Case presentation

A 32-year-old man presented to the emergency department with haemoptysis and cough developing over a few days. His medical history included MPO-ANCA-associated vasculitis (AAV), since 2014, which had resulted in end-stage kidney disease requiring haemodialysis. His AAV was believed to be exacerbated by the use of cocaine resulting in several relapses in previous years. The patient had been clean for several months and denied cocaine use.

Physical examination revealed an oxygen saturation of 100% with ambient air, a heart rate of 70 beats/min and a blood pressure of 139/94 mmHg. The remainder of the physical examination was unremarkable, in particular no purpura or other skin alterations were seen that could be associated with a relapse of his known vasculitis.

Laboratory studies showed a normocytic anaemia of 6.5 mmol/l with a normal platelet count, low inflammation markers, serum creatinine was 567 μmol /l and urea 8.9 mmol/l. Clotting times were not determined. A chest X-ray showed subtle bilateral consolidations (figure 1, panel A). A CT scan demonstrated opacification of the right lower lobe suggestive of alveolar haemorrhage.

Based upon clinical recognition, the patient’s history and recently measured high ANCA-MPO levels in the weeks prior to admission (>221.9 kU/l), a relapse of AAV was assumed and treatment was initiated with high-dose prednisolone (60 mg a day). Bronchoalveolar lavage (BAL) was performed and aliquots with fresh blood from both lungs were retrieved. Unfortunately, sequential BAL could not be finished because the patient experienced major distress. Cultures and molecular diagnostics of BAL fluid came back negative for infectious agents. Diffuse alveolar haemorrhage was diagnosed based on the combination of the radiological pattern, clinical presentation, high ANCA-MPO level, the findings during BAL and with an infectious or cardiogenic aetiology being unlikely. His clinical situation slowly deteriorated, with progressive dyspnoea, hypoxia and ongoing haemoptysis, despite the start of rituximab at day 5 and high-dose methylprednisolone (1000 mg a day, three days) at day 13.

Acute coronary syndrome as a cause of the clinical deterioration was excluded by ECG and stable troponin levels during follow-up. Dialysis was intensified to avoid any fluid overload. Haemodynamic instability was not observed during dialysis. An echocardiogram was not performed.

On day 17 he was admitted to the ICU requiring respiratory support with high-flow oxygen therapy. The chest X-ray displayed extensive bilateral alveolar consolidation (figure 1, panel C).

With ongoing clinical deterioration leading to respiratory failure, four days after intensifying immunosuppressant therapy with methylprednisolone, plasmapheresis was initiated with the aim of avoiding the need for invasive ventilation and progression of both the life-threatening pulmonary bleeding and pulmonary manifestation of AAV. After initiation of plasmapheresis, clinical improvement occurred rapidly in accordance with major improvement of the radiological findings. Analysis of the patient’s hair confirmed recent use of cocaine.

Discussion

Diffuse alveolar haemorrhage is most commonly due to pulmonary capillaritis and is considered an imminent lifethreatening clinical syndrome, requiring fast diagnosis and treatment. The main symptoms include haemoptysis, anaemia, hypoxia and dyspnoea.[1]