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Idiophatic Pulmonary Fibrosis (IPF)
Key Messages
- Idiopathic Pulmonary Fibrosis is a chronic, progressive fibrosing interstitial pneumonia with an unknown cause, usually affecting older adults.
- Diagnosis relies heavily on HRCT showing a UIP pattern, supported by PFTs and multidisciplinary discussion.
- The disease commonly presents with exertional breathlessness, dry cough, and bibasilar “velcro-like” crackles.
- Antifibrotic drugs like pirfenidone and nintedanib slow disease progression, while lung transplantation is the only curative option.
- IPF is often underdiagnosed in Africa due to limited HRCT access, leading to misdiagnosis and delayed treatment.
IPF is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults. Pathologically characterized by usual interstitial pneumonia (UIP) pattern. Genetics (telomerase mutations, MUC5B promoter variant) and environmental factors (smoking, agriculture) contribute.
Global & African Context: Incidence increases with age (>50/100,000 in >75 age group). Underrecognized in Africa due to diagnostic challenges. Nigerian data scarce; likely misdiagnosed as chronic TB or COPD. (Khalil et al., 2018; European Respiratory Review)
Symptoms & Clinical Features
- Progressive exertional dyspnea (insidious onset)
- Dry, hacking cough
- Physical signs: Bibasilar inspiratory velcro-like crackles (90%), digital clubbing (25-50%)
- Disease course: Variable; acute exacerbations carry high mortality.
Differential Diagnosis
- Other interstitial lung diseases: Hypersensitivity pneumonitis, connective tissue disease-ILD, asbestosis
- Chronic heart failure
- Chronic hypersensitivity pneumonitis
- Sarcoidosis (Stage IV)
- Drug-induced lung disease
Investigations
- HRCT chest: Essential; shows UIP pattern (subpleural, basal reticulation, honeycombing ± traction bronchiectasis).
- PFTs: Restrictive defect (↓TLC, ↓VC), impaired gas exchange (↓DLCO).
- 6MWT: Desaturation >4% predicts mortality.
- Multidisciplinary discussion (MDD): Gold standard for diagnosis; radiologist, pulmonologist, pathologist.
- Bronchoscopy/BAL: Exclude alternative diagnoses (infection, malignancy)
Resource-limited setting: HRCT availability is major constraint; clinical-radiological diagnosis often necessary (Micheal et al., 2021; African Journal of Thoracic and Critical Care Medicine).
Treatment
- Antifibrotics: Pirfenidone or nintedanib slow FVC decline (reduce by ~50% annually).
- Supportive: Oxygen for hypoxemia, pulmonary rehabilitation, palliative care.
- Lung transplantation: Only curative option; limited in Africa.
- Avoid: Long-term steroids, immunosuppressants (harmful in IPF per PANTHER trial).
- African access: Antifibrotics largely unavailable/unaffordable; focus on supportive care.
- Taking walks: Physical activities help to improve symptoms in patients.
Follow-up
- 3-4 monthly for monitoring progression
- Serial PFTs (FVC, DLCO) every 3-6 months
- Monitor for acute exacerbations, complications (pulmonary hypertension)
Prevention & Control
- Smoking cessation
- Prompt treatment of GERD (possible association)
- Influenza/pneumococcal vaccination to prevent infections
- Genetic counselling in familial cases
Conclusion
IPF is likely underdiagnosed in Nigeria due to limited HRCT access and specialist expertise. Developing national ILD registries and multidisciplinary teams in tertiary centres is essential. Advocacy for affordable antifibrotic access is urgently needed.
The fact that presentation of IPF is mainly as worsening chronic dry cough with reducing exercise tolerance, chronic hypersensitivity pneumonitis (Chronic HP) and COPD (Chronic bronchitis predominant) are the greatest mimics and treatments tends to be tailored in such direction. Prolonged steroid use especially in the aim to managed HP; usually worsen IPF alongside enormous steroid side-effects.
Functioning MDT, role of chest imaging particularly HRCT and spirometry cannot be over-emphasized in the management of IPF, while adequate management and prophylaxis for GERD go a long way to prevent exacerbation of IPF.
A 68-year-old man presents with a progressive 8-month history of exertional breathlessness and a persistent dry cough. On examination, he has bibasilar inspiratory “velcro-like” crackles, and HRCT reveals a typical UIP pattern with subpleural, basal honeycombing. Pulmonary function tests show a restrictive defect with reduced TLC and DLCO. He is diagnosed with Idiopathic Pulmonary Fibrosis after multidisciplinary evaluation, and treatment with antifibrotic therapy is initiated to slow further decline.
- Khalil N, et al. (2018). Challenges in diagnosing IPF in resource-limited settings. African Journal of Thoracic and Critical Care Medicine.
- American Thoracic Society/European Respiratory Society. (2022). Idiopathic Pulmonary Fibrosis Guidelines.
- Wong M. Interstitial lung disease in Africa – a need for recognition and earlier diagnosis. Afr J Thorac Crit Care Med. 2022;28(2). doi:10.7196/ajtccm.2022.v28i2.258
- Olaosebikan H, Adeyeye O, Akintayo R, Akpabio A, Adenitan A, Adelowo O, et al. Connective tissue disease–associated interstitial lung disease: an underreported cause of interstitial lung disease in Sub-Saharan Africa. Clin Rheumatol. 2021;40:3455–60. doi:10.1007/s10067-020-05336-5.
- Anyabolu AE, Enemuo EH, Ele PU, Ugoeze FC, Ufoaro CU, Nwagbara CT, et al. Idiopathic Pulmonary Fibrosis with complications at Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria: A case report [Internet]. Afrimedic J. Vol. 4, No. 2. [cited 2026 Mar 6]. Available from: https://www.ajol.info/index.php/afrij/article/download/106049/96032
- Bloem AEM, Dolk HM, Wind AE, Vis JJ, Custers JWH, Spruit MA, et al. Prognostic value of 6-min walk test derived attributes in patients with idiopathic pulmonary fibrosis. Respir Med. 2025;236:107862.
More topics to explore
Author's details
Reviewer's details
Idiophatic Pulmonary Fibrosis (IPF)
IPF is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults. Pathologically characterized by usual interstitial pneumonia (UIP) pattern. Genetics (telomerase mutations, MUC5B promoter variant) and environmental factors (smoking, agriculture) contribute.
Global & African Context: Incidence increases with age (>50/100,000 in >75 age group). Underrecognized in Africa due to diagnostic challenges. Nigerian data scarce; likely misdiagnosed as chronic TB or COPD. (Khalil et al., 2018; European Respiratory Review)
- Khalil N, et al. (2018). Challenges in diagnosing IPF in resource-limited settings. African Journal of Thoracic and Critical Care Medicine.
- American Thoracic Society/European Respiratory Society. (2022). Idiopathic Pulmonary Fibrosis Guidelines.
- Wong M. Interstitial lung disease in Africa – a need for recognition and earlier diagnosis. Afr J Thorac Crit Care Med. 2022;28(2). doi:10.7196/ajtccm.2022.v28i2.258
- Olaosebikan H, Adeyeye O, Akintayo R, Akpabio A, Adenitan A, Adelowo O, et al. Connective tissue disease–associated interstitial lung disease: an underreported cause of interstitial lung disease in Sub-Saharan Africa. Clin Rheumatol. 2021;40:3455–60. doi:10.1007/s10067-020-05336-5.
- Anyabolu AE, Enemuo EH, Ele PU, Ugoeze FC, Ufoaro CU, Nwagbara CT, et al. Idiopathic Pulmonary Fibrosis with complications at Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria: A case report [Internet]. Afrimedic J. Vol. 4, No. 2. [cited 2026 Mar 6]. Available from: https://www.ajol.info/index.php/afrij/article/download/106049/96032
- Bloem AEM, Dolk HM, Wind AE, Vis JJ, Custers JWH, Spruit MA, et al. Prognostic value of 6-min walk test derived attributes in patients with idiopathic pulmonary fibrosis. Respir Med. 2025;236:107862.
Content
Author's details
Reviewer's details
Idiophatic Pulmonary Fibrosis (IPF)
Background
IPF is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults. Pathologically characterized by usual interstitial pneumonia (UIP) pattern. Genetics (telomerase mutations, MUC5B promoter variant) and environmental factors (smoking, agriculture) contribute.
Global & African Context: Incidence increases with age (>50/100,000 in >75 age group). Underrecognized in Africa due to diagnostic challenges. Nigerian data scarce; likely misdiagnosed as chronic TB or COPD. (Khalil et al., 2018; European Respiratory Review)
Further readings
- Khalil N, et al. (2018). Challenges in diagnosing IPF in resource-limited settings. African Journal of Thoracic and Critical Care Medicine.
- American Thoracic Society/European Respiratory Society. (2022). Idiopathic Pulmonary Fibrosis Guidelines.
- Wong M. Interstitial lung disease in Africa – a need for recognition and earlier diagnosis. Afr J Thorac Crit Care Med. 2022;28(2). doi:10.7196/ajtccm.2022.v28i2.258
- Olaosebikan H, Adeyeye O, Akintayo R, Akpabio A, Adenitan A, Adelowo O, et al. Connective tissue disease–associated interstitial lung disease: an underreported cause of interstitial lung disease in Sub-Saharan Africa. Clin Rheumatol. 2021;40:3455–60. doi:10.1007/s10067-020-05336-5.
- Anyabolu AE, Enemuo EH, Ele PU, Ugoeze FC, Ufoaro CU, Nwagbara CT, et al. Idiopathic Pulmonary Fibrosis with complications at Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria: A case report [Internet]. Afrimedic J. Vol. 4, No. 2. [cited 2026 Mar 6]. Available from: https://www.ajol.info/index.php/afrij/article/download/106049/96032
- Bloem AEM, Dolk HM, Wind AE, Vis JJ, Custers JWH, Spruit MA, et al. Prognostic value of 6-min walk test derived attributes in patients with idiopathic pulmonary fibrosis. Respir Med. 2025;236:107862.
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