Respiratory : Interstitial Lung disease (Complete)

• Basics: What Is Interstitial Lung Disease? | Assessing Suspected ILD
• Fibrotic lung disease: Idiopathic Pulmonary Fibrosis | Acute Exacerbation of IPF | Progressive Pulmonary Fibrosis | Non-specific Interstitial Pneumonia
• ILD with a cause: Hypersensitivity Pneumonitis | Connective Tissue Disease ILD | Drug- and Radiation-induced Lung Disease | Occupational Lung Disease | Smoking-related ILD
• Other ILDs: Organising Pneumonia | Eosinophilic Lung Disease | Cystic Lung Diseases | Pulmonary Alveolar Proteinosis
• Sarcoidosis: Sarcoidosis: What It Is | Clinical Features of Sarcoidosis | Diagnosing Sarcoidosis | Treating Sarcoidosis
• Revision: Drug Reference Table | Spot the Diagnosis | Exam-style Scenarios | Key Points | Guidelines and Further Reading

🧬 Interstitial lung disease (ILD) is a group of over 200 conditions that inflame or scar the lung tissue around the air sacs, making the lungs stiff and gas exchange poor. Some have a clear cause (birds, drugs, dusts, connective tissue disease); others, like idiopathic pulmonary fibrosis, do not. This chapter also covers sarcoidosis, a granulomatous disease that can affect the lungs and almost any other organ.
Your four questions for every patient: Is there a cause or exposure (drugs, birds, moulds, dusts, autoimmune disease)? What does the HRCT pattern show? Is it inflammatory (may respond to steroids) or fibrotic (needs antifibrotics)? Is it progressing?

What Is Interstitial Lung Disease?

Mechanism: The interstitium is the thin tissue between the air sacs and the capillaries. In ILD it becomes inflamed or scarred (fibrosis). This makes the lungs:

• Stiff - small, restricted lung volumes.
• Poor at gas exchange - low TLCO and oxygen levels, worse on exercise.

A practical classification:

• Known cause:
  • Hypersensitivity pneumonitis.
  • Connective tissue disease.
  • Drugs and radiation.
  • Occupational dusts (asbestosis, silicosis).
  • Smoking-related.
• Idiopathic interstitial pneumonias:
  • Idiopathic pulmonary fibrosis (the commonest).
  • Non-specific interstitial pneumonia.
  • Cryptogenic organising pneumonia.
  • Others.
• Granulomatous: Sarcoidosis.
• Rare: Lymphangioleiomyomatosis, Langerhans cell histiocytosis, alveolar proteinosis, eosinophilic pneumonias.

Clinical pattern:

• Progressive exertional breathlessness.
• Dry cough.
• Fine "Velcro" crackles (especially at the bases in fibrosis).
• Clubbing (IPF, asbestosis).
• Desaturation on walking.
• Late: right heart failure (pulmonary hypertension).

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Assessing Suspected ILD

• History - exposures are key:
  • Drugs - past and present (methotrexate, amiodarone, nitrofurantoin, chemotherapy, immunotherapy).
  • Birds (pigeons, budgies, feather duvets), moulds (damp homes, hot tubs, humidifiers).
  • Occupation - asbestos, silica, coal, metals, farming.
  • Smoking.
  • Radiotherapy.
  • Family history (familial pulmonary fibrosis).
• Connective tissue disease symptoms:
  • Joint pain; Raynaud's; skin thickening.
  • Rash, mechanic's hands.
  • Muscle weakness; dry eyes and mouth; dysphagia.
• Tests:
  • HRCT - the key test.
  • Full lung function - restrictive pattern, low TLCO.
  • 6-minute walk test (desaturation).
  • Bloods: FBC, U&E, LFTs, CRP; autoimmune screen (ANA, ENA, rheumatoid factor, anti-CCP, myositis antibodies including anti-Jo-1, ANCA); serum precipitins (bird, mould) if hypersensitivity pneumonitis is possible.
  • Echo if pulmonary hypertension is suspected.
• Bronchoalveolar lavage - lymphocytosis suggests hypersensitivity pneumonitis or sarcoidosis; also excludes infection.
• Lung biopsy (transbronchial cryobiopsy or surgical) - only when the diagnosis is still uncertain and the result would change treatment.
• ILD multidisciplinary team (MDT) discussion - respiratory physicians, radiologists and pathologists agree the diagnosis. This is the gold standard.

💡 Clinical Pearl: Before labelling anyone with "idiopathic" pulmonary fibrosis, actively look for a cause - a hidden bird exposure, a drug, or an early connective tissue disease. Finding one changes treatment and prognosis.

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Idiopathic Pulmonary Fibrosis

Mechanism: Progressive scarring of the lungs with no known cause, thought to result from repeated injury to the alveolar lining with abnormal healing. It is the commonest and most serious idiopathic ILD. Median survival from diagnosis is about 3–5 years - worse than many cancers.

Who: Usually over 60; more common in men and ex-smokers; some cases are familial (e.g. telomere gene mutations).

Clinical pattern:

• Slowly progressive breathlessness; dry cough.
• Bibasal fine crackles.
• Clubbing (about half).
• Associated with reflux, lung cancer and pulmonary hypertension.

Diagnosis:

• HRCT with a usual interstitial pneumonia (UIP) pattern:
  • Basal and subpleural reticulation.
  • Honeycombing.
  • Traction bronchiectasis.
  • Little ground-glass.
• Exclusion of known causes.
• MDT agreement. Biopsy is rarely needed when the HRCT pattern is typical.

Management:

• Antifibrotic drugs slow the decline in FVC by about half - they do not reverse scarring:
  • Pirfenidone or nintedanib for people with FVC 50–80% predicted (NICE TA504, TA379).
  • Nintedanib is now also recommended when FVC is above 80% (NICE TA864).
  • Pirfenidone side effects: photosensitivity rash, nausea, weight loss. Nintedanib side effects: diarrhoea, raised LFTs.
  • Monitor LFTs.
  • NICE stopping rules apply for some indications (e.g. FVC decline of 10% or more in 12 months).
• Do not use steroids or immunosuppressants for stable IPF - they cause harm.
• Supportive care:
  • Pulmonary rehabilitation.
  • Oxygen (ambulatory and long-term) for hypoxaemia.
  • Smoking cessation; vaccinations.
  • Treat reflux if symptomatic; manage cough.
  • Screen for pulmonary hypertension and lung cancer.
• Lung transplantation for suitable patients - refer early (usually under about 65).
• Palliative care - breathlessness and cough management, advance care planning. Many patients die of respiratory failure.

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Acute Exacerbation of IPF

• What it is: A sudden worsening of breathlessness over less than a month, with new bilateral ground-glass change on CT on top of the fibrosis. Not explained by heart failure or fluid overload.
• Triggers: Infection, surgery, bronchoscopy or biopsy, often none.
• Prognosis: Very poor - about half die in hospital.
• Management:
  • Exclude other causes - infection, PE, heart failure, pneumothorax.
  • Oxygen (often high-flow); antibiotics while infection is excluded.
  • High-dose steroids are often given, though evidence is weak.
  • Continue antifibrotics.
  • Early discussion of goals of care - mechanical ventilation rarely helps unless it is a bridge to transplant.

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Progressive Pulmonary Fibrosis

• What it is: Any fibrotic ILD other than IPF that keeps getting worse despite treatment of the cause. Examples include fibrotic hypersensitivity pneumonitis, connective tissue disease ILD and fibrotic NSIP.
• Definition - two or more of these in a year:
  • Worse symptoms.
  • Falling lung function (e.g. FVC down by 5% or more, or TLCO down by 10% or more).
  • More fibrosis on CT.
• Treatment: Nintedanib slows FVC decline (NICE TA747), alongside treatment of the underlying condition.

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Non-specific Interstitial Pneumonia

• What it is: A pattern of more even, uniform inflammation and fibrosis - often in younger people and women.
• Causes: Frequently linked to connective tissue disease (especially systemic sclerosis and myositis), drugs or hypersensitivity pneumonitis. It can also be idiopathic.
• HRCT: Bilateral basal ground-glass with fine reticulation, often sparing the area just under the pleura; little or no honeycombing.
• Treatment: Treat the cause. Immunosuppression (prednisolone, mycophenolate) often helps - unlike IPF.
• Prognosis: Better than IPF, especially in the inflammatory (cellular) form.

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Hypersensitivity Pneumonitis

Mechanism: An immune reaction in the small airways and alveoli to an inhaled organic antigen in a sensitised person.

Common causes:

• Birds - bird fancier's lung (pigeons, budgies, feather duvets and pillows).
• Mouldy hay or grain - farmer's lung.
• Moulds - damp homes, humidifiers, air conditioning, hot tubs.
• Metalworking fluids; some drugs.
• No cause is found in up to half.

Types:

• Non-fibrotic (inflammatory): Episodes of cough, breathlessness, fever and flu-like symptoms 4–8 hours after exposure, or gradual onset. Often reversible.
• Fibrotic: Chronic progressive scarring - can mimic IPF, and has a worse outlook.

Diagnosis:

• Exposure history - ask specifically about birds, feathers and damp.
• HRCT: Ground-glass, centrilobular nodules, mosaic attenuation with air trapping (the "three-density" sign); upper and mid zones.
• Serum precipitins (specific IgG) - show exposure, not disease.
• BAL lymphocytosis.
• Biopsy if uncertain.

Management:

• Avoid the antigen completely - the most important step (remove birds, feather bedding; fix damp).
• Prednisolone for significant disease; mycophenolate or azathioprine to spare steroids.
• Nintedanib if progressive fibrosis.

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Connective Tissue Disease ILD

• Systemic sclerosis:
  • ILD affects up to half - the leading cause of death in this disease.
  • Usually an NSIP pattern; associated with anti-Scl-70 (topoisomerase) antibodies.
  • Also pulmonary arterial hypertension (limited disease, anticentromere) - screen yearly.
  • Treatment: Mycophenolate (or cyclophosphamide); tocilizumab or rituximab in selected cases; nintedanib for progression.
• Rheumatoid arthritis:
  • ILD (often UIP pattern, especially in male smokers).
  • Also bronchiectasis, pleural effusions (very low glucose), rheumatoid nodules.
  • Caplan's syndrome - nodules with coal or silica exposure.
  • Drug toxicity - methotrexate, and infection risk with biologics.
• Inflammatory myositis (especially antisynthetase syndrome - anti-Jo-1):
  • ILD, myositis, arthritis.
  • Mechanic's hands, Raynaud's, fever.
  • Anti-MDA5 dermatomyositis can cause rapidly progressive ILD.
  • Treatment: Steroids plus immunosuppression.
• Sjögren's syndrome: Lymphocytic interstitial pneumonia, cysts, small airways disease, lymphoma.
• SLE:
  • Pleurisy and effusions (commonest).
  • Acute lupus pneumonitis.
  • Alveolar haemorrhage.
  • "Shrinking lung syndrome" (diaphragm dysfunction).
  • PE (antiphospholipid syndrome).

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Drug- and Radiation-induced Lung Disease

Common culprits (pneumotox.com lists hundreds):

• Methotrexate - acute or subacute pneumonitis (fever, cough, breathlessness); can occur at any dose.
• Amiodarone - pneumonitis and fibrosis; dose- and duration-related. High-density lungs and liver on CT. Monitor lung function and chest X-ray.
• Nitrofurantoin - acute reaction (fever, eosinophilia) or chronic fibrosis with long-term use (e.g. UTI prophylaxis).
• Chemotherapy - bleomycin (made worse by high oxygen), busulfan, cyclophosphamide.
• Immune checkpoint inhibitors (pembrolizumab, nivolumab) - immune-related pneumonitis.
• Biologics and others - anti-TNF, rituximab, leflunomide, sulfasalazine.

Radiation pneumonitis: 1–6 months after thoracic radiotherapy, with breathlessness, cough and shadowing that matches the radiation field. It can progress to fibrosis.

Management:

• Stop the drug; exclude infection.
• Prednisolone for significant disease.
• Record as an adverse reaction (Yellow Card).
• Avoid rechallenge (immune checkpoint pneumonitis - specialist decision).

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Occupational Lung Disease

• Asbestos-related disease (shipbuilding, construction, plumbing, electricians - exposure often decades earlier):
  • Pleural plaques - calcified, benign, a marker of exposure only.
  • Diffuse pleural thickening - can restrict the lungs.
  • Benign asbestos pleural effusion.
  • Asbestosis - lower-zone fibrosis, like IPF, needing heavy exposure.
  • Mesothelioma and lung cancer (risk multiplied by smoking).
• Silicosis (quarrying, sandblasting, stonemasonry, engineered stone worktops):
  • Upper-zone nodules.
  • "Eggshell" calcification of hilar nodes.
  • Progressive massive fibrosis.
  • Increased risk of TB, lung cancer, autoimmune disease.
• Coal worker's pneumoconiosis: Upper-zone nodules; progressive massive fibrosis; Caplan's syndrome with RA.
• Berylliosis (aerospace, electronics): A granulomatous disease identical to sarcoidosis - the beryllium lymphocyte proliferation test distinguishes it.
• Management:
  • Remove from exposure; stop smoking.
  • Supportive care; screen for TB in silicosis.
  • Advise on compensation (Industrial Injuries Disablement Benefit, civil claims).
  • Some cases (e.g. mesothelioma) are reportable to the coroner on death.

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Smoking-related ILD

• Respiratory bronchiolitis ILD (RB-ILD) and desquamative interstitial pneumonia (DIP):
  • Smoke-laden macrophages fill the small airways and alveoli.
  • Ground-glass on CT.
  • Improves with stopping smoking (steroids sometimes).
• Pulmonary Langerhans cell histiocytosis:
  • Young adult smokers.
  • Upper-zone nodules and odd-shaped cysts (sparing the bases).
  • Pneumothorax.
  • Sometimes diabetes insipidus or bone lesions.
  • Stopping smoking is essential.
• Combined pulmonary fibrosis and emphysema:
  • Upper-lobe emphysema with lower-lobe fibrosis.
  • Near-normal spirometry but a very low TLCO.
  • High risk of pulmonary hypertension and lung cancer.

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Organising Pneumonia

• What it is: Plugs of granulation tissue fill the small airways and alveoli after injury.
  • Cryptogenic - no cause.
  • Or secondary - infection, connective tissue disease, drugs, radiotherapy (often breast cancer), immune checkpoint inhibitors.
• Clinical pattern: A flu-like illness with cough, breathlessness, fever and weight loss over weeks - often treated as "pneumonia that won't resolve" after several courses of antibiotics.
• Imaging: Patchy, often migratory, peripheral or peribronchial consolidation; "reversed halo" sign.
• Treatment: Usually responds dramatically to prednisolone over months. Relapses are common when steroids are tapered.

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Eosinophilic Lung Disease

• Simple pulmonary eosinophilia (Löffler's syndrome): Transient shadows with blood eosinophilia, often from parasites (e.g. Ascaris) or drugs. Usually self-limiting.
• Acute eosinophilic pneumonia:
  • Rapid severe hypoxaemic illness in a previously well person - often after starting smoking (including vaping) or a new drug.
  • BAL eosinophils are high (blood eosinophils may be normal at first).
  • Responds quickly to steroids.
• Chronic eosinophilic pneumonia:
  • Middle-aged women, often with asthma.
  • Weeks of cough, fever and weight loss.
  • Peripheral consolidation ("photographic negative of pulmonary oedema").
  • High blood eosinophils.
  • Steroid-responsive, but relapses.
• Other causes to consider:
  • ABPA.
  • EGPA (asthma, neuropathy, vasculitis).
  • Drugs (nitrofurantoin, NSAIDs, antibiotics, anticonvulsants - including DRESS).
  • Parasites (Strongyloides - check serology before steroids in at-risk patients).
  • Hypereosinophilic syndrome.

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Cystic Lung Diseases

• Lymphangioleiomyomatosis (LAM):
  • Who: Almost exclusively women of childbearing age; sporadic or with tuberous sclerosis.
  • Lungs: Many thin-walled round cysts throughout both lungs.
  • Features: Breathlessness, recurrent pneumothorax, chylous (milky) pleural effusions, renal angiomyolipomas.
  • Diagnosis: Raised serum VEGF-D supports it.
  • Treatment: Sirolimus (mTOR inhibitor) stabilises lung function. Avoid oestrogens.
• Pulmonary Langerhans cell histiocytosis: Smokers; upper-zone irregular cysts and nodules (see Smoking-related ILD).
• Birt–Hogg–Dubé syndrome:
  • Inherited (FLCN gene).
  • Basal cysts; recurrent pneumothorax.
  • Skin fibrofolliculomas.
  • Renal tumours - screen with MRI.
• Also lymphocytic interstitial pneumonia (Sjögren's) and PCP pneumatoceles.

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Pulmonary Alveolar Proteinosis

• Mechanism: The alveoli fill with surfactant because macrophages cannot clear it. Usually autoimmune - antibodies against GM-CSF. Rarely secondary to dusts or blood cancers.
• Clinical pattern: Gradual breathlessness, often milder than the imaging suggests; infection risk (e.g. Nocardia).
• Imaging: "Crazy paving" on CT - ground-glass with thickened septa.
• Diagnosis: Milky BAL fluid; anti-GM-CSF antibodies.
• Treatment: Whole-lung lavage (specialist); inhaled GM-CSF.

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Sarcoidosis: What It Is

Mechanism: A multisystem disease of unknown cause in which clumps of immune cells - non-caseating granulomas - form in organs. It is thought to be an exaggerated immune response to an environmental trigger in genetically susceptible people. The lungs and lymph nodes are involved in about 90%.

Who: Usually ages 20–40, with a second peak in women over 50. Commoner and often more severe in people of Black African and African-Caribbean descent; also common in Scandinavians.

Course: Many cases - especially Löfgren's syndrome - resolve on their own within 2 years. About a third become chronic, and a minority develop lung fibrosis or serious organ damage (heart, eyes, nervous system).

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Clinical Features of Sarcoidosis

Lungs and nodes:

• Often found on a chest X-ray done for another reason.
• Dry cough, breathlessness, chest discomfort, fatigue.
• Chest X-ray (Scadding) stages:
  • 0 - normal.
  • I - bilateral hilar lymphadenopathy (BHL) only.
  • II - BHL plus lung infiltrates.
  • III - infiltrates only.
  • IV - fibrosis (upper zone).
  Higher stages are less likely to resolve.

Löfgren's syndrome - an acute form with an excellent prognosis (usually resolves within 2 years):

• Erythema nodosum.
• Bilateral hilar lymphadenopathy.
• Ankle arthritis (periarticular).
• Fever.

Outside the lungs:

• Skin: Erythema nodosum; lupus pernio (purple plaques on the nose - chronic disease); scar infiltration.
• Eyes: Uveitis (anterior or posterior), dry eyes - can threaten sight. Everyone needs an eye examination.
• Heart (cardiac sarcoidosis):
  • Heart block, ventricular arrhythmias, sudden death, cardiomyopathy.
  • Can be the first presentation in a young adult.
  • Screen everyone with an ECG; cardiac MRI or FDG-PET if abnormal or symptomatic.
• Calcium: Granulomas make active vitamin D (calcitriol), causing hypercalcaemia and hypercalciuria (kidney stones, nephrocalcinosis).
• Nervous system (neurosarcoidosis):
  • Facial nerve palsy (sometimes bilateral).
  • Meningitis.
  • Pituitary and hypothalamic disease (diabetes insipidus).
  • Spinal cord lesions.
• Other: Liver and spleen enlargement; parotid swelling; kidneys; joints; fatigue (very common and disabling).
• Heerfordt's syndrome: Parotid enlargement, uveitis, fever and facial palsy.

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Diagnosing Sarcoidosis

Sarcoidosis is diagnosed by three things:

• A compatible clinical and radiological picture.
• Non-caseating granulomas on biopsy (usually not needed in classic Löfgren's syndrome).
• Exclusion of other causes of granulomas - TB (caseating granulomas, culture), fungal infection, lymphoma, berylliosis, drug reactions (including immune checkpoint inhibitors), hypersensitivity pneumonitis.

Tests:

• Biopsy: EBUS-guided needle aspiration of mediastinal nodes is the usual route; skin lesions are easy to biopsy too.
• Bloods: Calcium, U&E, LFTs, FBC (lymphopenia).
• Serum ACE - often raised, but neither sensitive nor specific; not diagnostic.
• Urine calcium; immunoglobulins.
• Lung function (restriction, low TLCO) and HRCT (nodules along lymphatics, upper zones; nodes).
• Screening for organ involvement in everyone:
  • ECG.
  • Eye examination.
  • Calcium.
  • Renal and liver function.
• FDG-PET to find biopsy sites, assess activity and look for cardiac disease.

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Treating Sarcoidosis

• Many patients need no treatment - monitor stage I and mild stage II disease with symptoms, lung function and imaging. Löfgren's syndrome needs only NSAIDs for joint pain and erythema nodosum.
• Treat if there is:
  • Significant or worsening lung disease (symptoms with falling lung function).
  • Organ- or life-threatening disease - cardiac, eye (posterior uveitis), neurological, hypercalcaemia, renal.
  • Disfiguring skin disease (lupus pernio).
• First line: Oral prednisolone (e.g. 20–40 mg daily for pulmonary disease), tapered to the lowest effective dose over 6–12 months or more. Give bone protection.
• Steroid-sparing: Methotrexate (most used), azathioprine, mycophenolate, leflunomide.
• Refractory disease: Anti-TNF (infliximab) - especially for cardiac, neuro and skin disease.
• Cardiac sarcoidosis: Steroids plus immunosuppression; pacing for heart block; an ICD for ventricular arrhythmias or severe LV dysfunction.
• Hypercalcaemia: Steroids; avoid vitamin D supplements and excess sun.
• Topical or local steroids for anterior uveitis and skin.
• End-stage fibrosis: Oxygen, pulmonary hypertension assessment, transplant.
• Recognise and support fatigue.

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Drug Reference Table

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Spot the Diagnosis

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Exam-style Scenarios

1. A 74-year-old ex-smoker has 18 months of breathlessness and dry cough. He has clubbing and bibasal crackles. HRCT shows subpleural basal honeycombing. FVC is 68%. No exposures or autoimmune features.
Answer: Idiopathic pulmonary fibrosis (UIP pattern).

• ILD MDT confirmation.
• Offer an antifibrotic (pirfenidone or nintedanib).
• Pulmonary rehabilitation; oxygen assessment; advance care planning.
• No steroids.

2. A 50-year-old woman has 6 months of breathlessness. She has a feather duvet and two budgies. HRCT shows ground-glass, centrilobular nodules and air trapping. Bird precipitins are positive.
Answer: Non-fibrotic hypersensitivity pneumonitis.

• Remove the birds and feather bedding completely.
• Consider prednisolone if there is significant impairment.
• Monitor lung function.

3. A 28-year-old woman has painful red lumps on her shins, ankle swelling and fever. Chest X-ray shows bilateral hilar lymphadenopathy.
Answer: Löfgren's syndrome.

• NSAIDs for symptoms; no biopsy needed if typical.
• Screen with ECG, calcium and an eye examination.
• Excellent prognosis - usually resolves.

4. A 35-year-old man with known pulmonary sarcoidosis has palpitations and a syncopal episode. ECG shows second-degree heart block.
Answer: Suspected cardiac sarcoidosis.

• Urgent cardiology review.
• Cardiac MRI and/or FDG-PET.
• Pacing or ICD as indicated; immunosuppression.

5. A woman with RA on methotrexate has 2 weeks of fever, dry cough and breathlessness. CT shows new bilateral ground-glass.
Answer: Possible methotrexate pneumonitis - but exclude infection (including PCP) first.

• Stop methotrexate.
• Investigate for infection (BAL if needed).
• Steroids once infection is excluded.

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Key Points

• ILD causes restriction, low TLCO and exertional desaturation; fine crackles and sometimes clubbing.
• Always search for a cause - drugs, birds, moulds, occupation, connective tissue disease.
• HRCT and the ILD MDT are central to diagnosis.
• IPF: UIP pattern with honeycombing; antifibrotics (pirfenidone or nintedanib) slow decline; no steroids.
• Acute exacerbation of IPF has a very high mortality - discuss goals of care early.
• Progressive non-IPF fibrosis: add nintedanib.
• Hypersensitivity pneumonitis: antigen avoidance first; steroids and mycophenolate if needed.
• Drug-induced ILD: methotrexate, amiodarone, nitrofurantoin, bleomycin, checkpoint inhibitors - stop the drug.
• Silicosis raises the risk of TB; asbestos causes plaques, asbestosis, mesothelioma and lung cancer.
• Sarcoidosis: non-caseating granulomas; often resolves; screen everyone for heart, eye and calcium involvement.
• Treat sarcoidosis only for progressive or organ-threatening disease - steroids first, then methotrexate or infliximab.

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Guidelines and Further Reading

• NICE CG163: Idiopathic pulmonary fibrosis in adults
• NICE TA504 (pirfenidone), TA379 and TA864 (nintedanib for IPF), TA747 (nintedanib for progressive fibrosing ILD)
• ATS/ERS/JRS/ALAT: Idiopathic pulmonary fibrosis and progressive pulmonary fibrosis guideline (2022)
• ATS/JRS/ALAT: Hypersensitivity pneumonitis guideline (2020)
• BTS Clinical statement on pulmonary sarcoidosis (2021); ERS sarcoidosis treatment guideline (2021)
• Pneumotox (www.pneumotox.com) - drug-induced lung disease
• BNF - for current doses

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