Welcome to our new #accredited #Tweetorial on idiopathic pulmonary fibrosis (IPF), part of our ongoing micro-learning series on interstitial lung disease and pulmonary fibrosis.

🫁 This activity provides 0.25 AMA PRA Category 1 Credits™

Supported by an independent educational grant; delivered in joint providership with CCI.

#IPF #PulmonaryFibrosis #ILD #CME

📄Accreditation and disclosures 👉 click here

Supported by an independent educational grant from Bristol Myers Squibb and delivered in joint providership with CCI.

So… how confident are you in recognising IPF early, distinguishing UIP from IPF, and applying antifibrotic therapy in practice? 🤔

Stay tuned 👇

🎥 In this clip, Prof. Corey Kershaw clarifies what truly defines idiopathic pulmonary fibrosis (IPF).

🫁 IPF requires a UIP pattern on HRCT or histology and the absence of an identifiable cause, including environmental exposures, drug toxicity, or connective tissue disease.

📌 IPF is a diagnosis of exclusion, not a CT finding alone.

Because IPF is a diagnosis of exclusion, history is not optional.

A high-quality ILD history must assess:

• occupational & environmental exposures
• smoking history
• medications with pulmonary toxicity
• autoimmune symptoms (Raynaud’s, rashes, arthritis, dysphagia)

🧪 Autoantibodies alone don’t diagnose CTD-ILD.

🖥️ High-resolution CT (HRCT) is pivotal once secondary causes are addressed.

HRCT patterns are classified as:

• definite UIP
• probable UIP
• indeterminate for UI
• alternate diagnosis

🧩 Definite or probable UIP, in the right clinical context, can establish IPF without surgical biopsy.

🎥 Look at a typical UIP pattern shared by Prof. Corey Kershaw in this clip.

Modern IPF guidelines emphasise restraint. When a cause was not identified:

✔️ Definite UIP → very high histologic concordance → no surgical biopsy
✔️ Probable UIP → high histologic concordance → no surgical biopsy

🧠 Remember: UIP ≠ IPF.

Only UIP without an identifiable cause meets criteria for IPF.

🛑 Biopsy is reserved for cases where results are expected to change management, not confirm what is already clear.

🎥 Listen to Prof. Corey Kershaw discussing about what the guidelines suggest when interpreting HRCT in suspected IPF.

Pulmonary fibrosis is driven by dysregulated wound repair, not persistent inflammation alone.

Key mechanisms include:

• Epithelial injury
• Myofibroblast differentiation, leading to excessive collagen deposition
• Profibrotic signaling pathways, including TGF-β, PDGF, and downstream cytokines
• Extracellular matrix accumulation, which progressively restricts lung function

🧬 This biology explains why fibrosis is often irreversible, and why therapy focuses on slowing progression.

🧬 Antifibrotic therapy in IPF, mechanisms matter
🎯 Different pathways. Same goal: slow progression.

Fibrosis evolves through a cascade:

epithelial injury → immune activation → fibroblast recruitment → myofibroblast transformation → matrix deposition.

New therapies target two levels:

🟦 Immunomodulatory (upstream)
→ macrophages (e.g. tocilizumab)
→ lymphocytes (cyclophosphamide, mycophenolate, abatacept)
→ B cells (rituximab)

🟩 Fibromodulatory (downstream)
→ TGF-β signalling
→ fibroblast–myofibroblast transition
→ extracellular matrix accumulation

🎯 Different entry points. Same goal: slow or stop fibrotic progression.

🎥 Listen to Prof. Corey Kershaw explaining how our growing understanding of this cascade has opened the door to multiple therapeutic targets.

🧪 Key emerging therapies in pulmonary fibrosis

🟦 Admilparant (LPA₁ receptor antagonist)
→ blocks LPA-driven fibroblast migration

📊 Phase 2: slowed FVC decline in IPF & PPF

⏳ Phase 3 (IPF + PPF) nearing completion

🟩 Inhaled treprostinil
→ antifibrotic effects beyond PAH (↓ TGF-β–driven collagen, ↓ fibrocyte recruitment)

📊 INCREASE & TETON-2: slowed FVC decline over 52 wks

🔄 TETON-1 & TETON-PPF ongoing

🎯 Targeting fibrosis from new angles, results soon.

“Am I going to feel better?”

Approved antifibrotics:

✔️ slow the rate of lung-function decline

✔️ reduce the risk of rapid progression

❌ They do not reliably improve symptoms like cough or dyspnoea.

High-quality care includes:

🫁 screening for comorbidities (pulmonary hypertension, reflux, sleep apnoea)

🗣️ actively managing cough (often the biggest quality-of-life burden)

🏃 pulmonary rehab and symptom-directed support for dyspnoea

🧠 addressing anxiety, depression, and social impact

🤝 early use of palliative care and patient support groups

🎯 Treat the whole person, not just the fibrosis.

🧠 Managing side effects of antifibrotics = preserving persistence
🎯 Proactive AE management = better adherence & outcomes.

IPF care is a journey, not a prescription.

🩺 Diagnose by exclusion
⏳ Treat early to slow decline
🤝 Support persistence & quality of life
🎯 Precision in diagnosis enables precision in care.

Which challenge do you face most in IPF care: diagnostic delay, treatment tolerance, or persistence?
#IPF #PulmonaryFibrosis #CME

Thank you for joining this #accredited #Tweetorial on idiopathic pulmonary fibrosis (IPF).

🧾 Claim your 0.25 CME credit ➡️ https://forms.gle/VpiQWZCYRTzWM7vT6

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