A 67-year-old male patient presented with worsening shortness of breath and productive cough with intermittent febrile episodes. He also complained of a 20-pound unintentional weight loss over the last few months. His medical history was positive for emphysema-type chronic obstructive pulmonary disease (COPD) on 3 litres of home oxygen therapy diagnosed 3 years previously with right upper lobe bullous changes, IgM deficiency on replacement therapy that had been diagnosed 4 years previously after recurrent respiratory tract infections, and hypothyroidism. No history of body implants was noted. He had a negative colonoscopy 4 years previously. He also had a history of smoking and a previous diagnosis of idiopathic pulmonary fibrosis diagnosed a few months earlier where extensive work-up for autoimmune disease was unyielding. In the emergency department, examination showed tachypnoea with a respiratory rate of 30 cycles per minute, bilateral diminished breaths sounds, bilateral diffuse crackles and thin build. Examination also showed dystrophic yellowish nails on all extremities (Fig. 1) with no objective fever. Laboratory finding showed only leucocytosis with lymphopenia at 2 (reference 20-53%). A chest x-ray showed worsening bilateral infiltrates and pleural effusion compared with the same study findings 1 month earlier (Fig. 2). The patient was started on high-flow oxygen therapy, systemic steroids and bronchodilators, and empiric antibiotic therapy.
Computed tomography (CT) of the chest showed severe rapid cystic, cavitary and bronchiectatic changes mainly in the right upper lobe with extensive peribronchial thickening and persistent left lower lobe mass-like infiltrate (Fig. 3). Infectious work-up was negative. Thoracentesis showed exudative serosanguineous fluid that was negative for malignancy, bacterial and fungal infection, and tuberculosis. Nail clipping and histopathological examination showed no evidence of fungal infection. Mild initial improvement in clinical status was achieved and allowed for bronchoscopy (Fig. 4) which showed copious mucoid secretions. Bronchoalveolar lavage (BAL) with transbronchial biopsies were obtained during the procedure. Biopsy was negative but likely inadequate given the lack of observed alveolar macrophages. BAL bacterial culture, silver stain, acid fast bacilli culture and stain were negative. However, fungal culture was positive for Candida albicans. BAL analysis showed predominant inflammatory cells, mainly macrophages.
Despite escalation of supportive care, the patient's respiratory status continued to worsen with increased incremental oxygen requirements over 2 weeks of hospitalization. The patient refused aggressive medical intervention. Palliative care discussion prompted consideration of comfort care.