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INTERSTITIAL LUNG DISEASE 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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1  Introduction 
Synonyms: interstitial lung disease, diffuse parenchymal lung disease. 
1.1 
Description 
  Interstitial lung disorders (ILD) encompass a diverse range of diseases affecting 
the gas exchanging regions of the lung, which may progress to diffuse lung 
fibrosis.[1][2] 
  Some of these present acutely, whereas others have a sub-acute or chronic 
course.   
  The overall prevalence in the United Kingdom is 1 in 3,000 – 4,000, and ILDs 
account for 3,000 deaths per year.[3]  
  Aetiology and individual prevalence are described under the separate conditions 
below.  Sarcoidosis is also a cause of interstitial lung fibrosis (see Tuberculosis 
and Sarcoidosis Protocol). 
1.2 
Diagnosis 
  Common features of ILD are a history of progressive dyspnoea and a dry cough, 
associated with a chest radiograph (CXR) that shows widespread pulmonary 
shadows.   
  The presence of finger clubbing is a variable sign in ILD.  It occurs in 70% of 
patients with cryptogenic fibrosing alveolitis and the interstitial alveolitis 
associated with rheumatoid arthritis; however, it is almost never seen in the 
fibrosing alveolitis associated with systemic sclerosis and extrinsic allergic 
alveolitis.[4]  
  On auscultation, dry, fine end-inspiratory, basal ‘velcro’ crackles are commonly 
heard. 
1.3 
Investigations  
1.3.1 
Lung Function Tests 
  Spirometry: a restrictive defect reduced total lung capacity and forced vital 
capacity (FVC), with normal FEV1/FVC ratio of greater than 70% (FEV1  = forced 
expiratory volume in 1 second). 
  Blood gases: hypoxaemia and hypocapnia.  Impaired gas diffusion (reduced 
transfer factor1a). 
  Serial measurements over a period of time are often needed to assess slightly 
low values and recognise excessive longitudinal decline. 
                                                 
1a Transfer factor is normally measured by the carbon monoxide absorption rate per unit volume of ventilated lung - DLCO – 
the gas transfer coefficient. 
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1.3.2 
Radiography  
  CXR: -  lung size; distribution; size and nature of nodular and reticular 
abnormalities; the presence of pleural disease; hilar lymphadenopathy and 
confluent shadows allow the experienced eye to differentiate the types of 
interstitial lung disorders.  This has largely been superseded by computed 
tomography scans for diagnosis, but CXR and physiology are the mainstays of 
follow up. 
  High-resolution computed tomography (HRCT) allows detailed evaluation of the 
lung parenchyma by using 1 - 2mm thick slices with a reconstruction algorithm 
that maximises spatial resolution.  This allows earlier diagnosis and narrows the 
diagnosis based on HRCT pattern. 
1.3.3 
Biopsy 
  Small samples of lung parenchyma can be obtained by transbronchial biopsy 
with a flexible bronchoscope; however, because of the small sample size it 
should not be used to assess the degree of fibrosis.[5] 
  Larger samples can be obtained under GA by either thoracotomy or video-
assisted thoracoscopy.  Surgical lung biopsy is recommended in patients without 
contraindications to surgery.   
  The histology is often diagnostic in the early stages, but in advanced disease 
may show non-specific lung fibrosis with no clue to aetiology. 
1.3.4 
Bronchoalveolar Lavage (BAL) 
 Performed during bronchoscopy; yields fluid for cytology and biochemical 
analysis. 
1.4 
Differential Diagnosis 
  Other causes of diffuse lung infiltrates such as pulmonary oedema, 
bronchiectasis, and alveolar cell carcinoma are usually distinguishable with the 
above investigations, which also help to differentiate the following conditions. 
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Idiopathic Pulmonary Fibrosis 
Synonym: - Cryptogenic Fibrosing Alveolitis (CFA). 
2.1 
Definition 
Historically, Idiopathic Pulmonary Fibrosis (IPF) was a family of idiopathic 
pneumonias, sharing the clinical features of dyspnoea, radiological diffuse 
pulmonary infiltrates and various findings of inflammation, fibrosis or both on biopsy. 
 (Appendix A: Table 1)  
A consensus statement, published in 2000,[5] now limits IPF to patients with a 
specific histological finding on biopsy, that of ‘usual interstitial pneumonia’ (UIP).  
However, as few patients in the UK undergo open lung biopsy,[6] the group will retain 
its heterogeneity, making prognosis more difficult.   
2.1.1 
Aetiology of IPF 
  The cause of IPF is unknown, but it is probably an inflammatory and immune 
response to lung damage in genetically predisposed individuals.[4]  Reports have 
described familial cases of IPF with an autosomal dominant pattern of 
inheritance.[7]  Some cases may be associated with previous exposure to dusts 
(e.g. metal or wood), and about 30% have auto-antibodies such as rheumatoid 
or anti-nuclear factor.  Cigarette smoking may be an independent risk factor, 
75% of cases of IPF are current or former smokers.[8]  Latent viral infections have 
also been implicated, but to date no candidate virus has been shown to cause 
IPF.[8]  
2.1.2 
Prevalence of IPF 
  The precise prevalence of IPF is unknown but is estimated at between 3 - 6 
cases per 100,000 in the general population[5] and it is slightly more common in 
males than females.[8] 
  The incidence of IPF increases with age.  Patients become symptomatic in the 
fifth and sixth decade and approximately 2/3 of patients are over the age of 60 at 
the time of diagnosis.[5] In the age group 35 - 44 years the prevalence is 
2.7/100,000.[5]  
2.2 
Diagnosis 
  Insidious onset of non-productive cough and progressive dyspnoea. 
  Dyspnoea most prominent and disabling symptom. 
 Clubbing 
in 
70%. 
  Dry, end-inspiratory, ‘velcro’ basal crackles in 80%.  With disease progression 
these become pan-inspiratory and extend to the upper zones.   
  Cyanosis, cor pulmonale, an accentuated pulmonary second sound, right 
ventricular heave and peripheral oedema may be observed in the late phases of 
the disease. 
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2.3 
Investigation 
2.3.1 
Pulmonary Function Testing in IPF 
  Reduced total lung capacity (TLC), functional residual capacity (FRC) and 
residual volume (RV). 
  Smokers and patients with superimposed COPD may have normal volumes 
early in the disease. 
  Patients are tachypnoeic, taking rapid shallow breaths. 
 FEV1 and FVC are reduced.  But FEV1/FVC ratio is normal or raised (> 70%). 
  The gas transfer factor DLCO is reduced because of a reduction in capillary 
volumes as well as ventilation and perfusion abnormalities. 
  Formal cardiopulmonary exercise testing is more sensitive in the detection of 
abnormalities of oxygen transfer.  Exercise gas exchange is a sensitive 
parameter to monitor the clinical course, but is not often performed in UK 
practice. 
2.3.2 
CXR in IPF 
  Most have abnormal CXR at time of presentation and basal reticular shadowing 
may be present for years before the development of symptoms.  A normal CXR 
does not exclude the histological finding of usual interstitial pneumonia (UIP) on 
biopsy. 
  Peripheral reticular opacities at lung bases, with reduced lung volumes are 
common findings.  Patients with co-existing emphysema may have preserved or 
increased lung volumes. 
2.3.3 
High Resolution Computed Tomography in IPF 
  Pattern in IPF of patchy, predominantly peripheral, sub-pleural, bibasal reticular 
abnormalities.[5]  A normal HRCT scan cannot exclude infiltrative disease.  One 
third of cases will be missed if HRCT scans alone are used for diagnosis.[5]  
  Patients with predominant reticular opacity or honeycombing usually progress 
despite treatment. 
  The extent of lung fibrosis on HRCT is an important predictor of survival.[5]  
2.3.4 
Bronchoalveolar Lavage in IPF 
  Despite its value as a research tool, the diagnostic use of BAL in IPF is limited.[5]  
2.3.5 
Lung Biopsy 
  UIP is the histological pattern that identifies patients with IPF.[5]   However, a 
review of 200 patients in the UK showed that transbronchial or open lung 
biopsies were performed in only 33% and 7.5% of patients respectively: the 
diagnosis of IPF was made on clinical grounds in most cases.[6]  
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2.4 
Treatment 
  Conventional treatment options include corticosteroids, immunosuppressives 
(e.g. azathioprine), cytotoxic agents (e.g. cyclophosphamide) and antifibrotic 
agents (colchicines or d-penicillamine) either alone or in combination. 
  Given that there is no clinical evidence that treatment improves survival or 
quality of life, (and that treatment is associated with risk of complications) 
therapy may not be indicated for all patients.[5]  
2.4.1 
Corticosteroids 
  Used ubiquitously, but no randomised controlled trial data: 10-30% objective 
improvement; 40% report subjective improvement.[3]  
  High doses used 40-100mg for 2-4 months and then dose reduced. 
  If improvement occurs, it happens in the first 3 months of treatment, and 
responsive patients are maintained on corticosteroid therapy. 
  Relapses or deterioration whilst on steroids warrant escalation of dose or 
addition of an immunosuppressive agent. 
2.4.2 
Cytotoxic Treatment (Azathioprine/Cyclophosphamide) 
  Used for steroid non-responders or those with side effects on steroids.[3][5]  
  Favourable responses in a few small treatment trials. 
2.4.3 
Lung Transplantation 
  Patients <55 years old, without complicating medical illnesses should be referred 
early to regional transplantation centres.[8]   Patients up to 65 years old may be 
considered for transplantation. 
2.5 
Prognosis 
  IPF kills about 1500 people p.a.  in the UK[3] and the mortality is increasing.  The 
highest rates of mortality in the UK occur in the industrialised central areas of 
England and Wales.  The mean length of survival from the time of diagnosis 
varies between 3.2 and 5 years.[5 
  The cause of death is respiratory failure in 40%.  In the majority death is 
triggered by a complicating illness, mainly coronary artery disease and 
infections.  Bronchogenic carcinoma occurs in 10-15% of patients. 
  Spontaneous remissions do not occur.   
  Some patients follow an indolent course over many years, and these are thought 
to represent other disorders misdiagnosed as IPF.[7] 
  Indicators of longer survival include: younger age at onset (< 50), female sex, 
beneficial response or stable disease 3 - 6 months after initial corticosteroid 
treatment.[5]  
  Following lung transplantation a 5-year survival rate of 50 - 60% is quoted.[5]  
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Connective Tissue Diseases 
3.1 
Description 
In about 35% of cases the typical features of IPF occur as part of a connective 
tissue disease, which often has various other lung complications.[9]  
3.2 
Rheumatoid Disease 
  Interstitial lung disease is clinically detected in less than 5% of patients with 
rheumatoid arthritis, although studies have shown a much higher prevalence of 
interstitial changes using HRCT scans.[10][11]  
  The natural history of interstitial lung disease in rheumatoid arthritis has not been 
well studied, and there are no data on prognostic factors.  The radiological 
changes on HRCT scanning were more peripherally distributed in RA compared 
to IPF.[12]  Rheumatoid factor may prove to be protective against progressive 
fibrosis.  Drugs such as methotrexate, gold or penicillamine may cause lung 
fibrosis. 
3.3 
Systemic Sclerosis (Scleroderma) 
  Progressive systemic sclerosis and the CREST variant: (Calcinosis; Raynaud’s 
phenomenon; oesophageal dysfunction/dysmotility; Sclerodactyly; and 
Telangiectasia) have interstitial lung involvement that is indistinguishable from 
idiopathic fibrosing alveolitis.[13]  
3.4 
Systemic Lupus Erythematosus 
  Acute lupus pneumonitis occurs in only 0.9% of cases and generally develops 
during a generalised flare of SLE with multi-system involvement.  This presents 
clinically as severe dyspnoea of recent onset, tachypnoea, fever, basal crackles 
and hypoxaemia.  CXR shows diffuse basal alveolar infiltrates and pleural 
effusions.  The mortality is high (50%) and survivors have evidence of a 
restrictive ventilatory defect with hypoxaemia.[9][14]  
  Chronic diffuse interstitial lung disease.  Symptomatic interstitial lung disease 
has a prevalence of 3%.  All patients present with dyspnoea and half have 
pleuritic pain.  Restrictive defects are present on pulmonary function testing and 
diffusion capacity is reduced.[9][14]  
  Shrinking lungs syndrome (SLS) is characterised by unexplained dyspnoea, 
small lung volume with restrictive physiology and an elevated diaphragm that is 
thought to be due to a myopathy.  Corticosteroids are the mainstay of treatment 
with most patients returning to their previous pulmonary function.[15]  
 

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3.5 
Polymyositis and Dermatomyositis 
  Interstitial lung disease is detected in 5 - 40% of patients and may present 
clinically as three types[16]: 
1. Acute/sub-acute: with severe rapidly progressive dyspnoea and 
hypoxaemia. 
2.  Chronic: with slowly progressive dyspnoea. 
3. Asymptomatic. 
  The onset of respiratory symptoms may precede, coincide or follow the onset of 
the myositis.  Corticosteroids are the mainstay of treatment. 
 
Immunosuppressant and cytotoxic agents are used second-line.  The decision to 
treat is usually based on clinical, (rather than radiological), activity. 
 
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Extrinsic Allergic Alveolitis 
Synonym: - hypersensitivity pneumonitis. 
4.1 
Description 
Extrinsic allergic alveolitis (EAA) is an immunologically induced inflammatory lung 
disease resulting from repeated inhalations of any one of a variety of causative 
agents, including organic dusts and active chemicals.  This response involves 
antibody reactions, immune complex formation, complement activation and cellular 
responses, causing inflammation of the lung parenchyma, alveolar walls and 
terminal airways.[17]  
4.1.1 
Aetiology 
  Farmer’s and mushroom/malt/sewage-worker’s lung – inhalation of spores from 
various fungi which grow in warm damp hay/straw/grain; vegetable/mushroom 
compost; whisky maltings and sewage.  Occupational exposure to cheese 
mould, mouldy corks and sugar cane mould are also implicated in EAA. 
  Bird-fancier’s lung – inhalation of avian antigens by keepers of racing pigeons 
and pet birds. 
  Rodent-handler’s lung – inhalation of rodent urinary protein. 
  Humidifier lung/ventilation pneumonitis – inhalation of bacteria/fungi/moebae/ 
nematode debris from water in air conditioners/humidifiers. 
  Pituitary snuff-taker’s lung – inhalation of cattle or pig pituitary extracts. 
  Plastic/laboratory workers and paint/vineyard-sprayers – inhalation of solvents, 
isocyanates, fungicides and other chemicals.[17]  Isocyanates cause occupational 
asthma in the majority of cases, with hypersensitivity pneumonitis accounting for 
1-4.7%.[18] 
4.1.2 
Prevalence 
  Estimated to contribute less than 1% to occupational lung disease.[19]  
Approximately 50% of cases affect farm workers, and 15% affect workers in 
material, metal or electrical processing trades.[17]  Smokers are less likely to 
develop all types of EAA. 
  In the UK bird-fancier’s lung is the most prevalent at present, since 12% of the 
population keep birds and of these 0.5 to 7.5% will develop bird-fancier’s lung. 
  In areas of high rainfall, where ‘traditional’ farming methods are used, the 
prevalence of farmer’s lung may reach 10%.  However, where modern farming 
methods are used, the prevalence is 2 - 3% or less and the farming population 
represents only 1 - 2% of the total population. 
  In developed countries humidifier lung is being recognised with increasing 
frequency, both at work and at home. 
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4.2 
Diagnosis 
  There is no diagnostic test that is pathognomonic for EAA.  The clinical 
presentation can be acute, sub-acute or chronic, as summarised in Table 2 
(Appendix A). 
  EAA should be distinguished from the effects of toxins (e.g. paraquat) and dusts 
(e.g. asbestos). 
4.3 
Treatment 
The acute exacerbation of EAA may be treated with corticosteroids, which produces 
a rapid clinical improvement.  There is some debate as to whether this rapid 
improvement increases the likelihood of further exposure, by decreasing antigen 
avoidance.[20]  
The mainstay is complete avoidance of exposure to the provoking antigen.  Those 
unable or unwilling to change their causative occupation can use industrial 
respirators, which filter out 98% of respirable dust from the ambient air.[17]   
Continuing exposure should be accompanied by surveillance (with regular CXRs 
and lung function tests).  When there is progressive disease exposure should cease. 
4.4 
Prognosis 
If exposure to the antigen ceases: The risk of continuing symptoms on cessation of 
the exposure increases with the duration of exposure.  Following acute EAA, 
continuing inflammation and membrane leakiness was found for 2 - 15 years, even 
when patients were asymptomatic.   
Continuation of antigen exposure: The risk of continued exposure is progressive 
fibrosis; however this only occurs in a minority of affected subjects.[17]  A follow-up 
study of farmers with acute farmer’s lung showed that, whilst the majority continued 
to live on farms, only 39% developed radiological changes of fibrosis and only 30% 
developed an impairment of gas transfer.[21]   
 
  
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Main Disabling Effects  
  The chronic interstitial lung disorders cause a restrictive pattern of ventilatory 
impairment, which leads to an overall reduction in lung volume and impaired gas 
diffusion across the alveolar-capillary membrane.  As lung function deteriorates, 
progressive dyspnoea will restrict exertional activities. 
  Corticosteroids are the mainstay of treatment in IPF and other forms of diffuse 
lung disease.  These are used at high doses and for long periods of time, 
increasing the risk of side-effects, which may be disabling (Table 3).   
  Problems may occur with reduced atmospheric pressure.  Commercial air travel 
usually involves depressurisation to a cabin altitude of 6,000ft (1,830m).  The 
reduced partial pressure of oxygen has little effect on healthy travellers, but with 
pulmonary disease may cause symptoms or subtle signs of hypoxia.   
5.1 
Assessing The Claimant 
  Clinical respiratory examination findings do not correlate well with functional 
ability and the assessment is best made from the: 
1.  The History of Activities of Daily Living (Typical Day), taking variation into 
account. 
2.  Informal Observation of the claimant’s activities at examination. 
  In the IB-PCA, the functional areas first affected are ‘walking up/down stairs’, 
and ‘walking’.  Other physical functional areas may be affected by the musculo-
skeletal side effects of high-dose steroid therapy, namely proximal myopathy 
and osteoporotic fracture.  Vision may be affected by the development of 
cataracts and glaucoma.  Mental health may also be affected by high-dose 
steroid therapy and psychological side effects are more common with increasing 
age.[5]  Euphoria may cause patients to underestimate their level of disability. 
  Exemption from the IB-PCA should be considered if the limitation of effort 
tolerance is severe and progressive, causing significant limitation of normal daily 
activities that require more than minimal exertion (e.g. climbing stairs/washing/ 
dressing).  Clinical examination in these cases may show clinical signs of cor 
pulmonale. 
5.2 
Occupational and  Legislative Issues 
  Assessment of fitness for work in the presence of chronic lung disease depends 
essentially on whether the various elements of pulmonary function are adequate 
and whether there is any restriction, through breathlessness, of capacity to 
undertake the level of physical exertion required by the job in its particular 
environment.  Rarely, cough alone may be sufficiently distressing to the worker 
(or fellow workers) to limit effective work capacity. 
  With mild degrees of impairment (e.g. FEV1 > 60% predicted) the results of lung 
function tests correlate poorly with symptoms and the results of exercise tests.  
However, most subjects with FEV1 in the range 40 - 60% of predicted have 
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symptoms on strenuous exertion, and with FEV1 < 40% of predicted heavy 
manual work becomes very difficult to sustain.[22]  
  The degree of impairment of lung function is only one of a number of factors that 
determine work capacity, psychological factors such as motivation and mood are 
also important.   
  Most occupational respiratory hazards are controlled by the Control of 
Substances Hazardous to Health (COSHH) Regulations 1994 and/or the 
management of Health and Safety at Work Regulations 1992.   
  These regulations place a duty on employers to assess whether the use of any 
substance constitutes a risk to the health of employees.  Where a risk of ill 
health exists and medical tests can identify pathological change at an early 
stage (when remedial action can be taken), such tests must be provided.   
  The risk must also be controlled by appropriate means, such as elimination of 
the harmful agent, enclosure of the process, exhaust ventilation or the provision 
of respiratory protection.  Workers exposed to respiratory sensitisers giving a 
risk of EAA should be subject to periodic assessment by questionnaire and 
measurements of ventilatory function.  EAA is a prescribed disease (B6) under 
the Social Security Contributions and Benefits Act (1982), so affected workers in 
the relevant occupations are eligible to claim Industrial Injuries Scheme Benefit 
which, if awarded, is usually payable for life. 
  Most occupational causes of EAA are associated with the handling of mouldy 
vegetable produce; drying vegetable material before storage could prevent most 
cases.   
5.3 
Rehabilitation 
  Pulmonary rehabilitation programmes use a supportive environment in which to 
restore muscle strength and endurance, maximise functional level and improve 
quality of life.   
  Objective measures are necessary to assess functional improvement, such as 
the 6-minute walking test or the shuttle walk test.  It has been claimed that a 6-
minute walking distance of 150 feet represents the minimum distance necessary 
to maintain independent living in an apartment setting.   
  There is little published data on exercise reconditioning and ILD.  One study 
showed an improvement in 6-minute walking distance from 213 to 506 feet after 
the training programme, despite no substantial improvement in pulmonary 
function tests.  Although the improvement appeared substantial, the 
investigators did not assess the impact on quality of life scores.[23]  
  Patients with end-stage interstitial lung disease may not be referred to 
rehabilitative programmes as they may be considered beyond rehabilitation.  
Referral early in the course of the disease may improve functional capacity.[23]  
 
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Appendix A - Tables 
Table 1:  Historical Classification  
 
IDIOPATHIC INTERSTITIAL PNEUMONIAS 
Clinical Terminology 
Pathological Findings 
Idiopathic Pulmonary Fibrosis 
Usual interstitial pneumonia 
Desquamative interstitial  Desquamative interstitial 
pneumonia or 
pneumonia or 
Respiratory bronchiolitis interstitial 
Respiratory bronchiolitis interstitial 
lung disease 
lung disease 
Acute interstitial Pneumonia 
Diffuse Alveolar Damage 
Non specific interstitial pneumonia 
Non specific interstitial pneumonia 
Cryptogenic organising pneumonia 
Organising pneumonia, 
Bronchiolitis obliterans organising 
peribronchiolar inflammation 
pneumonia 
Table 2:  Presentation of Extrinsic Allergic Alveolitis 
 
 PRESENTATION 
Features Acute 
Sub-acute 
Chronic 
Fever Chills 
+ - 

Dyspnoea 
+ + 

Pr
Produc
od
Cough 
Non-productive 
tive 
uct
ive 
Malaise 
+ + 

Weight loss 
- + 

Dif
Crackles 
Bibasal Diffuse 
fus

Nodula
Fib

CXR 
Nodular infiltrates 
ros
infiltrat
is 
es 
Pulmonary Function 
Restrictive Mixed 
Mi
xe
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Tests 

De
Decrea
cre
DLCO 
Decreased 
sed 
as
ed 
Adapted from Grammer 1999[24] 
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Table 3:  Potential Side Effects of High Dose Steroids 
 
 SIDE 
EFFECT 
Osteoporosis 
Vertebral compression fracture 
Musculo-skeletal 
Aseptic necrosis of femoral or 
humeral head 
Myopathy 
Depression 
Psychological 
Euphoria 
Psychosis 
Cardiovascular 
Hypertension 
Truncal obesity 
Hyperglycaemia/diabetes 
Endocrine and Metabolic 
Metabolic alkalosis 
Secondary adrenal insufficiency 
Posterior capsular cataracts 
Ophthalmic 
Raised intra-ocular pressure 
 
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References 
1. 
Du Bois R.  Diffuse Parenchymal Lung Disease.  In: Weatherall D, Ledingham 
J, Warrell D, editors.  Oxford Textbook of Medicine: Oxford University Press, 
1995:2779-2786. 
2. 
Bourke S, Brewis R.  Interstitial Lung Disease.  Lecture Notes on Respiratory 
Medicine.  5th edition ed.  Oxford: Blackwell Science, 1998. 
3. 
Du Bois R.  Diffuse Lung Disease: an approach to management.  British 
Medical Journal 1994;309:175-9. 
4. 
Du Bois R.  Cryptogenic Fibrosing Alveolitis.  In: Weatherall D, Ledingham J, 
Warrell D, editors.  Oxford Textbook of Medicine.  Third ed.  Oxford: Oxford 
University Press, 1995:2786-2795. 
5. 
Anonymous.  American Thoracic Society.  Idiopathic pulmonary fibrosis: 
diagnosis and treatment.  International consensus statement.  American 
Thoracic Society (ATS), and the European Respiratory Society (ERS). 
 
American Journal of Respiratory & Critical Care Medicine 2000;161(2 pt 1):646-
64. 
6. 
Johnston I, Gomm S, Kalra S, Woodcock A, Evans C, Hind C.  The 
management of cryptogenic fibrosing alveolitis in three regions of the United 
Kingdom.  European Respiratory Journal 1993;6:891-3. 
7. 
Ryu J, Colby T, Hartman T.  Idiopathic pulmonary fibrosis: current concepts.  
Mayo Clinic Proceedings 1998;73(11):1085-101. 
8. 
Gross T, Hunninghake G.  Idiopathic pulmonary fibrosis.  New England 
Journal of Medicine 2001;345(7):517-25. 
9. 
Shaw R.  The Lung in Collagen Vascular diseases.  In: Weatherall D, 
Ledingham J, Warrell D, editors.  The Oxford Textbook of Medicine.  Third Ed: 
Oxford University Press, 1995:2796-2800. 
10. 
Fewins H, McGowan I, Whitehouse G, Williams J, Mallaya R.  High definition 
computed tomography in rheumatoid arthritis associated pulmonary disease.  
British Journal of Rheumatology 1991;30:214-6. 
11. 
Gochuico B.  Potential pathogenesis and clinical aspects of pulmonary fibrosis 
associated with rheumatoid arthritis.  American Journal of the Medical Sciences 
2001;321(1):83-8. 
12. 
Rajasekaran B, Shovlin D, Lord P, Kelly C.  Interstitial lung disease in patients 
with rheumatoid arthritis: A comparison with cryptogenic fibrosing alveolitis.  
Rheumatology 2001;40(9):1022-25. 
13.  Semenzato G, Adami F, Maschio N, Agostini C.  Immune mechanisms in 
interstitial lung diseases.  Allergy 2000;55(12):1103-20. 
14.  Cheema G, Quismorio FJ.  Interstitial lung disease in systemic lupus 
erythematosus.  Current Opinion in Pulmonary Medicine 2000;6(5):424-9. 
15. 
Warrington K, Moder K, Brutinel W.  The shrinking lungs syndrome in systemic 
lupus erythematosus.  Mayo Clinic Proceedings 2000;75(5):467-72. 
16.  Hirakata M, Nagai S.  Interstitial lung disease in polymyositis and 
dermatomyositis.  Current Opinion in Rheumatology 2000;12(6):501-8. 
EBM – Interstitial Lung Disease 
Version: 2 a (draft) 
MED/S2/CMEP~0053(n)  
 
Page  17 
 

 
 Medical Services 
 
 
17.  Hendrick D.  Extrinsic Allergic Alveolitis.  In: Weatherall D, Ledingham J, 
Warrell D, editors.  Oxford Textbook of Medicine.  Third ed.  Oxford: Oxford 
University Press,
 1995:2809-2817. 
18.  Raulf-Heimsoth M, Baur X.  Pathomechanisms and pathophysiology of 
isocyanate-induced diseases--summary of present knowledge.  American 
Journal of Industrial Medicine
 1998;34(2):137-43. 
19.  Meyer J, Holt D, Chen Y, Cherry N, Mcdonald C.  Sword 99:surveillance of 
work-related and occupational respiratory disease in the UK.  Occupational 
Medicine
 2001;51(3):204-208. 
20. 
Kokkarinen JI, Tukiainen HO, Terho EO.  Effect of corticosteroid treatment on 
the recovery of pulmonary function in farmer's lung.  American Review of 
Respiratory Disease
 1992;145(1):3-5. 
21.  Braun SR, doPico GA, Tsiatis A, Horvath E, Dickie HA, Rankin J.  Farmer's 
lung disease: long-term clinical and physiologic outcome.  American Review of 
Respiratory Disease
 1979;119(2):185-91. 
22.  Scarisbrick D, Hendrick D.  Respiratory Disorders.  In: Cox R, Edwards F, 
McCallum R, editors.  Fitness for Work.  Second Ed: Oxford University Press, 
1995. 
23.  Markovitz G, Cooper C.  Exercise and interstitial lung disease.  Current 
Opinion in Pulmonary Medicine 1998;4(5):272-80. 
24.  Grammer L.  Occupational allergic alveolitis.  Annals of Allergy, Asthma, & 
Immunology 1999;83(6 Pt 2):602-6. 
 
 
EBM – Interstitial Lung Disease 
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