12
Figures
ILDs other than IPF
Idiopathic interstitial
pneumonia (IIP)
Autoimmune-ILDs
Unclassifiable
Myositis
b
Sjögrens
Vasculitis
Systemic lupus
erythematosus
(SLE)
Others
Eosinophilic
a
e shaded area ( ) represents the committee's estimate of the
proportion of patients with various types of ILD. Note that
IPF is not included in the figure because it is excluded from
the definition of PPF. e proportion of patients with ILDs
other than IPF who manifest PPF is based on the consensus of
opinions and the perception of the international committee.
ere are no data to provide the exact or estimated proportion
of patients manifesting PPF in ILDs, other than IPF.
Figure 4. ILD Manifesting PPF
Idiopathic
pleuroparenchymal
fibroelastosis (iPPFE)
Idiopathic desquamative
interstitial pneumonia
(iDIP)
Acute interstitial
pneumonia (AIP)
Cryptogenic
organizing
pneumonia (COP)
Idiopathic lymphoid
interstitial
pneumonia (iLIP)
Systemic sclerosis
(SSc)
Acute fibrinous and
organizing pneumonia
(AFOP)
Idiopathic non-specific
interstitial pneumonia
(iNSIP)
Rheumatoid
arthritis (RA)
Mixed connective
tissue disease
(MCTD)
