Pulmonary fibrosis Flashcards
History must cover
The pattern of symptoms
Pets
Occupation
Past medical, medication and smoking history
Signs
Cyanosis
Clubbing
Cachexia
Fine end-inspiratory crackles
What will the Spirometry show?
FEV1/FVC ratio >0.7 = restrictive pattern
Total lung capacity – reduced
Gold-standard investigation for Intestinal lung disease is what?
HRCT
= Honeycombing
Ground-glass change
Traction bronchiectasis – areas of contracting fibrosis pulling the bronchioles apart
What is Idiopathic pulmonary fibrosis?
progressive, fibrotic lung disease of unknown cause
Both genetic and environmental factors have been implicated
It is the commonest form of ILD
Treatment for IPF
Pirfenidone – reduces fibroblast proliferation
Nintedanib – a tyrosine-kinase inhibitor targeting specific growth factors that contribute to fibrosis
What is ‘Farmer’s lung’?
induced by mould spores on hay or straw, was one of the first subtypes identified almost over a century ago and remains one of the most common forms today.
Prompt diagnosis through a thorough history is vital
Treatment for Farmers lung disease
Corticosteroids
Or failing this, other immunosuppressive therapies (eg. cyclophosphamide, azathioprine)
What is Sarcoidosis?
noncaseating granulomas in different organs that impair their function
A 64-year-old man presents to his general practitioner (GP) with a 3-month history of progressively worsening shortness of breath that is worse on exertion and a non-productive cough.
Respiratory examination reveals clubbing, symmetrically reduced chest expansion and fine end-inspiratory crackles on auscultation. Vital signs are stable. His GP suspects a diagnosis of pulmonary fibrosis.
What investigation wold be used for the best diagnosis?
High-resolution CT scan
A 26-year-old woman with a past medical history of cystic fibrosis presents to the general practitioner with a one-month history of bulky, foul-smelling greasy stool. Both physical examination and blood tests are unremarkable.
What is the most appropriate management plan?
Creon supplement
A 78-year-old gentleman is admitted to hospital with bloody diarrhoea and crampy abdominal pain. On examination, his abdomen is soft with mild generalised tenderness and bowel sounds are present. A stool sample is collected and sent for Clostridium Difficile Toxin (CDT) and microscopy, culture and sensitivity (MC&S). He is treated for gastroenteritis with intravenous fluids and analgesia. He later develops paraesthesia and weakness in his legs, which progresses to his upper limbs. A diagnosis of Guillain-Barre Syndrome (GBS) is suspected and he is started on IV immunoglobulin. What is the cause and why?
Campylobacter jejuni
= Guillain-Barré syndrome (GBS) is an immune-mediate demyelination of the peripheral nervous system, which typically presents with ascending and progressive weakness of all four limbs. It is often precipitated by an infection, most commonly Campylobacter jejuni
A 52-year-old male patient is seen in the Respiratory clinic as a follow-up appointment for their Idiopathic Pulmonary Fibrosis (IPF). The Consultant asks you to examine him.
What do you expect to hear on auscultation of the chest?
Fine bi-basal end inspiratory crackles
rose-coloured macules suggest what
Salmonella
A 68-year-old female attends a follow-up appointment at the respiratory clinic. She recently had a high resolution CT (HRCT) scan to investigate chronic, progressive breathlessness and dry cough. The CT scan confirmed a diagnosis of pulmonary fibrosis. Upon further questioning the patient reports that they have had multiple courses of antibiotics in the community for recurrent urinary tract infections (UTIs). What is the most likely drug responsible for the fibrosis?
Nitrofurantoin
