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Gold standard case presentation:
Interstitial lung disease / idiopathic pulmonary fibrosis
For the MRCP PACES examination, candidates would need to confidently identify the presence of a pulmonary fibrosis. They would also be expected to propose an appropriate management plan and provide some differentials regarding the underlying cause.
In the video shown here the patient exhibits lower zone fine crackles in keeping with interstitial lung disease.
Initial investigation is with a chest x-ray and spirometry, swiftly followed by HRCT chest and bronchoscopy. The goal being to determine the underlying pathological mechanism and exclude infection.
Following such investigations, a diagnosis of either usual interstitial pneumonia or non-specific interstitial pneumonia would be anticipated. These differ markedly in terms of prognosis. UIP is generally typified by relentless progression with poor long-term survivial, whereas NSIP has a far more favourable outlook with excellent five year survival rates.
Remember the difference as follows: U=unfortunate, NS=not so bad.
Interstitial lung disease has long been a condition treated with corticosteroids, which has recently been found to have no impact upon the prognosis, and even be adversely affecting patients. The previous advice of triple therapy with prednisolone, azathioprine and N-acetylcysteine (NAC) was found with the PANTHER-IPF trial to increase hospitalisation and mortality in the treatment arm. Thankfully, novel therapies are now coming through which have been shown to improve disease burden and prognosis.
Under the National institute of Clinical Excellence (NICE) guidance, patients with forced vital capacity (FVC) of greater than 80% of predicted do not qualify for treatment. Treatment is advised if FVC is between 50-80%. In those who do not qualify, or do not want treatment, spirometry every 3-6 months is advised to monitor disease severity.
FVC drop of greater than 10% or transfer factor for carbon monoxide (TLCO) of greater than 15% demonstrates significant progression of disease and hence significant increases in mortality in the following 12 months.  However, it is increasingly recognised that even smaller drops in FVC, even as low as 5% confer an increased mortality and poorer prognosis.
Pirfenidone is the only drug in the United Kingdom to be licensed for treatment (although see below), for patients with FVC of between 50-80%. The exact mechanism of action is unknown. Low body mass index (BMI) and frailty are associated with an increased side effect profile. Treatment failure is defined as a fall of over 10% within the first year of treatment, which should lead to cessation of treatment.
Side effe primarily gastrointestinal, such as nausea and vomiting. In addition to generalised fatigue.
Nintedanib, which is not currently licensed by NICE (but will likely be in the future) blocks receptors to specific growth factors, in particular vascular endothelial growth factor, platelet-derived growth factor and FGF, which are recognised modulators of fibrogenic pathways and, as well as pirfenidone, has been shown to reduce inflammation and fibrosis.
Side effects are again gastrointestinal, and in this case mild to moderate diarrhoea.
- Bradley B, Branley HM, Egan JJ, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008;63:v1–58
- Network The Idiopathic Pulmonary Fibrosis Clinical Research. Prednisolone, azathioprine and N-acetylcysteine for pulmonary fibrosis. N Engl J Med2012;366:1968–77
- du Bois RM, Weycker D, Albera C, et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med 2011;184:1382–9
- Review / Skip
Question 1 of 5
1. Question1 point(s)
What could you hear when auscultating the patient’s chest?CorrectIncorrect
Question 2 of 5
2. Question1 point(s)
This lady presented with progressive breathlessness, but has no relevant past medical history or medication. What is the likely diagnosis.CorrectIncorrect
Question 3 of 5
3. Question1 point(s)
What defect would you expect to see on spirometry in interstitial lung disease?CorrectIncorrect
Question 4 of 5
4. Question1 point(s)
A chest x-ray shows interstitial shadowing and spirometry confirms a restrictive defect. Which test would be most useful to get next?CorrectIncorrect
Question 5 of 5
5. Question1 point(s)
What is the commonest type of interstitial lung disease?