Pulmonary renal syndrome (PRS) is a combination of diffuse pulmonary haemorrhage and
glomerulonephritis (GN). Though an established form of presentation in
anti-neutrophil cytoplasmic autoantibody (
ANCA)-associated GN and
vasculitis, diffuse pulmonary haemorrhage is extremely unusual in those with
ANCA-negative GN. We present here a case of a 76-year-old Hispanic female with stage IV
chronic kidney disease (serum
creatinine of 2 mg/dL), who presented with diffuse alveolar haemorrhage and nephritic syndrome. Less than 1 week prior to the full-blown PRS, she was treated for an apparent
pneumonia as was evidenced by a right lower lobe infiltrate on her chest X-ray. Retrospectively, this was likely a focal pulmonary haemorrhage.
ANCA were persistently negative, and the remainder of her immunologic workup was normal. Renal biopsy was diagnostic of crescentic pauci-immune GN. The patient required a
ventilator and haemodialysis support (serum
creatinine 6 mg/dL), and was successfully treated with
methylprednisolone,
cyclophosphamide and a total of six cycles of
plasmapheresis. Once her
oliguria resolved, the
creatinine plateaued at 2.7 mg/dL. Our case illustrates that diffuse alveolar haemorrhage can be a distinct clinical feature even in patients with
ANCA-negative pauci-immune crescentic
glomerulonephritis.