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DIAGNOSTIC NOTE TEMPLATE SOAP NOTE TEMPLATE WHEN CONSIDERING A DIAGNOSIS OF IDIOPATHIC PULMONARY FIBROSIS (IPF) CHIEF COMPLAINT HISTORY OF PRESENT ILLNESS Consider IPF as possible diagnosis if any of the following applies 1 >50 years of age with any of the following Chronic cough Dyspnea upon exertion History of smoking SUBJECTIVE MEDICAL HISTORY Medical Conditions It is important to rule out other causes of ILD such as connective tissue disease-related interstitial lung disease, chronic hypersensitivity pneumonitis, sarcoidosis, and radiation- and drug-induced lung disease 1 The following comorbidities are common in patients with IPF 2 * Cardiovascular disease Chronic obstructive pulmonary disease Gastroesophageal reflux disease Lung cancer Metabolic disorders Obstructive sleep apnea Pulmonary arterial hypertension Pulmonary embolism *Listed in alphabetical order Medications Inquire specifically about pneumotoxic drugs 3 SMOKING HISTORY Consider IPF as a possible diagnosis if patient presents with a smoking history 1 FAMILY HISTORY Consider IPF as a possible diagnosis if patient presents with a family history of IPF 1 SOCIAL HISTORY Ensure work history and environmental exposures are discussed Ensure work to history rule out and other environmental causes of ILDexposures 1 are discussed to rule out other causes of ILD 1 ESB/040615/0145(2)g(1) 12/17

VITAL SIGNS SEROLOGICAL TESTING Consider IPF as a possible diagnosis if autoimmune workup is negative and other features consistent with IPF are positive 1 LUNG AUSCULTATION Consider IPF as a possible diagnosis if patient demonstrates inspiratory, bibasilar Velcro -like crackles 1 Lung Volume Flow Gas Exchange TLC FEV 1 DL CO FVC FEV 1 /FVC OBJECTIVE LUNG FUNCTION Consider IPF as a possible diagnosis if patient demonstrates the following lung function results Reduced TLC and/or FVC 1 FEV 1 /FVC consistent with restrictive pattern NOTE In certain situations, IPF patients may exhibit normal or obstructive patterns if concomitant obstructive disease exists. Therefore, while a restrictive pattern is indicative of IPF, lacking a restrictive pattern does not preclude an IPF diagnosis. 1 Reduced DL co TLC = total lung capacity; FEV = forced expiratory volume in 1 second; FVC = forced vital capacity; DL co = diffusing capacity of lung for carbon monoxide Oxygen Saturation with Exertion (6-MWT) Oxygen Saturation at Rest EXERCISE CAPACITY Consider IPF as a possible diagnosis if patient demonstrates the following exercise testing results Low oxygenation 6-MWT = 6-minute walk test RADIOGRAPHIC ASSESSMENT ***Order chest HRCT when considering IPF*** For quality imaging, ensure HRCT is conducted using proper technique 4 Slice thickness 2 mm Noncontrast conditions Full inspiration without respiratory motion Field of view to include lungs only High-resolution reconstruction algorithm List not exhaustive. Please see reference 4. Consider IPF if HRCT shows a pattern of usual interstitial pneumonia (UIP) 1 Predominant subpleural distribution Reticular abnormalities Honeycombing Absence of features inconsistent with UIP In cases of possible UIP*, consider pathological assessment * The ATS diagnostic guidelines are based on categorization of HRCT patterns such as UIP, possible UIP, and inconsistent with UIP. The Fleischner Society has since released a white paper with revised categories typical UIP, probable UIP, and indeterminate for UIP. The white paper states that a confident diagnosis of IPF can be made in the correct clinical context with a radiographic pattern of typical or probable UIP. The ATS guidelines may be updated to reflect the revised categories. 5

DIAGNOSIS Include IPF in your differential diagnosis if other known causes have been excluded and a UIP pattern is present on HRCT 1 ASSESSMENT/PLAN MANAGEMENT PLAN Initiate a comprehensive management plan and consider the following Pharmacologic treatment and nonpharmacologic treatments are important to comprehensive patient management 6 FDA-approved, IPF-specific therapy; management of comorbidities; symptom management; clinical trials enrollment 6-8 Supplemental oxygen, pulmonary rehabilitation, disease education, psychosocial support, evaluation for lung transplant, and palliative care 1 Consider comorbidities and concomitant medications when selecting the most appropriate management plan 7 Manage comorbidities as medically appropriate 7 Monitor disease progression at least every 6 months or as medically appropriate 1 A collaborative conversation with your patients can encourage them to follow their management plan 9 Important information to communicate when discussing IPF with your patients includes - The patient s lung function will deteriorate at any time, so intervening upon diagnosis is crucial 10,11 - Educate patients on management options for IPF 6 - Adhering to a management plan is important to managing IPF and associated comorbidities 7 Provide support for caregivers 7 Schedule next follow-up References 1. Raghu, et al. Am J Respir Crit Care Med. 2011;183788-824. 2. Raghu, et al. Eur Respir J. 2015;461113-1130. 3. Mayo Clinic website. http//www.mayoclinic.org/diseases-conditions/interstitial-lung-disease/basics/causes/con-20024481. Accessed December 4, 2017. 4. Raghu, et al. Am J Respir Crit Care Med. 2011;183788-824 (online supplement). 5. Lynch, et al. Published online ahead of print November 15, 2017. Lancet Respir Med. doi 10.1016/S2213-2600(17)30433-2. Accessed December 4, 2017. 6. Raghu, et al. Am J Respir Crit Care Med. 2015;192e3-e19. 7. Lee, et al. Curr Opin Pulm Med. 2011;17348-354. 8. Padilla. Am J Manag Care. 2015;21(suppl 14)s276-s283. 9. Pulmonary Fibrosis Foundation. Pulmonary Fibrosis Patient Information Guide. Chicago, IL Pulmonary Fibrosis Foundation; 2015. 10. Cottin and Richeldi. Eur Respir Rev. 2014;23106-110. 11. Ley, et al. Am J Respir Crit Care Med. 2011;183431-440.

FOLLOW-UP NOTE TEMPLATE SOAP NOTE TEMPLATE WHEN MANAGING A PATIENT WITH IPF CHIEF COMPLAINT HISTORY OF PRESENT ILLNESS Evaluate for change in symptoms and oxygen requirement, and determine whether these are due to IPF- or non-ipf-related causes 1 Medical Conditions Medications SUBJECTIVE MEDICAL HISTORY Any updates since last visit? SMOKING HISTORY If patient is a current smoker, stress the benefits of quitting 2 FAMILY HISTORY Any updates since last visit? SOCIAL HISTORY

VITAL SIGNS LUNG AUSCULTATION Any change since last visit? Presence of inspiratory, bibasilar Velcro -like crackles? Lung Volume Flow Gas Exchange TLC FEV 1 DL CO OBJECTIVE LUNG FUNCTION FVC FEV 1 /FVC Monitor every 3-6 months or as medically appropriate 1 TLC = total lung capacity; FEV = forced expiratory volume in 1 second; FVC = forced 1 vital capacity; DL CO = diffusing capacity of lung for carbon monoxide Oxygen Saturation with Exertion (6-MWT) Oxygen Saturation at Rest EXERCISE CAPACITY Monitor every 3-6 months or as medically appropriate 1

DIAGNOSIS Any change in disease status or new diagnoses since last visit? ASSESSMENT/PLAN MANAGEMENT PLAN Continue to implement a comprehensive management plan and consider the following Pharmacologic treatment and nonpharmacologic treatments are important to comprehensive patient management 3 FDA-approved, IPF-specific therapy; management of comorbidities; symptom management; clinical trials enrollment 2-4 Supplemental oxygen, pulmonary rehabilitation, disease education, psychosocial support, evaluation for lung transplant, and palliative care 1 Consider comorbidities and concomitant medications when selecting the most appropriate management plan 2 Manage comorbidities as medically appropriate 2 Monitor disease progression at least every 6 months or as medically appropriate 1 A collaborative conversation with your patients can encourage them to follow their management plan 5 Important information to communicate when discussing IPF with your patients includes - The patient s lung function will deteriorate at any time, so intervening upon diagnosis is crucial 6,7 - Educate patients on management options for IPF 3 - Adhering to a management plan is important to managing IPF and associated comorbidities 2 Provide support for caregivers 2 Schedule next follow-up References 1. Raghu, et al. Am J Respir Crit Care Med. 2011;183788-824. 2. Lee, et al. Curr Opin Pulm Med. 2011;17348-354. 3. Raghu, et al. Am J Respir Crit Care Med. 2015;192e3-e19. 4. Padilla. Am J Manag Care. 2015;21(suppl 14)s276-s283. 5. Pulmonary Fibrosis Foundation. Pulmonary Fibrosis Patient Information Guide. Chicago, IL Pulmonary Fibrosis Foundation; 2015. 6. Cottin and Richeldi. Eur Respir Rev. 2014;23106-110. 7. Ley, et al. Am J Respir Crit Care Med. 2011;183431-440.