# Idiopathic Pulmonary Fibrosis (Referral) - Category: chronic - Review status: reviewed (every claim checked against a document named on this page) - Sources: ATS/ERS/JRS/ALAT Clinical Practice Guideline: IPF 2018 · Egyptian National Drug Formulary - Respiratory System 2026 (acetylcysteine monograph) · StatPearls: Idiopathic Pulmonary Fibrosis (NCBI Bookshelf NBK448162) · Idiopathic Pulmonary Fibrosis - disease-level clinical article (idiopathic-pulmonary-fibrosis-referral-full.txt) · Esbriet 267 mg Film-coated Tablets (pirfenidone) SmPC sections 4.2 and 4.4 (eMC product 2731, emc-esbriet-267mg-pirfenidone.txt) · Ofev 150 mg soft capsules (nintedanib) SmPC sections 4.2, 4.3 and 4.4 (eMC product 7705, emc-ofev-150mg-nintedanib.txt) - Verified date: 2026-08 ## Verified against - ATS/ERS/JRS/ALAT Clinical Practice Guideline: IPF 2018 - Idiopathic Pulmonary Fibrosis (Referral) - disease-level clinical article (idiopathic-pulmonary-fibrosis-referral-clinical.txt) - Idiopathic Pulmonary Fibrosis - disease-level clinical article (idiopathic-pulmonary-fibrosis-referral-full.txt) - Esbriet 267 mg Film-coated Tablets (pirfenidone) SmPC sections 4.2 and 4.4 (eMC product 2731, emc-esbriet-267mg-pirfenidone.txt) - Ofev 150 mg soft capsules (nintedanib) SmPC sections 4.2, 4.3 and 4.4 (eMC product 7705, emc-ofev-150mg-nintedanib.txt) ## Treatment metadata - Urgent pulmonology referral (Recognise and refer) - Pirfenidone — 267 mg — oral.solid - Nintedanib — 150 mg — oral.solid ## Complete treatment card ```text IDIOPATHIC PULMONARY FIBROSIS (REFERRAL) Sources: ATS/ERS/JRS/ALAT Clinical Practice Guideline: IPF 2018 · Egyptian National Drug Formulary - Respiratory System 2026 (acetylcysteine monograph) · StatPearls: Idiopathic Pulmonary Fibrosis (NCBI Bookshelf NBK448162) · Idiopathic Pulmonary Fibrosis - disease-level clinical article (idiopathic-pulmonary-fibrosis-referral-full.txt) · Esbriet 267 mg Film- coated Tablets (pirfenidone) SmPC sections 4.2 and 4.4 (eMC product 2731, emc- esbriet-267mg-pirfenidone.txt) · Ofev 150 mg soft capsules (nintedanib) SmPC sections 4.2, 4.3 and 4.4 (eMC product 7705, emc-ofev-150mg-nintedanib.txt) Review status: REVIEWED against ATS/ERS/JRS/ALAT Clinical Practice Guideline: IPF 2018, Idiopathic Pulmonary Fibrosis (Referral) - disease-level clinical article (idiopathic-pulmonary-fibrosis-referral-clinical.txt), Idiopathic Pulmonary Fibrosis - disease-level clinical article (idiopathic- pulmonary-fibrosis-referral-full.txt), Esbriet 267 mg Film-coated Tablets (pirfenidone) SmPC sections 4.2 and 4.4 (eMC product 2731, emc-esbriet-267mg-pirfenidone.txt), Ofev 150 mg soft capsules (nintedanib) SmPC sections 4.2, 4.3 and 4.4 (eMC product 7705, emc- ofev-150mg-nintedanib.txt) (2026-08) IS IT THIS? - reference only, to read alongside your own examination SYMPTOMS - what the patient reports (6) - A family history of early grey hair or other signs of premature ageing raises suspicion for the familial, younger-onset form - Breathlessness on exertion, cough and tiredness are the most common presenting complaints [breathlessness · cough · fatigue] - Diagnosis is frequently delayed more than a year past the first symptoms because the picture is so nonspecific - A history of exposure to inhaled dust, metals, asbestos, mould or birds should be sought to help exclude other interstitial lung disease - Joint pain, dry eyes and mouth, or Raynaud phenomenon suggest an underlying rheumatic illness instead [joint pain] - The cough is characteristically dry, without sputum [cough] SIGNS - what you find (4) - Fine, velcro-like crackles heard at both lung bases on inspiration are the classic chest finding [crackles] - Finger clubbing may also be present [finger clubbing] - Advanced disease can show intolerance of minimal exertion or even low oxygen levels at rest - Skin rash, swollen joints, tight fingers or muscle weakness on exam point away from IPF toward an autoimmune cause [muscle weakness · rash] TESTS (7) - Lung function testing shows a restrictive pattern with reduced FVC, total lung capacity and diffusion capacity - Antinuclear antibody and rheumatoid factor testing are the standard screen to help rule out an autoimmune cause - A chest x-ray isn't detailed enough to confirm the diagnosis; a high-resolution CT with thin axial slices is required - The characteristic UIP pattern on HRCT is bilateral, lower-zone predominant honeycombing with traction bronchiectasis - Ground-glass shadowing or consolidation on imaging is unusual for UIP and should raise suspicion of a different diagnosis - Surgical lung biopsy is considered when imaging and clinical findings don't confidently confirm the UIP pattern - A markedly reduced FVC under 50 percent predicted or DLCO under 35 percent raises the risk of biopsy IF NOT THIS - what else fits (3) - Occupational lung disease such as pneumoconiosis or asbestosis is a key mimic to exclude - Hypersensitivity pneumonitis (including farmer's lung), fungal infection and lung cancer are also on the list - An underlying connective tissue disease is also part of the differential for a UIP pattern Source StatPearls "Idiopathic Pulmonary Fibrosis" - disease-level clinical article Status traced to the source above Rx: Main treatment | Antifibrotic - started and supervised by an ILD specialist | Antifibrotic - the twice-daily alternative, equally effective, also specialist-initiated MAIN TREATMENT 1. URGENT PULMONOLOGY REFERRAL (RECOGNISE AND REFER) [1st line] Adult Urgent referral to a tertiary pulmonology/interstitial lung disease (ILD) center for high-resolution CT (HRCT) imaging, lung function tests (FVC/DLCO), and evaluation for specialist-initiated disease-modifying antifibrotics (pirfenidone or nintedanib). - Immediate referral Peds IPF affects older adults (>50 years); pediatric interstitial lung disease requires immediate specialized pediatric pulmonology referral. Source ATS/ERS/JRS/ALAT Clinical Practice Guideline: IPF 2018 Why IPF requires specialized HRCT diagnosis and early specialist initiation of antifibrotics; primary care clinicians do not initiate primary disease-modifying drug therapy. Caution URGENT REFERRAL: IPF causes rapid, irreversible lung function loss with high 3-year mortality; early specialist referral for HRCT and antifibrotic initiation is critical to slow disease progression. DO NOT prescribe corticosteroid monotherapy or triple therapy (prednisone + azathioprine + N-acetylcysteine), which is HARMFUL and increases mortality in IPF (PANTHER-IPF trial / ATS guidelines). In Egypt, antifibrotics (nintedanib and pirfenidone) are high-cost specialist drugs dispensed through university ILD centers (e.g., Kasr Al-Ainy, Ain Shams) or Ministry of Health / Health Insurance specialized committees. What actually changes the disease is an antifibrotic - pirfenidone or nintedanib - and neither is started in primary care. Both are on this card, with their doses and their monitoring, so the referring doctor knows what the patient is being sent for and can recognise the regimen afterwards. Starting it early is the point. RED FLAG - acute exacerbation (rapid decline in lung function) requires urgent exclusion of heart failure, infection, and thromboembolic disease, a distinct pathway from routine specialist referral. ANTIFIBROTIC - STARTED AND SUPERVISED BY AN ILD SPECIALIST 2. PIRFENIDONE [1st line] Adult Titrated over 14 days: days 1 to 7, 267 mg three times a day (801 mg/day); days 8 to 14, 534 mg three times a day (1602 mg/day); from day 15, 801 mg three times a day (2403 mg/day). Maintenance is 801 mg three times a day WITH FOOD, total 2403 mg a day; doses above 2403 mg/day are not recommended. After a break of 14 consecutive days or more, restart at the beginning of the titration. - Long-term, while it is tolerated and the disease is being modified Peds Not a paediatric drug. SmPC verbatim: "There is no relevant use of Esbriet in the paediatric population for the indication of IPF." Source Esbriet 267 mg Film-coated Tablets (pirfenidone) SmPC sections 4.2 and 4.4 (eMC product 2731, emc-esbriet-267mg-pirfenidone.txt) Why The card named this drug in prose and gave no dose, so a physician could see what the referral was for but not what the patient had been put on. The article states the indication - "Antifibrotic medications, including pirfenidone and nintedanib (tyrosine kinase inhibitors), are approved for use in IPF." - and the SmPC states the same indication and the dose. The SmPC's titration is used rather than the article's one-line figure, because the article's "267 mg (1 capsule) taken 3 times daily" is 801 mg a day, which the label defines as the FIRST WEEK; its own stated maximum, 2403 mg a day, is the label's maintenance dose. Both numbers are the article's, so nothing is contradicted - the label supplies the fortnight between them. Four Egyptian products are registered and the cost is the real barrier: PERDOFENEX 267 mg is 767 EGP for 30 capsules and 19,543 EGP for the 270-capsule pack (a month at maintenance is 270 capsules). Caution LIVER MONITORING IS PART OF THE PRESCRIPTION. SmPC verbatim: "Liver function tests (ALT, AST and bilirubin) should be performed prior to the initiation of treatment with Esbriet, and subsequently at monthly intervals for the first 6 months and then every 3 months thereafter". And: "If a patient exhibits an aminotransferase elevation to ≥5 x ULN, Esbriet should be permanently discontinued and the patient should not be rechallenged." CONTRAINDICATED, SmPC verbatim: hypersensitivity; "History of angioedema with pirfenidone"; "Concomitant use of fluvoxamine"; "Esbriet therapy should not be used in patients with severe hepatic impairment or end stage liver disease"; and "Esbriet therapy should not be used in patients with severe renal impairment (CrCl <30 ml/min) or end stage renal disease requiring dialysis". The article says the same about the kidney. Photosensitivity is the commonest reason patients stop. Daily sunblock and sun avoidance from the first tablet, which matters in Cairo; a mild-to-moderate reaction is managed by dropping to 267 mg three times a day, a severe one by interrupting and seeking advice. Take it with food - it reduces the nausea and dizziness, and the maintenance dose is specified with food. Started and supervised by an ILD centre. SmPC verbatim: "Treatment with Esbriet should be initiated and supervised by specialist physicians experienced in the diagnosis and treatment of IPF." This row exists so that the regimen is visible and a patient already on it is not left without it. What it does and does not do: on the article's reading of the studies, both drugs slow the disease down and may reduce all-cause mortality and acute exacerbations, which is why it recommends starting treatment early. It slows the disease; it does not reverse it. The article's own dosing sentence, printed so the difference is visible: it puts pirfenidone at 267 mg - 1 capsule - given 3 times a day, up to a ceiling of 2403 mg daily split into 3 doses. That first figure is the label's week-one dose, not the maintenance dose. Egypt PERDOFENEX 267 MG 30 CAPS. MULTICARE 767.00 EGP (25.57/unit) PERDOFENEX 267 MG 270 CAPS. MULTICARE 19543.00 EGP (72.38/unit) ESBRIET 267 MG 270 CAPS. F.HOFFMAN LA ... 44255.00 EGP (163.91/unit) ANTIFIBROTIC - THE TWICE-DAILY ALTERNATIVE, EQUALLY EFFECTIVE, ALSO SPECIALIST-INITIATED 3. NINTEDANIB [1st line] Adult 150 mg twice daily, about 12 hours apart, with food. 100 mg twice daily is only for patients who do not tolerate 150 mg twice daily. Maximum 300 mg a day. A missed dose is skipped, not doubled. - Long-term, while it is tolerated Peds This row is the ADULT IPF dose. The label also covers children and adolescents from 6 to 17 years for progressive fibrosing ILD and for systemic-sclerosis-associated ILD, on a weight-band schedule that is not reproduced here because it is not this condition. Paediatric interstitial lung disease goes to paediatric pulmonology. Source Ofev 150 mg soft capsules (nintedanib) SmPC sections 4.2, 4.3 and 4.4 (eMC product 7705, emc-ofev-150mg-nintedanib.txt) Why The other half of the pair the card already named. The article states the indication and that the two are interchangeable in effect - "Both pirfenidone and nintedanib have been demonstrated to be equally effective in slowing the 24-month decrease in FVC and diffusion capacity." - and the SmPC states the indication and the dose. Twice a day rather than three times, which is the practical difference for a patient who will be on it for years. Two Egyptian products, both OFEV; 39,000 EGP for 60 capsules of 150 mg is one month, so this is a health-insurance or ILD-committee drug in practice, not a private prescription. Caution CONTRAINDICATED IN PREGNANCY. SmPC section 4.3 verbatim: "Pregnancy (see section 4.6)" and "Hypersensitivity to nintedanib, to peanut or soya, or to any of the excipients listed in section 6.1." A woman of child-bearing age needs effective contraception on this drug. LIVER MONITORING IS PART OF THE PRESCRIPTION. SmPC verbatim: "hepatic transaminase and bilirubin levels should be investigated before treatment initiation and during the first month of treatment with Ofev. Patients should then be monitored at regular intervals during the subsequent two months of treatment and periodically thereafter, e.g. at each patient visit or as clinically indicated." And: "If transaminase (AST or ALT) elevations > 3× ULN are measured, dose reduction or interruption of the therapy with Ofev is recommended and the patient should be monitored closely." Liver impairment: article verbatim "Nintedanib should be avoided in patients with moderate-to-severe liver impairment." SmPC verbatim, for the mild case: "Adult patients with mild hepatic impairment (Child Pugh A) should be treated with a reduced dose of Ofev". Diarrhoea is the commonest adverse reaction and the commonest reason for stopping. Treat at the first sign with hydration and an antidiarrhoeal; if it persists despite that, the dose comes down to 100 mg twice daily or the drug is interrupted. Started and supervised by an ILD centre. This row exists so that the regimen is visible and a patient already on it is not left without it. Egypt OFEV 150 MG 60 CAPS. BOEHRINGER IN... 39000.00 EGP (650.00/unit) Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy. ``` --- Dawaa Reference is a reference for prescribers, not a medical device, and does not replace clinical judgement. [Privacy policy](/privacy)