Intestitial Lung Disease Flashcards
What’s hypersensitivity type III and hypersensitivity type IV reaction? (in general)
Hypersensitivity type III: accumulation of immune complexes (antigen-antibody complex) —>
inflammatory response + attraction of leukocytes
Hypersensitivity type IV: (delayed type) reaction takes several days to develop; unlike other
types it is not antibody - mediated but cell -mediated response
Diagnosis of Extrinsic Allergic Alveolitis
A) symptoms after exposure + history
B) clinical tests: blood test (inflammation), CXR and lung function test (restrictive loss of lung
function)
C) lung biopsy - to confirm diagnosis
Treatment of extrinsic allergic alveolitis
A) Avoidance of provoking antigen (chronic exposure - permanent damage); if avoidance is
difficult e.g. farmer’s lung —> protective masks with filters use
B) Corticosteroids (e.g. prednisolone) - > to relieve symptoms (but side effects)
What is Sarcoidosis? (characteristics)
- granulomatous inflammatory condition
- Granulomas are nodules of inflammation full of macrophages
Extrapulmonary symptoms of sarcoidosis
- multiple extra-pulmonary manifestations
- erythema nodosum
- lymphadenopathy
Main symptoms: chest/lungs
What is the population profile typical for sarcoidosis?
- Two peaks: young adulthood and around age 60
- Women are affected more
- more frequent in black people
What may be pulmonary manifestations in sarcoidosis?
Lungs (affecting over 90%)
- Mediastinal lymphadenopathy
- Pulmonary fibrosis
- Pulmonary nodules
Systemic manifestations in sarcoidosis
Systemic Symptoms
- Fever
- Fatigue
- Weight loss
Skin manifestations in sarcoidosis
Skin (affecting around 15%)
- Erythema nodosum (tender, red nodules on the shins caused by inflammation of the subcutaneous fat)
- Lupus pernio (raised, purple skin lesions commonly on cheeks and nose)
- Granulomas develop in scar tissue
What’s Lofgren’s Syndrome
- triad of symptoms
Lofgren’s Syndrome
This is a specific presentation of sarcoidosis. It is characteristic by a triad of:
- Erythema nodosum
- Bilateral hilar lymphadenopathy
- Polyarthralgia (joint pain in multiple joints)
Blood tests (and results) in sarcoidosis
- Raised serum ACE. This is often used as a screening test.
- Hypercalcaemia (rasied calcium) is a key finding.
- Raised serum soluble interleukin-2 receptor
- Raised CRP
- Raised immunoglobulins
Imaging in sarcoidosis (results)
- Chest xray shows hilar lymphadenopathy
- High resolution CT thorax shows hilar lymphadenopathy and pulmonary nodules
- MRI can show CNS involvement
- PET scan can show active inflammation in affected areas
- EBUS (fine - needle aspiration of
mediastinal lymph nodes); tissue from biopsy will be tested to rule out other cases e.g. cancer, microorganisms
What’s a gold standard/ confirmatory of Dx of Sarcoidosis?
What is seen?
- histology from a biopsy
- done by doing bronchoscopy with ultrasound guided biopsy of mediastinal lymph nodes - EBUS
- histology shows characteristic non-caseating granulomas with epithelioid cells.
Other tests (other organ involvement) in sarcoidosis
- U&Es for kidney involvement
- Urine dipstick or urine albumin-creatinine ratio to look for proteinuria indicating nephritis
- LFTs for liver involvement
- Ophthalmology review for eye involvement
- ECG and echocardiogram for heart involvement
- Ultrasound abdomen for liver and kidney involvement
Treatment of sarcoidosis?
- No treatment is considered as first line in patients with no or mild symptoms as the condition often resolves spontaneously
- Oral steroids are usually first line where treatment is required and are given for between 6 and 24 months. Patients should be given bisphosphonates to protect against osteoporosis whilst on such long term steroids.
- Second line options are methotrexate or azathioprine
- Lung transplant is rarely required in severe pulmonary disease
Prognosis of sarcoidosis
- Sarcoidosis spontaneously resolves within 6 months in around 60% of patients
- small number of patients it progresses with pulmonary fibrosis and pulmonary hypertension, potentially requiring a lung transplant
- death in sarcoidosis is usually when it affects the heart (causing arrhythmias) or the central nervous system
What’s this?
Hilar adenopathy - enlargement
(seen in sarcoidosis)
Combination of symptoms in Lofgren syndrome
Combination of: erythema nodusm (inflammation of fat cells under the skin —> red and tender
lumps on both shins) + hilar lymphadenopathy (enlargement of lymph nodes near to the inner
border of the lungs) + joint pain
Treatment of Lofgren syndrome
NSAIDs (non steroid anti-inflammatory drug)
Prognosis: disease will resolve in >90% of patients over 2 years
What are pneumoconioses?
Pneumoconioses
- group of intestitial lung diseases
- mostly of occupational origin -> inhalation of mineral or dust
What are 3, the most common pneumoconioses?
silicosis, asbestosis and coal worker pneumoconiosis
Diagnosis of pneumoconioses
- history of work exposure
- physical examination
- pulmonary function studies
- biopsy
- Chest X-ray/ CT characteristics: patchy, subpleural, bibasilar intrstitial infiltrates or small cystic radilucencies = honeycombing
Management of pneumoconioses
No cure -> but measures to stop from getting worse and to control the symptoms
- avoiding exposure
- not smoking
- oxygen use
- bronchodilators use
What is seen in the imaging of Interstitial pulmonary fibrosis?
- lower volume of diseased lungs compared with normal lungs due to restrictive nature of disease = volume loss
- on CXR - lots of lines - reticulations = fibrous tissue on the lungs
- honeycombing -> advanced fibrosis
What’s seen on the restrictive pattern of spirometry?
Medical therapy for Idiopathic Pulmonary Fibrosis
- Pirfenidone is an antifibrotic and anti-inflammatory
- Nintedanib is a monoclonal antibody targeting tyrosine kinase
