Fibrosing Alveolitis

Pulmonology

Clinicals - History

Fact Explanation
Exertional dyspnoea Breathlessness on exertion begins insidiously and progressively worsens so that the patient ultimately develops dyspnoea at rest Exertional dyspnoea
Breathlessness on exertion begins insidiously and progressively worsens so that the patient ultimately develops dyspnoea at rest
Nonproductive cough Is a common symptom in patients with fibrosing alveolitis Nonproductive cough
Is a common symptom in patients with fibrosing alveolitis
Low-grade fevers Is a nonspecific symptom of fibrosing alveolitis Low-grade fevers
Is a nonspecific symptom of fibrosing alveolitis
Fatigue Can be a symptom of fibrosing alveolitis or symptom of right heart failure secondary to pulmonary hypertension Fatigue
Can be a symptom of fibrosing alveolitis or symptom of right heart failure secondary to pulmonary hypertension
Weight loss Constitutional symptoms are not very common but are seen in some patients Weight loss
Constitutional symptoms are not very common but are seen in some patients
Arthralgia Has been reported in patients with fibrosing alveolitis, more commonly in women Arthralgia
Has been reported in patients with fibrosing alveolitis, more commonly in women
Malaise Initial flu-like illness with malaise is seen in some patients with fibrosing alveolitis Malaise
Initial flu-like illness with malaise is seen in some patients with fibrosing alveolitis
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 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
History of smoking Cigarette smoking has shown to have an association with lung fibrosis History of smoking
Cigarette smoking has shown to have an association with lung fibrosis
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 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
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 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

Clinicals - Examination

Fact Explanation
Bibasal fine end-inspiratory crackles Is the clinical diagnostic sign of fibrosing alveolitis. These can become pan inspiratory in advanced disease Bibasal fine end-inspiratory crackles
Is the clinical diagnostic sign of fibrosing alveolitis. These can become pan inspiratory in advanced disease
Digital clubbing Is seen in 30-50% of patients Digital clubbing
Is seen in 30-50% of patients
Increased respiratory rate Patients become tachypneic with progression of disease. Increased respiratory rate
Patients become tachypneic with progression of disease.
Dyspnea at rest Is a sign of advanced disease Dyspnea at rest
Is a sign of advanced disease
Pedal oedema Can develop due to cor-pulmonale (right heart failure secondary to pulmonary hypertension) Pedal oedema
Can develop due to cor-pulmonale (right heart failure secondary to pulmonary hypertension)
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) 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)
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) 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)
Cyanosis Patients with advanced disease may develop central cyanosis Cyanosis
Patients with advanced disease may develop central cyanosis
Loud P2 Is a sign of pulmonary hypertension, which can arise secondary to advanced fibrosing alveolitis Loud P2
Is a sign of pulmonary hypertension, which can arise secondary to advanced fibrosing alveolitis
Fixed split S2 Is a sign of pulmonary hypertension which, can arise secondary to advanced fibrosing alveolitis Fixed split S2
Is a sign of pulmonary hypertension which, can arise secondary to advanced fibrosing alveolitis
Holosystolic tricuspid regurgitation murmur Can develop due to cor-pulmonale secondary to a dilated right ventricle (right heart failure secondary to pulmonary hypertension) Holosystolic tricuspid regurgitation murmur
Can develop due to cor-pulmonale secondary to a dilated right ventricle (right heart failure secondary to pulmonary hypertension)

Investigations - 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 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
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 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
Rheumatoid factor (RF) About 10% of patients with fibrosing alveolitis will be RF positive Rheumatoid factor (RF)
About 10% of patients with fibrosing alveolitis will be RF positive
Antinuclear antibodies (ANA) About 30% of patients with fibrosing alveolitis will be ANA positive Antinuclear antibodies (ANA)
About 30% of patients with fibrosing alveolitis will be ANA positive
Arterial blood gas analysis PaO2 will be reduced and PaCO2 will be increased Arterial blood gas analysis
PaO2 will be reduced and PaCO2 will be increased
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 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
Diffusion capacity for carbon monoxide[DLCO] Will be decreased in patients with fibrosing alveolitis Diffusion capacity for carbon monoxide[DLCO]
Will be decreased in patients with fibrosing alveolitis
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 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
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 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
C- reactive protein Will be increased but not specific or diagnostic of fibrosing alveolitis C- reactive protein
Will be increased but not specific or diagnostic of fibrosing alveolitis

Investigations - Management

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 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
Lung function tests Serial lung function tests will help to determine the disease progression and response to treatment Lung function tests
Serial lung function tests will help to determine the disease progression and response to treatment

Management - Supportive

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 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
Oxygen therapy Supplemental oxygen therapy given to patients with dyspnoea improves the symptom and the quality of life Oxygen therapy
Supplemental oxygen therapy given to patients with dyspnoea improves the symptom and the quality of life
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. 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.
Palliative care Should be considered in patients with incurable advanced disease Palliative care
Should be considered in patients with incurable advanced disease
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 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
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. 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.

Management - Specific

Fact Explanation
Prednisolone Corticosteroids are considered first line treatment given together with an immuno-modulator but majority are unresponsive to treatment Prednisolone
Corticosteroids are considered first line treatment given together with an immuno-modulator but majority are unresponsive to treatment
Immunomodulator agents (azathioprine or cyclophosphamide) Is given together with prednisolone as first line treatment but most patients do not respond well to treatment Immunomodulator agents (azathioprine or cyclophosphamide)
Is given together with prednisolone as first line treatment but most patients do not respond well to treatment
N -acetylcysteine (NAC) Adding NAC to treatment has shown to have a significantly better treatment effect than
corticosteroid and immunomodulator alone
N -acetylcysteine (NAC)
Adding NAC to treatment has shown to have a significantly better treatment effect than
corticosteroid and immunomodulator alone
Lung transplant Is an option in patients who are unresponsive to medical treatment and have significant functional impairment Lung transplant
Is an option in patients who are unresponsive to medical treatment and have significant functional impairment

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