[vc_row row_type=”row” use_row_as_full_screen_section=”no” type=”full_width” oblique_section=”no” text_align=”left” css_animation=””][vc_column][ultimate_heading main_heading=”Candidate brief” main_heading_color=”#000000″ sub_heading_color=”#000000″ main_heading_style=”font-weight:bold;” main_heading_font_size=”desktop:36px;”]

“This patient has presented to the acute medical unit on account of shortness of breath worsening over several months.  Please examine their respiratory system to identify why then tell the examiners what signs you find and discuss your proposed management”

[/ultimate_heading][vc_empty_space image_repeat=”no-repeat”][vc_video link=”https://vimeo.com/138191692″][vc_empty_space image_repeat=”no-repeat”][vc_row_inner row_type=”row” type=”full_width” use_row_as_full_screen_section_slide=”no” text_align=”left” css_animation=””][vc_column_inner width=”1/4″][stat_counter icon_size=”32″ counter_title=”Station Time” counter_value=”20″ counter_suffix=” minutes” speed=”3″][/vc_column_inner][vc_column_inner width=”1/4″][stat_counter icon_size=”32″ counter_title=”Time for this encounter” counter_value=”10″ counter_suffix=” minutes” speed=”3″][/vc_column_inner][vc_column_inner width=”1/4″][stat_counter icon_size=”32″ counter_title=”Maximum time to examine your patient” counter_value=”6″ counter_suffix=” minutes” speed=”3″][/vc_column_inner][vc_column_inner width=”1/4″][stat_counter icon_size=”32″ counter_title=”Minimum time for discussion and questions” counter_value=”4″ counter_suffix=” minutes” speed=”3″][/vc_column_inner][/vc_row_inner][vc_empty_space image_repeat=”no-repeat”][/vc_column][/vc_row][vc_row row_type=”row” use_row_as_full_screen_section=”no” type=”full_width” oblique_section=”no” text_align=”left” css_animation=””][vc_column][vc_accordion style=”accordion”][vc_accordion_tab title=”Common examiner questions”][vc_column_text]Common examiner questions include the following:

  1. What do you think this patient has?
  2. What clinical signs were you able to elicit?
  3. How would you like to investigate this patient next?
  4. What do you think the underlying cause of this patient’s signs is?

[/vc_column_text][/vc_accordion_tab][vc_accordion_tab title=”Diagnosis and clinical signs”][vc_column_text]This patient pulmonary fibrosis.  This is one of the commonest clinical scenarios encountered in the respiratory station during PACES.

The clinical signs exhibited in this video include:

  1. Clubbing;
  2. Normal-appearing chest expansion (note that in some patients this may be reduced);
  3. Fine fibrotic “Velcro-like” crepitations bibasally.

Candidates should also look for evidence that the patient may be on supplementary oxygen – some portable cylinders may appear quite discrete and won’t necessarily be obvious if you’re not paying attention, and if the patient is on short burst treatment they may not have their nasal cannulae in.[/vc_column_text][/vc_accordion_tab][vc_accordion_tab title=”Discussion”][vc_column_text]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 is floridly clubbed.  This quite strongly supports a diagnosis of idiopathic pulmonary fibrosis, although strictly speaking this is a diagnosis of exclusion once other possible causes of pulmonary fibrosis have been ruled out.

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.[1][2] Thankfully, novel therapies are now coming through which have been shown to improve disease burden and prognosis.

Early Disease

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. [3] However, it is increasingly recognised that even smaller drops in FVC, even as low as 5% confer an increased mortality and poorer prognosis.

Pirfenidone

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

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.

References

  1. 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
  2. Network The Idiopathic Pulmonary Fibrosis Clinical Research. Prednisolone, azathioprine and N-acetylcysteine for pulmonary fibrosis. N Engl J Med2012;366:1968–77
  3. 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

[/vc_column_text][/vc_accordion_tab][vc_accordion_tab title=”Specimen case presentation”][vc_column_text]”This patient has clubbing and basal crackles suggestive of pulmonary fibrosis.  The likely cause is idiopathic pulmonary fibrosis, but I would like to exclude other causes.  To investigate this patient next I would like to arrange a chest x-ray, spirometry and an HRCT chest.  Subsequently I would arrange a bronchoscopy to determine whether the features are in keeping with UIP or NSIP to aid with prognostication.”[/vc_column_text][/vc_accordion_tab][/vc_accordion][/vc_column][/vc_row]