12/1/2021

Interstitial Pneumonia with

Autoimmune Features (IPAF):
Interesting Case

Amornpun Wongkarnjana, MD, MSc

Division of Pulmonary and Critical Care Medicine
Department of Medicine, Chulalongkorn University
King Chulalongkron Memorial Hospital

Clinical case
• 71-year-old female teacher
• CC: Progressive dyspnea on exertion (DOE)
• First presentation January 2020: DOE for 2 months
• Breathlessness after climbing up stairs
• Atypical chest tightness
• No cough, phlegm or fever
• No joint pain or weakness
• Visited at Cardiology department
• Completed all cardiac investigations – unremarkable -> HRCT

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71-year-old female teacher

January Normal cardiac investigations April

Progressive
DOE for • Dyspnea after climbing up stairs • Could not finish stairs
2 months • Atypical chest tightness • Dry cough
• No cough, phlegm, fever Gradually worse • Dry mouth
• No systemic symptoms • Dry hands

• Normal S1S2, no edema • Bibasilar fine crackles

• Occasional end-inspiratory squeaks
• No clubbing of fingers
PH: HT, old transient ischemic stroke (2015) • Dry and cracked skins on both palms
Meds: Amlodipine 5 mg/d, Aspirin 325 mg/d
• CXR
Lifelong non-smoker, no drug allergy • HRCT
No family history of lung diseases/ cancers
No pets or significant exposure risks

71-year-old female teacher

January Normal cardiac investigations April June

Progressive
DOE for • Dyspnea after climbing up stairs • Could not finish stairs
2 months • Atypical chest tightness • Dry cough
• No cough, phlegm, fever Gradually worse • Dry mouth
• No systemic symptoms • Dry hands

• Normal S1S2, no edema • Bibasilar fine crackles

• Occasional end-inspiratory squeaks
• No clubbing of fingers
PH: HT, old transient ischemic stroke (2015) • Dry and cracked skins on both palms
Meds: Amlodipine 5 mg/d, Aspirin 325 mg/d
• CXR
Lifelong non-smoker, no drug allergy • HRCT
No family history of lung diseases/ cancers
No pets or significant exposure risks

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supine prone

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supine prone

71-year-old female teacher

• ANA negative <1:80, cytoplasmic staining
• ANA profile: Ro52 strongly positive
• RF 23 IU/ml

• HRCT: definite UIP pattern with GGO in both basal lungs

no esophageal dilatation or subcutaneous calcification

• Spirometry: FEV1 1.50 L (80% of predicted value)

FVC 1.68 L (78% of predicted value)
FEV1/FVC ratio 89%

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Interstitial Pneumonia with Autoimmune Features (IPAF)

• Individuals with both ILD and combinations of

other clinical, serologic, and/or pulmonary morphologic features
which putatively stem from an underlying systemic autoimmune
condition, but do not meet current rheumatologic criteria for a
characterized connective tissue diseases (CTD)

• Some of those individuals develop autoimmune CTD after following up

Fischer et al. Eur Respir J 2015; 46: 976–987

Fischer et al. Eur Respir J 2015; 46: 976–987

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Fischer et al. Eur Respir J 2015; 46: 976–987

• UIP was not included in the criteria

• Patients with interstitial pneumonia,
the presence of UIP pattern alone
does not increase the likelihood
of having CTD.
• Having a radiologic UIP pattern
does not exclude categorization
as IPAF neither

Fischer et al. Eur Respir J 2015; 46: 976–987

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Study from Mayo Clinic

-101/111 UCTD-ILD = IPAF (91%)

Not include Not include

ANCA UIP

Most of IPAF patients will be fit to this criteria

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71-year-old female teacher

January Normal cardiac investigations April Prednisolone May - June

Progressive
DOE for • Dyspnea after climbing up stairs • Could not finish stairs • Unchanged, stable
2 months • Atypical chest tightness • Dry cough • Add azathioprine
• No cough, phlegm, fever Gradually worse • Dry mouth 50 mg/d (ALT 24)
• No systemic symptoms • Dry hands 100 mg/d (ALT 56)
*75 mg/d (ALT 29)
• Normal S1S2, no edema

Date FVC (liter) % of predicted value Note

PH: HT, old transient ischemic stroke (2015)
Mar 2021 1.84 90% Prednisolone 5 + Aza 75 mg/d
Meds: Amlodipine 5 mg/d, Aspirin 325 mg/d
Sep 2020 1.87 88% Prednisolone 15 + Aza 75 mg/d
Lifelong non-smoker, no drug allergy
May 2020 1.59 74% Prednisolone 30->25 mg/d
No family history of lung diseases/ cancers
Jan 2020 1.68 78% -
No pets or significant exposure risks

Oldham JM et al. Eur Respir J. 2016;47(6):1767-75

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INBUILD Trial: Nintedanib in progressive fibrosing ILD (PF-ILD)

• Relative decline in FVC ≥10% predicted
• Relative decline in FVC ≥5–<10% predicted AND
(a) worsened respiratory symptoms OR
(b) increased extent of fibrosis on HRCT
• Worsened respiratory symptoms AND
increased extent of fibrosis on HRCT

Based on random coefficient regression with fixed effects for treatment, HRCT pattern (only for the overall population), and baseline FVC (mL), and terms for treatment-
by-time and baseline-by-time interactions.
Flaherty KR, et al. N Engl J Med 2019; doi: 10.1056/NEJMoa1908681.

Nintedanib and immunomodulatory therapies in

progressive fibrosing interstitial lung diseases

Cottin, V., Richeldi, L., Rosas, I. et al. Respir Res 22, 84 (2021).

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Thank You

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