Intestitial Lung Disease Flashcards

1
Q

What’s hypersensitivity type III and hypersensitivity type IV reaction? (in general)

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Diagnosis of Extrinsic Allergic Alveolitis

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Treatment of extrinsic allergic alveolitis

A

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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is Sarcoidosis? (characteristics)

A
  • granulomatous inflammatory condition
  • Granulomas are nodules of inflammation full of macrophages
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Extrapulmonary symptoms of sarcoidosis

A
  • multiple extra-pulmonary manifestations
  • erythema nodosum
  • lymphadenopathy

Main symptoms: chest/lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the population profile typical for sarcoidosis?

A
  • Two peaks: young adulthood and around age 60
  • Women are affected more
  • more frequent in black people
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What may be pulmonary manifestations in sarcoidosis?

A

Lungs (affecting over 90%)

  • Mediastinal lymphadenopathy
  • Pulmonary fibrosis
  • Pulmonary nodules
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Systemic manifestations in sarcoidosis

A

Systemic Symptoms

  • Fever
  • Fatigue
  • Weight loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Skin manifestations in sarcoidosis

A

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What’s Lofgren’s Syndrome

  • triad of symptoms
A

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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Blood tests (and results) in sarcoidosis

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Imaging in sarcoidosis (results)

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What’s a gold standard/ confirmatory of Dx of Sarcoidosis?

What is seen?

A
  • 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.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Other tests (other organ involvement) in sarcoidosis

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Treatment of sarcoidosis?

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Prognosis of sarcoidosis

A
  • 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
17
Q

What’s this?

A

Hilar adenopathy - enlargement

(seen in sarcoidosis)

18
Q

Combination of symptoms in Lofgren syndrome

A

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

19
Q

Treatment of Lofgren syndrome

A

NSAIDs (non steroid anti-inflammatory drug)

Prognosis: disease will resolve in >90% of patients over 2 years

20
Q

What are pneumoconioses?

A

Pneumoconioses

  • group of intestitial lung diseases
  • mostly of occupational origin -> inhalation of mineral or dust
21
Q

What are 3, the most common pneumoconioses?

A

silicosis, asbestosis and coal worker pneumoconiosis

22
Q

Diagnosis of pneumoconioses

A
  • 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
23
Q

Management of pneumoconioses

A

No cure -> but measures to stop from getting worse and to control the symptoms

  • avoiding exposure
  • not smoking
  • oxygen use
  • bronchodilators use
24
Q

What is seen in the imaging of Interstitial pulmonary fibrosis?

A
  • 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
25
Q

What’s seen on the restrictive pattern of spirometry?

A
26
Q

Medical therapy for Idiopathic Pulmonary Fibrosis

A
  • Pirfenidone is an antifibrotic and anti-inflammatory
  • Nintedanib is a monoclonal antibody targeting tyrosine kinase