Interstitial Lung Disease + Sarcoidosis Flashcards
What are Interstitial lung diseases?
Collective term for a group of disorders that cause inflammation and fibrosis disrupting the interstitium and the parenchyma of the lungs
How do patients with interstitial lung diseases typically present?
Chronic:
-SOB
-dry/non productive cough
-fatigue
Dry Inspiratory crackles
Finger clubbing
Chest pain
Weight loss?
What are the 2 main types of Interstitial Lung Disease?
Primary ILD
Secondary ILD
What is primary interstitial Lung disease?
Idiopathic/not caused by anything else
What is secondary interstitial lung disease?
When the interstitial lung disease is caused by something else
What is the most common cause of primary interstitial lung diseases?
Idiopathic pulmonary fibrosis / idiopathic interstitial pneumonia
What are some examples to ask a patient you think might have an interstitial lung disease?
SOB?
Cough non-productive?
Chest pain?
Occupational history
Family history and PMH
Acute on chronic? (Infectious signs)
What are some medications that can cause secondary interstitial lung disease?
Amiodarone
Bleomycin
Nitrofurantoin
What are some occupational causes of interstitial lung diseases?
Pneumoconiosis
Silicosis
Asbestosis
Byssinosis (cotton)
Hypersensitivity pneumonitis:
-bird fanciers lung
-cheese workers lung
What pre-existing medical conditions can cause pulmonary fibrosis?
Rheumatoid arthritis
Systemic sclerosis
Idiopathic myositis
Primary Sjögren’s syndrome
Mixed connective tissue disease
Sarcoidosis
Alpha-1-anti trypsin deficiency
What first line investigations would you do for a patient presenting with chronic SOB, chest pain and a dryer productive cough? (?ILD)
Routine bloods
ABG
O2 sats
6min walk tests
ECG
CXR
When you are then suspecting interstitial lung disease after doing your first line investigations, what further investigations will you do to help diagnose?
HRCT
Pulmonary function tests/Spirometryy
Echocardiography (pulmonary HTN)
Bronchoscope+- biopsy
Autoantibody screen (ANA, anti-Scl-70, rheumatoid factor, anti ro, anti la)
Bronchioalveolar lavage
Lung biopsy
What is expected to be seen on a HRCT of a patient with interstitial lung disease?
Bilateral bi-basal reticular shadowing
Honey combing
Ground glass opacity for idiopathic pulmonary fibrosis
Mediastinal lymphadenopathy
What type of result would you expect to see on pulmonary function tests/spirometry for an interstitial lung disease?
Restrictive curve (wizard hat) NOT SCALLOPING, that’s for obstructive
FEV1:FVC ratio above 0.7
How do you manage an interstitial lung disease?
Oxygen therapy
Cease smoking
Avoid cause
Treat autoimmune condition
Physiotherapist
Transplant if severe and young patient
What types of medications do you give to treat/manage idiopathic pulmonary fibrosis?
Antifibrotic agents
What are some anti-fibrotic medications you can give to slow the progression of idiopathic pulmonary fibrosis?
Perfenidone
Nintedanib
Both reduce fibrosis and inflammation.
What is the pathophysiology of hypersensitivity pneumonitis?
Extrinsic allergic alveolitis involving type III and type IV hypersensitivity reactions to inhaled allergens
How is a hypersensitivity pneumonitis diagnosed?
Bronchoalveolar lavage contains lymphocytes
How is hypersensitivity pneuomitis treated?
Oxygen therapy
Corticosteroids
What is sarcoidosis?
Non caseating granulomatous mutlisystem disease that most commonly affects the lungs
What are granulomas?
Inflammatory nodules containing macrophages
What is the difference between the granulomatous inflammation seen in TB compared to sarcoidosis?
TB = caseating granulomatous inflammation
Sarcoidosis = non caseating granulomatous inflammation
Who is sarcoidosis most common in?
Black women. 20-40 or 60+
What parts of the body can sarcoidosis affect?
Lungs
Kidneys (stones)
Heart (Heart block)
MSK (arthritis)
GI
Cutaneous
CNS (facial palsy)
Ophthal (uveitis)
What are some extra pulmonary skin changes that are often seen with sarcoidosis?
Erythema nodosum
Lupus pernio
What is erythema nodosum and where is it commonly seen with sarcoidosis?
Nodules of inflammed subcutaneous fat on the shins
What is lupus pernio seen in sarcoidosis?
Specific feature of sarcoidosis
Raised purple skin lesions on chin and nose
What affect does sarcoidosis have on the lungs?
Mediastinal lymphadenopathy
Pulmonary nodules
Pulmonary fibrosis
What systemic effects can sarcoidosis have on the body?
Fever
Malaise
Weight loss
What affect can sarcoidosis have on the liver?
Liver nodules
Cholestasis
Cirrhosis
What effect can. Sarcoidosis have on the eyes?
Uveitis
Conjunctivitis
Optic neuritis
What affect can sarcoidosis have on the heart?
Bundle branch block
Heart block
Myocardium involvement
What affect can sarcoidosis have on the kidneys?
Stones
Intersitial. Nephritis
What affect can sarcoidosis have on the CNS?
Diabetes Insipidus (pituitary)
Nodules
What affect can sarcoidosis have on the MSK system?
Arthritis
Arthralgia
Myopathy
What is lofgrens syndrome?
Triadic presentation for sarcoidosis
-Erythema nodosum
-bilateral hilar lymphadenopathy
-poly Arthralgia
What investigations would you do for a patient who you think might have sarcoidosis?
Routine bloods
LFTs (liver or gall bladder involvement?)
U+Es (kidney involvement, checking for Hypercalcaemia)
Urinalysis (Hypercalciuria, urine albumin-creatinine ratio for proteinuria)
ECG and ECHO if cardiac involvement?
Ophthalmology if eye involvement
SERUM ACE
If you could only do 2 investigations for sarcoidosis, which ones would you do?
Serum ACE levels (is it elevated)?
Hypercalcaemia?? U+Es
What imaging would you request to investigate a potential sarcoidosis?
Initially a CXR
Then a HRCT if needed
US if you think liver or kidney involvement
Why is serum ACE a good investigation to determine the likelihood of sarcoidosis?
ACE expression increased with granulomas
How many stages of classification are there for sarcoidosis based on a CXR?
4:
I
II
III
IV
What is stage I of sarcoidosis on a CXR?
Bilateral hilar lymphadenopathy/lymph involvement without lung involvement
JUST LYMPH NODES
What is stage II of sarcoidosis on a CXR?
Bilateral hilar lymphadenopathy + Pulmonary infiltrations/lung involvement
(Lymph nodes and parenchymal involvement)
What is stage III of sarcoidosis on a CXR?
Pulmonary infiltrations without bilateral hilar lymphadenopathy (lymph nodes regress and its mainly lung parenchyma involved)
What is stage IV of sarcoidosis on a CXR?
Pulmonary fibrosis
What is the management of sarcoidosis?
Smoking cessation
Oral corticosteroids first line (6months-24months):
-Bisphosphonates + Calcium + Vit D
-PPIs
2nd line:
-methotrexate
What may be considered if patietn has severe pulmonary disease with sarcoidosis?
Lung transplant
