Interstitial & Occupational Flashcards
Causes of pulmonary fibrosis B R E A S T C A
Bleomycin Radiation Extrinsic allergic alveolitis Ankylosing spondylitis Sarcoidosis Tuberculosis Cryptogenic fibrosing alveolitis Asbestosis
What is a granuloma?
What is the most common granulomatous lung disease?
A mass or nodule composed of chronically inflamed tissue formed by the response of the mononuclear phagocyte system (macrophage/histiocyte) to an insoluble or slowly soluble antigen or irritant.
Sarcoidosis (multisystem)
Sarcoidosis
Aetiology
Epidemiology
Unknown aetiology.
Epidemiology is afro-caribbean/female
What are the symptoms and signs of sarcoidosis?
Fatigue Weight loss Hilar lymphadenopathy Skin lesions Eye lesions Hypercalcaemia High serum ACE level Restrictive defect on lung function tests General respiratory symptoms
What is the treatment of sarcoidosis?
Usually self limiting but if not improved after 6 months, commence on prednisolone 30 mg for 6 weeks, then reducing to lower dose for 6-12 months
Wegener's Granulomatosis What type of disease? (Clue is in name) What is it characterized by? What is seen on CXR? Where are the best histological changes seen and what do they show?
Granulomatous disease with vasculitis.
Characterized by lesions involving the URT, lungs and kidneys.
On CXR - single or multiple nodular masses or pneumonic infiltrates with cavitation.
Best is renal biopsy - shows necrotizing microvascular glomerulonephritis
Churg-Strauss syndrome What type of disease? Who does it typically occur in? Typical presentation? Blood eosinophil count? What parts of the body does it involve? What class of drug treatment?
Granulomatous with vasculitis
Men in their 40s
Rhinitis & asthma, eosinophilia, systemic vasculitis
High blood eosinophil count
Typically involves the lungs, peripheral nerves & skin
Corticosteroids
What pulmonary conditions can be caused by rheumatoid disease?
Pleural adhesions, thickening and effusions
Fibrosing alveolitis
Rheumatoid nodules
Caplan’s syndrome - due to a combination of dust inhalation and the disturbed immunity of rheumatoid arthritis. It occurs particularly in coal workers pneumoconiosis but can occur in individual exposed to other dust e.g silica and asbestos
Systemic lupus erythematosus What is the most common manifestation? What type of disease is this? What organs does it affect? What is the course of the disease? Aetiology? Symptoms? Treatment
Pleurisy - inflammation of he pleura with or without an effusion
Autoimmune - the body attacks its own tissues
Affects the heart, joints, skin, lungs, blood vessels, liver, kidneys, and nervous system
Unpredictable, with periods of illness (called flare-ups) alternating with remissions
Afro-caribbean women
Low grade fever, photosensitivity, mouth ulcers, muscle aches, arthritis, poor peripheral circulation, “butterfly” rash on face
Treatment is with corticosteroids and anti-malarials
Idiopathic Pulmonary Fibrosis Typical presentation and progression? On auscultation? What do CXR, HRCT and lung function tests show? What type of cellular infiltrate? Treatment?
Progressive breathlessness, a non-productive cough, cyanosis, eventually leading to resp failure, pulmonary hypertension and cor pulmonale.
Bilateral fine end inspiratory crackles
CXR - ground glass appearance, followed by irregular reticulonodular shadowing
HRCT - subpleural reticular abnormalities with thick walled cysts
Restrictive defect
Increased numbers of neutrophils and macrophages
Treatment is prednisolone for disabling disease but no cure
Cryptogenic Organizing Pneumonia
Presentation
Treatment
Patients present with single or recurrent episodes of malaise associated with cough, breathlessness and fever
Treatment - corticosteroids
Goodpasture's Syndrome Symptoms and progression CXR Buzzwords Treatment
Initially, symptoms of URT infection, followed by a cough and intermittent haemoptysis, tiredness and eventually anaemia, although massive bleeding may occur.
CXR - transient blotchy shadows due to intra-pulmonary haemorrhage
Buzzwords - acute glomerulonephritis, anti-basement membrane antibodies
Treatment - self limiting or corticosteroids
Allergic Bronchopulmonary Aspergillosis What type of reaction and causative organism? Clinical features? Investigations? - Peripheral blood eosinophil count - Serum IgE level Treatment
Hypersensitivity reaction when the bronchi are colonized by Aspergillus
Clinical features - episodes of eosinophilic pneumonia throughout the year, particularly in autumn & winter. The episodes present with wheeze, cough, fever & malaise
PBEC - high
Serum IgE level - extremely high
Treatment is with prednisolone 30 mg daily
Buzzword “halo sign” above a round lesion
Aspergilloma
Hypersensitivity pneumonitis What is it? Examples? Histology? Clinical features? Investigations? Management
Disease with widespread diffuse inflammatory reaction in both small airways and alveoli due to inhalation of a variety of antigens.
E.g. farmer’s lung, bird fancier’s lung, maltworker’s lung etc
Initial infiltration with neutrophils, followed by T cells and macrophages, then development of granulomas.
Clinical features - fever, malaise, cough, SOB after exposure to antigen
CXR - fluffy nodular shadowing & streaky shadows
Polymorphonuclear leukocyte count is raised in acute cases
Prevention is the aim but prednisolone can be used
What two syndromes result from coal-workers penumoconiosis?
- Simple pneumoconiosis - simply reflects the deposition of coal dust in the lung, producing a fine micronodular shadowing on CXR.
Most common type of pneumoconiosis.
There are lots of small round opacities - Progressive massive fibrosis - patients develop large round fibrotic masses in the upper lobes
Which professions get silicosis?
Buzzword?
Stonemasons, sand blasters, pottery & ceramic workers
Egg-shell calcification
Berylliosis is associated with which industry?
Aerospace
What is chronic hypersensitivity pneumonitis?
Extensive fibrosis with honeycombing and air-trapping. Treatment is focused on antigen avoidance
Extrinsic Allergic Alveolitis What type of hypersensitivity? Pathological description Acute symptoms, signs, CXR and treatment Chronic signs, antibody found, treatment
Type III (immune complexes) hypersensitivity
PD - hypersensitivity pneumonitis
Acute - cough, SOB, fever, myalgia, crackles (no wheeze), hypoxia; CXR - widespread pulmonary infiltrates; treatment - oxygen, steroids, antigen avoidance
Chronic - progressive SOB, cough, crackles, CXR - pulmonary fibrosis in upper zones; IgG antibody; treatment - remove antigen exposure, oral steroid if breathless
Idiopathic pulmonary fibrosis CXR appearance CT scan Pathology Treatment
Bilateral infiltrates
Reticulonodular fibrotic change, worse at lung bases, “ground glass” suggests alveolitis
Pathology - chronic inflammatory infiltrate (neutrophils and fibrosis in alveolar walls, intra alveolar macrophages)
Treatment - steroids and immunosuppressives do not change the course of the disease; Pirfenidone is a new anti-fibrotic drug
Where in the lung is scarring from sarcoidosis typically found?
Apical
What causes fibrosis in the upper zones of the lungs?
Extrinsic allergic alveolitis, coal workers pneumoconiosis, TB, CF
What causes fibrosis in the lower zones of the lungs?
Rheumatoid arthritis, interstitial lung disease, congenital immune deficiency, recurrent aspiration
