Kevin R. Flaherty, MD, MS, prepared progressive fibrosing ILD infographics for this CME activity titled "Progressive Fibrosing Interstitial Lung Disease: Shining a Light on the Latest Clinical Advances." For the full presentation, monograph, complete CME information, and to apply for credit, please visit us at http://bit.ly/2LAetT6. CME credit will be available until August 12, 2019.

1. Historically Current Emphasis on Diagnosis
Looking Toward the Future:
Combining Diagnosis and Behavior
Many ILDs grouped
into general category
of pulmonary
fibrosis
Different subgroups ž different responses
Response in NSIP > UIP
Response ž better prognosis
Why Do Some Patients
Respond to Steroids?1-3
UIP confers a poor prognosis vs NSIP or RB-ILD/DIP3
Baseline DLCO and change in FVC associated with poor
prognosis independent of diagnosis of IPF or NSIP5
Diagnosis, Prognosis, and Disease Behavior
• IPF
• NSIP
• AIP
• RB-ILD
• DIP
• COP
Revised ATS/ERS Classification
of Major IIPs4
ATS/ERS Categorization of Major IIPs4
Clinico-Radiologic-
Pathologic Diagnosis
Major Radiology-
Pathology Patterns
Chronic Fibrosing
IPF
UIP
Idiopathic
NSIP
NSIP
Smoking-Related
RB-ILD
RB
DIP
DIP
Acute/Subacute
COP
Organizing
pneumonia
AIP
Diffuse
alveolar
damage
IPF
PF-ILD
NSIP
HP
UC
CTD-
ILD
Stable/Improved
Progressive Fibrosing Interstitial Lung Disease: Shining a Light on the Latest Clinical Advances
Full references, accreditation, and disclosure information available at www.peerview.com/FMH930.
Conceptualizing Progressive Fibrosing ILD

2. Progressive Fibrosing ILDs May Behave Like IPF
Antifibrotics may ameliorate
decline in lung function
Immunosuppressants not
effective once fibrosis present
Hypotheses in Treatment
of Non-IPF ILDs
?
?
Clinical Trials of
Antifibrotics in
Non-IPF Diseases
Nintedanib
Pirfenidone
Recurring microinjuries
Alveolar
epithelium
Capillaries
Abnormal Wound Healing Model of IPF15
Progressive Fibrosing Interstitial Lung Disease: Shining a Light on the Latest Clinical Advances
Full references, accreditation, and disclosure information available at www.peerview.com/FMH930.
Similarities Between IPF and Other Progressive Fibrosing ILDs
Similar survival6
Lone ILD CTD-ILD
Acute exacerbations
can develop
in RA-ILD9
LAM, PF-ILD
SSc-ILD16-19
RA-ILD, fibrotic HP,
PF-ILD, SSc-ILD20-24
Acute exacerbations
can develop in
fibrotic HP14
Evidence in
CTD-ILD6-9
Evidence in
HP10-14
Similar survival to IPF7
Worse prognosis vs
indeterminate or NSIP7
RA-ILD RA-ILD
+ UIP
Worse prognosis vs most
other histopathologies8
RA-ILD RA-ILD
+ UIP
Acute
Chronic
UIP pattern10
Fibrosis on biopsy
(≥5% of surface area)11,12
Fibrotic features of
HRCT (UIP,
honeycombing, traction
bronchiectasis)13
Poor Prognosis in HP
Associated With …
Case Studies:
Acute vs Chronic/Fibrotic HP
Non-IPF
ILDs
IPF
Fibrosis