MRCP2-4143
A 57-year-old smoker was referred to the respiratory outpatient clinic by his GP with a history of progressively worsening shortness of breath. His symptoms started several months ago with shortness of breath on extreme exertion but he gradually developed shortness of breath with minimal activity. He also complained of increasing tiredness and a non-productive cough had lost approximately 3 pounds in 6 months. He denied the presence of sputum production, haemoptysis or chest pain, and also denied the presence of both orthopnoea and paroxysmal nocturnal dyspnoea. He had a past medical history comprising ankylosing spondylosis diagnosed 30 years ago as well as hypertension and hypercholesterolaemia. His drug history comprised naproxen 500mg BD, lansoprazole 30 mg OD, felodipine M/R 2.5mg OD and atorvastatin 20 mg OD. He stated that he had been trialled with a course of methotrexate a few years ago for his ankylosing spondylosis and was subsequently stopped.
Examination revealed the presence of a well male with a blood pressure of 146/86 mmHg, heart rate 74 bpm and respiratory rate of 18/min. His oxygen saturations were 93% on air. There was no BCG scar seen on examination of his arm. Examination of his respiratory system revealed the presence of bilateral fine upper zone crackles but nil else and no respiratory distress. Examination of his cardiovascular system revealed no abnormalities including a normal JVP and the absence of pedal oedema, and examination of his gastrointestinal system was likewise unremarkable.
Initial investigations revealed the following:
Hb 166 g/l
Platelets 341 * 109/l
WBC 6.3 * 109/l
ESR 34 mm/hr
CRP 26 mg/l
Chest x-ray: Bilateral apical fibrosis
Pulmonary function testing
FEV1 2.4 l (predicted value 2.3)
FVC 2.6 l (predicted value 4.8)
FEV1/FVC ratio 92%
TLCO transfer factor 86% of predicted value
What is the most likely diagnosis?