History

Fact Explanation
Exertional dyspnoea Breathlessness on exertion begins insidiously and progressively worsens so that the patient ultimately develops dyspnoea at rest [2][3][4][7]
Nonproductive cough Is a common symptom in patients with fibrosing alveolitis [2][3][4][7]
Low-grade fevers Is a nonspecific symptom of fibrosing alveolitis [2]
Fatigue Can be a symptom of fibrosing alveolitis or symptom of right heart failure secondary to pulmonary hypertension [3][5]
Weight loss Constitutional symptoms are not very common but are seen in some patients [3]
Arthralgia Has been reported in patients with fibrosing alveolitis, more commonly in women [2][3][6]
Malaise Initial flu-like illness with malaise is seen in some patients with fibrosing alveolitis [2][3][7]
History of taking drugs such as amiodarone, bleomycin, and nitrofurantoin These drugs can cause lung fibrosis. Therefore it is important to exclude these in the history [4]
History of smoking Cigarette smoking has shown to have an association with lung fibrosis [2][4][6]
History of xposure to asbestos, silica, heavy metals, contaminated ventilation systems, moldy foliage, and/or pigeon droppings These agents can also cause pulmonary fibrosis.Therefore it is important to exclude these in the history [4]
History of arthritis, photosensitivity, Raynaud phenomenon, dry eyes, and/or dry mouth These symptoms indicate the presence of a collagen-vascular disease.Therefore it is important to exclude these in the history [4]
References
  1. JOHNSTON ID, BLEASDALE C, HIND CR, WOODCOCK AA. Accuracy of diagnostic coding of hospital admissions for cryptogenic fibrosing alveolitis. Thorax [online] 1991 Aug, 46(8):589-591 [viewed 20 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC463285
  2. JOHNSTON ID, PRESCOTT RJ, CHALMERS JC, RUDD RM. British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society Thorax [online] 1997 Jan, 52(1):38-44 [viewed 21 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC175841
  3. LONGMORE M. WILKINSON I. TURMEZEI T. CHEUNG C.K. Idiopathic pulmonary fibrosis. In: Oxford handbook of Clinical Medicine. Seventh edition. Oxford University press. 2007; 182-183
  4. TALBERT JL, SCHWARTZ DA. Pulmonary Fibrosis, Familial. 2005 Jan 21 [Updated 2010 Oct 19]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2014. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1230/
  5. DAVIDSON S.ed.BOON NA. COLLEDGE NR. WALKER BR. HUNTER JAA. Davidson's principles and practice of Medicine. 20th edition. 2008
  6. BOURKE S, CLAGUE H. Review of cryptogenic fibrosing alveolitis, including current treatment guidelines Postgrad Med J [online] 2000 Oct, 76(900):618-624 [viewed 22 May 2014] Available from: doi:10.1136/pmj.76.900.618
  7. BEHR J. The Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis Dtsch Arztebl Int [online] 2013 Dec, 110(51-52):875-881 [viewed 22 May 2014] Available from: doi:10.3238/arztebl.2013.0875

Examination

Fact Explanation
Bibasal fine end-inspiratory crackles Is the clinical diagnostic sign of fibrosing alveolitis. These can become pan inspiratory in advanced disease[1][2][3][4][5]
Digital clubbing Is seen in 30-50% of patients[1][2][3][4]
Increased respiratory rate Patients become tachypneic with progression of disease.[3]
Dyspnea at rest Is a sign of advanced disease[2][5]
Pedal oedema Can develop due to cor-pulmonale (right heart failure secondary to pulmonary hypertension)[2][4]
Left parasternal heave (right ventricular heave) Due to dilated right ventricle that can develop due to cor-pulmonale (right heart failure secondary to pulmonary hypertension)[2][4]
Elevation of the jugular venous pressure Right ventricular dilatation, leads to tricuspid regurgitation which causes an elevated jugular venous pressure. This develops due to cor-pulmonale (right heart failure secondary to pulmonary hypertension)[2][4]
Cyanosis Patients with advanced disease may develop central cyanosis[1][3][4]
Loud P2 Is a sign of pulmonary hypertension, which can arise secondary to advanced fibrosing alveolitis[3][4]
Fixed split S2 Is a sign of pulmonary hypertension which, can arise secondary to advanced fibrosing alveolitis[3][4]
Holosystolic tricuspid regurgitation murmur Can develop due to cor-pulmonale secondary to a dilated right ventricle (right heart failure secondary to pulmonary hypertension)[2][4]
References
  1. LONGMORE M. WILKINSON I. TURMEZEI T. CHEUNG C.K. Idiopathic pulmonary fibrosis. In: Oxford handbook of Clinical Medicine. Seventh edition. Oxford University press. 2007; 182-183
  2. JOHNSTON ID, PRESCOTT RJ, CHALMERS JC, RUDD RM. British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society Thorax [online] 1997 Jan, 52(1):38-44 [viewed 21 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1758413
  3. BOURKE S, CLAGUE H. Review of cryptogenic fibrosing alveolitis, including current treatment guidelines Postgrad Med J [online] 2000 Oct, 76(900):618-624 [viewed 22 May 2014] Available from: doi:10.1136/pmj.76.900.618
  4. DAVIDSON S.ed.BOON NA. COLLEDGE NR. WALKER BR. HUNTER JAA. Davidson's principles and practice of Medicine. 20th edition. 2008
  5. BEHR J. The Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis Dtsch Arztebl Int [online] 2013 Dec, 110(51-52):875-881 [viewed 22 May 2014] Available from: doi:10.3238/arztebl.2013.0875

Differential Diagnoses

Fact Explanation
Collagen-Vascular Disease Associated With Interstitial Lung Disease Causes lung fibrosis and can present with similar clinical features [1][2][3]
Asbestosis This is caused by inhalation of asbestos which is fibrogenic. Clinical presentation is similar to fibrosing alveolitis with a history of occupational asbestos exposure[2][3]
Extrinsic allergic alveolitis Can present with progressive dyspnoea, weight loss, type 1 respiratory failure, cor pulmonale which can also be seen in patients with fibrosing alveolitis[2][3]
Pneumonia Lung infections can also present with dyspnoea, tachypnoea, fever, malaise etc but the presentation is more acute presentation than fibrosing alveolitis. [1][2][3]
Silicosis Is caused by occupational exposure and inhalation of silica particles which cause lung fibrosis. Has a similar clinical picture to fibrosing alveolitis[1][2][3]
Sarcoidosis This is granulomatous disorder which involves multiple body systems. If lung fibrosis occurs as a result of this disease patients will develop symptoms similar to fibrosing alveolitis [1][2][3]
Pulmonary Eosinophilia Is characterized by allergic infiltration of the lungs with eosinophils, Allergens include several parasites. Presents with dry cough[2][3]
Histoplasmosis Histoplasma pneumonitis is a deep fungal infection that can cause fibrosis in its chronic form, particularly in the upper lung zones[2][3]
Coal Worker's Pneumoconiosis Occurs due to inhalation of coal dust particles. In the early disease the patient will be asymptomatic but with progression of disease may develop dyspnoea, fibrosis and eventual cor pulmonale [2][3]
Bronchiectasis Occurs as a result of chronic infection of lower air ways causing permanent dilatation of the affected airways. Presents with dyapnoea, finger clubbing, coarse inspiratory crackles, chronic cough with purulent sputum production etc.[1][2][3]
References
  1. JOHNSTON ID, PRESCOTT RJ, CHALMERS JC, RUDD RM. British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society Thorax [online] 1997 Jan, 52(1):38-44 [viewed 21 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1758413
  2. LONGMORE M. WILKINSON I. TURMEZEI T. CHEUNG C.K. Oxford handbook of Clinical Medicine. Seventh edition. Oxford University press. 2007
  3. DAVIDSON S.ed.BOON NA. COLLEDGE NR. WALKER BR. HUNTER JAA. Davidson's principles and practice of Medicine. 20th edition. 2008

Investigations - for Diagnosis

Fact Explanation
Chest X ray (posteroanterior and/or lateral views) Lung volume will be reduced. Bilateral lower zone reticulo-nodular shadows will be seen.Presence of honeycomb lung suggests advanced disease[1][2][3][4]
High-resolution computed tomography (HRCT) This is the investigation of choice for diagnosis of lung fibrosis. Has better sensitivity and specificity than plain chest X-ray. HRCT may also give an indication to the etiology of lung fibrosis [1][2][3][5][6][7]
Rheumatoid factor (RF) About 10% of patients with fibrosing alveolitis will be RF positive[2]
Antinuclear antibodies (ANA) About 30% of patients with fibrosing alveolitis will be ANA positive[2]
Arterial blood gas analysis PaO2 will be reduced and PaCO2 will be increased[2][5]
Lung function tests Will show features of restrictive lung disease. i.e. total lung capacity, forced expiratory volume per one second (FEV1) and the forced vital capacity (FVC) will be reduced. The FEV1/FVC ratio will be normal or increased[1][2][4][5][7]
Diffusion capacity for carbon monoxide[DLCO] Will be decreased in patients with fibrosing alveolitis [2][4][5]
Bronchoalveolar lavage (BAL) Will show features of alveolitis. If lymphocytic predominant the prognosis will be good but if neutrophils and eosinophils predominant, the prognosis is poor[2][5]
Surgical lung biopsy Histological studies will show fibrosis and feature of a chronic inflammatory alveolitis. The usage of this test is limited by its complications[1][2][6][7]
C- reactive protein Will be increased but not specific or diagnostic of fibrosing alveolitis [2]
References
  1. RUDD R. M, PRESCOTT R. J, CHALMERS J C, JOHNSTON I. D A. British Thoracic Society Study on cryptogenic fibrosing alveolitis: response to treatment and survival. Thorax [online] 2007 January, 62(1):62-66 [viewed 20 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2111279/
  2. LONGMORE M. WILKINSON I. TURMEZEI T. CHEUNG C.K. Idiopathic pulmonary fibrosis. In: Oxford handbook of Clinical Medicine. Seventh edition. Oxford University press. 2007; 182-183
  3. CORNE J. POINTON K. Chest X-ray made easy. Third edition. Churchill Livingstone Elsevier.2010; 95-98
  4. JOHNSTON ID, PRESCOTT RJ, CHALMERS JC, RUDD RM. British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society Thorax [online] 1997 Jan, 52(1):38-44 [viewed 21 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1758413
  5. TALBERT JL, SCHWARTZ DA. Pulmonary Fibrosis, Familial. 2005 Jan 21 [Updated 2010 Oct 19]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2014. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1230/
  6. BOURKE S, CLAGUE H. Review of cryptogenic fibrosing alveolitis, including current treatment guidelines Postgrad Med J [online] 2000 Oct, 76(900):618-624 [viewed 22 May 2014] Available from: doi:10.1136/pmj.76.900.618
  7. BEHR J. The Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis Dtsch Arztebl Int [online] 2013 Dec, 110(51-52):875-881 [viewed 22 May 2014] Available from: doi:10.3238/arztebl.2013.0875

Investigations - Followup

Fact Explanation
Chest X ray Fibrosing alveolitis is a progressive condition which requires follow up and monitoring of the disease course. Comparison of chest X rays will show changes that will indicate disease progression[1][2]
Lung function tests Serial lung function tests will help to determine the disease progression and response to treatment [1][2]
References
  1. LONGMORE M. WILKINSON I. TURMEZEI T. CHEUNG C.K. Idiopathic pulmonary fibrosis. In: Oxford handbook of Clinical Medicine. Seventh edition. Oxford University press. 2007; 182-183
  2. RUDD R. M, PRESCOTT R. J, CHALMERS J C, JOHNSTON I. D A. British Thoracic Society Study on cryptogenic fibrosing alveolitis: response to treatment and survival. Thorax [online] 2007 January, 62(1):62-66 [viewed 20 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2111279/

Management - General Measures

Fact Explanation
Quit smoking Cigarette smoking seems to be the most strongly associated risk factor in developing idiopathic pulmonary fibrosis. It also seems to contribute to accelerated loss of lung function. Therefore smoking cessation will be beneficial for these patients[6]
Oxygen therapy Supplemental oxygen therapy given to patients with dyspnoea improves the symptom and the quality of life[1][2][3]
Vaccination against influenza and pneumococcal infection Vaccination against pneumococci prevents pneumococcal pneumonia which is the most commonly seen community acquired pneumonia, in patients with chronic lung disease. Vaccination against influenza helps to prevents acute exacerbations of disease in patients with chronic lung disease.[5]
Palliative care Should be considered in patients with incurable advanced disease[2]
Treatment for gastro-esophageal reflux (GER) GER is seen in up to 80% of patients. It could be a trigger for disease progression and acute exacerbations. Therefore GER should be routinely treated in these patients[2]
Pulmonary rehabilitation Is a process done in order to benefit the patients with chronic lung diseases by decreasing symptoms, optimizing their functional state and improving the quality of life. This includes an individualized program which combines scheduled exercises, chest physiotherapy, nutritional interventions, ancillary treatment etc needed to help the patient recover gradually and to remain in a fully functional state as far as possible.[4]
References
  1. WEllS AU. HIRAN N.Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax[online] 2008;63(Suppl V):v1–v58.[viewed on 21 May 2014] Available from; http://thorax.bmj.com/content/63/Suppl_5/v1.full.pdf+html
  2. BEHR J. The Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis Dtsch Arztebl Int [online] 2013 Dec, 110(51-52):875-881 [viewed 22 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3928534/
  3. REES PJ, DUDLEY F. Oxygen therapy in chronic lung disease BMJ [online] 1998 Sep 26, 317(7162):871-874 [viewed 23 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1113951
  4. SHARMA BB, SINGH V. Pulmonary rehabilitation: An overview Lung India [online] 2011, 28(4):276-284 [viewed 23 May 2014] Available from: doi:10.4103/0970-2113.85690
  5. FURUMOTO AKITSUGU. OHKUSA YASUSHI. CHEN MENG. KAWAKAMI KENJI, MASAKI HIRONORI. SUEYASU YOSHIKO. IWANAGA TOMOAKI. AIZAWA HISAMICHI. NAGATAKE TSUYOSHI. OISHI KAZUNORI. Additive effect of pneumococcal vaccine and influenza vaccine on acute exacerbation in patients with chronic lung disease. NAOSITE: Nagasaki University's Academic Output SITE[online] 2008; [viewed on 23 May 2014] Available at; http://naosite.lb.nagasaki-u.ac.jp/dspace/bitstream/10069/18720/4/Vaccine26_4284.pdf
  6. OH CK, MURRAY LA, MOLFINO NA. Smoking and Idiopathic Pulmonary Fibrosis Pulm Med [online] 2012:808260 [viewed 23 May 2014] Available from: doi:10.1155/2012/808260

Management - Specific Treatments

Fact Explanation
Prednisolone Corticosteroids are considered first line treatment given together with an immuno-modulator but majority are unresponsive to treatment [1][2][5]
Immunomodulator agents (azathioprine or cyclophosphamide) Is given together with prednisolone as first line treatment but most patients do not respond well to treatment [1][2]
N -acetylcysteine (NAC) Adding NAC to treatment has shown to have a significantly better treatment effect than corticosteroid and immunomodulator alone[1][4][6]
Lung transplant Is an option in patients who are unresponsive to medical treatment and have significant functional impairment[2][3][5]
References
  1. RUDD R. M, PRESCOTT R. J, CHALMERS J C, JOHNSTON I. D A. British Thoracic Society Study on cryptogenic fibrosing alveolitis: response to treatment and survival. Thorax [online] 2007 January, 62(1):62-66 [viewed 20 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2111279/
  2. LONGMORE M. WILKINSON I. TURMEZEI T. CHEUNG C.K. Idiopathic pulmonary fibrosis. In: Oxford handbook of Clinical Medicine. Seventh edition. Oxford University press. 2007; 182-183
  3. TALBERT JL, SCHWARTZ DA. Pulmonary Fibrosis, Familial. 2005 Jan 21 [Updated 2010 Oct 19]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2014. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1230/
  4. WEllS AU. HIRAN N.Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax[online] 2008;63(Suppl V):v1–v58.[viewed on 21 May 2014] Available from; http://thorax.bmj.com/content/63/Suppl_5/v1.full.pdf+html
  5. BOURKE S, CLAGUE H. Review of cryptogenic fibrosing alveolitis, including current treatment guidelines Postgrad Med J [online] 2000 Oct, 76(900):618-624 [viewed 22 May 2014] Available from: doi:10.1136/pmj.76.900.618
  6. WOODCOCK HV, MAHER TM. The treatment of idiopathic pulmonary fibrosis F1000Prime Rep [online] :16 [viewed 22 May 2014] Available from: doi:10.12703/P6-16