approach to acute
exacerbation of idiopathic pulmonary fibrosis. Annals of Thoracic
Medicine, 8(2), 71-77. doi: 10.4103/1817-1737.109815
Doyle, T. Hunninghake, G., Rosas, I. (2012). Subclinical interstitial lung disease: Why
you should care. American Journal of Respiratory and Critical Care Medicine,
185 (11), 1147-1153. doi: 10218100114
duBois, R., Weycker, D., Albera, C., Bradford, W., Costabel, U. (2011). Ascertainment
of individual risk of mortality for patients with idiopathic pulmonary
fibrosis. American Journal of Respiratory and Critical Care Medicine, 184(4),
459-466. doi: 884295098
King, T., Pardo, A., Selman, M. (2011). Idiopathic pulmonary fibrosis. The Lancet,
378(9807) , 1949-1961. doi: 910067528
Lee, J., McLaughlin, S., Collard, H. (2011). Comprehensive care of the patient with
idiopathic pulmonary fibrosis. Current Opinion in Pulmonary Medicine, 17,
348-354. doi: 10.1097/MCP.ob013e328349721b

24. Leever, M. (2011). Cultural competence: Reflections on patient autonomy
and patient good. Nursing Ethics, 18(4), 560-670.
doi: 10.1177/0969733011405936
Leslie, K. (2012). Idiopathic pulmonary fibrosis may be a disease of
recurrent, tractional injury to the periphery of the aging
lung. Archives of Pathology & Laboratory Medicine, 136(6), 591-600.
doi: 10.5858/arpa.2011-0511-OA
McCance, K., Huether, S. (2010). Pathophysiology: The biological basis for
disease in adults and children (6th ed.). Maryland Hieghts, MO:
Mosby.