A 56-year-old gentleman was admitted to the Emergency Department with a two-week history of progressively increasing shortness of breath, with frank haemoptysis for the last couple of days. By the point of admission, he was short of breath on minimal exertion and at rest and was unable to complete full sentences. He also complained of increasing orthopnoea for the last few weeks. He had a long history of recurrent epistaxis, rhinitis and sinusitis, as well as a past medical history of diabetes, hypertension, hypercholesterolaemia and gout. His medication regimen comprised of Mometasone nasal spray, Allopurinol 100mg OD, Ramipril 2.5 mg OD, Amlodipine 5mg OD, Simvastatin 40mg OD, Metformin 500mg TDS and Gliclazide 80mg OD. He smoked 20 cigarettes per day and did not consume alcohol.
On examination, he appeared very unwell with obvious respiratory distress. His respiratory rate was 28/min, oxygen saturation was 90% on room air, BP was 108/72 mmHg, heart rate 130/min and the temperature was 37.9 C. Examination of his cardiovascular system revealed normal heart sounds with a JVP of 3 cm. Examination of the respiratory system revealed the use of accessory muscles with bibasal fine crackles. Examination of the gastrointestinal and neurological systems was unremarkable.
Initial investigations revealed the following:
18. Hb |
85 g/l |
Platelets |
331 * 109/l |
WBC |
15.2 * 109/l |
ESR |
79 mm/hr |
Na+ |
128 mmol/l |
K+ |
6.0 mmol/l |
Urea |
29 mmol/l |
Creatinine |
738 µmol/l |
CRP |
52 mg/l |
Glucose |
5.8 mmol/l |
Chest x-ray: bilateral patchy infiltration
ECG: heart rate 130 bpm and sinus rhythm.
Urinalysis: Blood ++++, Protein +++, Leuc/Nitrites – negative, Glucose – negative
ABG on 15 L/min oxygen:
PaO2 |
125 mmHg |
PaCO2 |
21 mmHg |
HCO3 |
18.3 mmol/l |
pH |
7.49 |
He was commenced on an IV infusion of normal saline, as well as Co-amoxiclav 625mg TDS, and quickly transferred to the Intensive Care Unit. He was cathetrized. A decision was made to institute immediate haemodialysis, for which preparations were being made.
Further investigations revealed the following:
Urine MCS: nil grown
Blood culture – interim results: nil grown
Transoesophageal echocardiogram: normal systolic function, valvular appearances and no vegetations seen
C3 |
1.22 (NR 0.65 – 1.65g/L) |
C4 |
0.32 (NR 0.16 – 0.60 g/L) |
ANA |
negative |
ENA |
negative |
dsDNA |
negative |
cANCA |
positive |
pANCA |
negative |
Rheumatoid factor |
negative |
Given the likely underlying diagnosis, which of the following interventions is the next best step whilst awaiting haemodialysis?
The diagnosis here is granulomatosis with polyangiitis, with severe pulmonary and renal involvement. The patient requires urgent immunosuppression. Of the available options, the next best step is to institute cyclophosphamide and methylprednisolone.