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Interstitial Pneumonia with Auto-immune Features
(IPAF)Pneumopathie interstitielle avec signes auto-immuns
(PISA)
Bruno CRESTANICentre constitutif du Centre de Référence
Maladies Pulmonaires Rares
Hôpital Bichat-Claude Bernard, Université Paris Diderot
www.dhuFIRE.org
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Clinique
EFR Biologie Fibroscopie
TDM-HR
Diagnostic
Pronostic
Décision thérapeutique
+/-Biopsie Pulmonaire
Contexte
épidémiologique
Signes extra-respi
Si pas trop âgé
Ni trop sévère
Diagnostic des PID
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Idiopathiques
Cause/ContexteCause/Contexteidentifié
• Pneumoconioses
• PHS
• Médicaments
• Proliférations
• Infections/ICG
• Connectivites
SarcoïdosePID
particulières
• Histiocytose
• …
• Histiocytose
• LAM
• Lipoprotéinose
• Eosinophiles
• …Fréquentes
•FPI
• PINS
• PID/PID-BR
• POC
• PIA
Rares•PIL
• PPFE
Classification étiologique des PID (Travis, AJRCCM 2013)
PISA (IPAF) • Inclassables
Fischer, Eur Respir J 2015
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Grands cadres étiologiques devant une fibrose pulmonaires
Connectivites
PHSParticules(amiante)
Médic.Idiopathiques
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Pourquoi rechercher une connectivite
Connectivite
• Biopsie généralement
non indiquée
• Corticoïdes et/ou
immunosuppresseurs
• Evolution plus lente
• Pronostic meilleur
(sauf PR)
FPI
• Biopsie parfois indiquée
• Contreindication aux CS
et immunosuppresseurs
• Traitement anti-fibrosant
• Pronostic médiocre
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• Clinical features associated with CTD in a patient with ILD
– Gender : female
– Younger age
– Detection of Auto-Abs
– HRCT : NSIP, OP
(UIP is possible in CTD-ILD)
(Moua, Respir Res 2014)
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CTD influences the prognosis
Park, AJRCCM 2007
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(Park, AJRCCM 2007)
Histology does not inform prognosis in CTD(except RA)
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Strand, Chest 2014
CTD-UIP has a better prognosis than idiopathic UIP (IPF)
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Strand, Chest 2014
CTD-UIP prognosis is influenced by the CTD
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Histology informs prognosis
Omote, Chest 2015Lung dominant connective tissue disease
P=0.031
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Histology informs prognosis in terms of treatment response
Omote, Chest 2015Lung dominant connective tissue disease
Treatment
h-UIP h-NSIP
corticosteroids
52% 69%
ciclosporin 44% 62%
pirfenidone 20% 0%
No Tt 44% 31%
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Final diagnosis in MDD informs prognosis
Omote, Chest 2015Lung dominant connective tissue disease
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Ex de Discussion Multi-Disciplinaire:
• PID suggérant une PINS/PO
• Auto-anticorps– ANA 1/320,
– anti-Sp100+
• Histologie : PINS fibrosante
• Idiopathic NSIP ?
• Forme fruste of CTD ?
• Lung-dominant CTD ?
• Undifferentiated CTD ?
• Auto-immune featured ILD ?
• Unclassifiable Lung Fibrosis ?
Que faire des patietns sans connectivite définie?
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Undifferentiated CTD criteria : no consensus
(Corte, ERJ 2012)
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Undifferentiated CTD criteria : no consensusDiagnosis depends on criteria
NSIP(n=45)
IPF(n=56)
Strict(Mosca, J Rheumatol 2002)
21% 13%
Loose(Kinder, AJRCCM 2007)
71% 36%
(Corte, ERJ 2012)
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Fischer, Eur Respir J 2015
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Idiopathic ILD(IPF, NSIP)
CTD
Chronic HP
• “Forme fruste” of CTD
• Lung-dominant CTD
• Undifferentiated CTD
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Idiopathic ILD(IPF, NSIP)
CTD
Chronic HP
IPAF(PISA)
Interstitial Pneumonia with Auto-immune Features (IPAF)Pneumopathie interstitielle avec signes auto-immuns (PISA)
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Idiopathic
Known cause
• Pneumoconiosis
• HP
• Drug-induced
• Proliferative dis.
• Infections/LVF
• CTD
SarcoidosisSpecific
ILDs
• Histiocytosis
• …
• Histiocytosis
• LAM
• PAP
• Eosinophilic
• …Frequent
•IPF
• NSIP
• DIP/RB-ILD
• COP
• AIP
Rare•LIP
• PPFE
Classification of ILDs(Travis, AJRCCM 2013)
IPAF Unclassifiable• Unclassifiable
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IPAF Criteria
1. Presence of an interstitial pneumonia (HRCT or
Biopsy)
2. Exclusion of alternative etiologies
3. Does not meet criteria of a definite CTD
4. At least one feature, from at least two of three
domains
Clinical
Serological
Morphological
Fischer, Eur Respir J 2015
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Fischer, Eur Respir J 2015
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Fischer, ERJ 2015
Mechanic hands
Gottron’s sign
Palmar telangiectasia
Digital edema
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Fischer, Eur Respir J 2015
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Fischer, Eur Respir J 2015
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MDD:
• ILD highly suggestive of
NSIP/OP
• Auto-Abs but No definite CTD
• Histology : fibrotic NSIP
Final diagnosis : IPAF
Does it help prognosis and
Treatment definition ?
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Table 1. IPAF Cohort Baseline Demographic and
Clinical Characteristics (n=144)
Age, mean (±SD) 63.2 (11)
Female Gender, n (%) 75 (52.1)
Gastroesophageal reflux, n (%) 76 (52.8)
Ever Smoker, n (%) 79 (54.9)
Crackles, n (%) 125 (89.3)
Clubbing, n (%) 21 (18.9)
UIP by HRCT, n (%) 77 (54.6)
UIP by SLB, n (%) 61 (73.5)
FVC (% predicted) 61.9 (18.3)
DLCO (% predicted) 45.3 (20.6)
Oldham JM et al, Eur Respir J 2016
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Initial diagnosis
Oldham JM et al, Eur Respir J 2016
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Survival by diagnosis
CTD-ILD
IPAF
IPF
Oldham JM et al, Eur Respir J 2016
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Survival by diagnosis
CTD-ILD
IPAF-non UIP
IPF
IPAF-UIP
Oldham JM et al, Eur Respir J 2016
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Survival by diagnosis
Ahmad, Respir Med 2019
N=57
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Survival by diagnosis
Kelly, Respirology 2018
N=101
IPF
IPAFUIP
IPAFnon UIP
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Série chinoise
Dai, Clin Rheumatol 2018
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Série chinoise
Dai, Clin Rheumatol 2018
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Stratégie thérapeutique
Wiertz, Eur Respir J 2018
HRCT pattern ?
Histo pattern :
fibrosis with OP
in 5/6
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• The concept of IPAF is important
• Fits to the real life
• Treat according to the HRCT/Histopattern ?
– UIP anti-fibrotics
– Non UIP steroids and I°S
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Patient
with
CTD
Pulmonologist Rheumatologist
InternalMedicine
Other specialists
Protect the lung
Improve QoL
Improve Survival
Radiologist Pathologist
The Multidisciplinary approach
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Mrs S. 55 yrs
• Non smoker
• Shortness of breath since 2 yrs
• No extra-respiratory symptoms, no exposure
• Biological screen normal
• Minor salivary glands biopsy : Chisholm 1
• FVC 56%, DLCO 39%
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Probable NSIP with OP
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Auto-immune screen :
• ANA 1/320, nuclear
dots;
• anti-Sp100+Rare pattern of fluorescence
• Primary Biliary Cirrhosis
(particularly AMA negative)
• Other CTD (SLE, Sjögren,
SSc)
Anti-Sp100 is the ELISA
counterpart
Nuclear dots (with membrane rim)
(Granito, Aliment Pharmcol Therap 2006)
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Endoscopy
• BAL: Ly 18%; Neutro : 6%
• Bronchial biopsies <0
• Trans-bronchial biopsies :
mild inflammation
Mrs S. 55 yrs
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Multi-Disciplinary Discussion:
• ILD highly suggestive of NSIP/OP
• Auto-Abs but No definite CTD
Biopsy or not ?
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• An NSIP pattern on HRCT in an idiopathic
context: 30% are UIP at histology
– Travis, AJRCCM 2013
• Histology informs prognosis in the context of
“Lung-dominant CTD”
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Detection of auto-Abs does not influence the prognosis in an idiopathic context
Brauer, Respir Med 2015
CTD-ILD
Idiopathic ILD with/without Auto-Abs
Auto-Abs tested : ANA, RF, anti-ENA
P=0.001
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Multi-Disciplinary Discussion:
• ILD highly suggestive of NSIP/OP
• Auto-Abs but No definite CTD
Surgical lung biopsy: fibrotic NSIP with
follicular bronchiolitis; no OP