Plasmapheresis for diffuse alveolar hemorrhage in a patient with Wegener's granulomatosis: case report and review of the literature

J Clin Apher. 2005 Dec;20(4):230-4. doi: 10.1002/jca.20069.

Abstract

We present a case of a 20-year-old male with Wegener's Granulomatosis involving the upper respiratory tract, lungs, and kidneys. In his fourth hospital admission, the patient presented with diffuse alveolar hemorrhage and poor pulmonary function: FiO2 of 100% and PEEP of 17cm H2O on intubation. Due to a fast clinical deterioration while receiving drug therapy (cyclophosphamide and methylprednisolone), we performed nine daily 1-volume therapeutic plasma exchanges (TPE) using 5% albumin as replacement fluid. TPE resulted in a decrease in cytoplasmic anti-neutrophil cytoplasm antibodies (c-ANCA) titer from 1:1,024 to 1:16. On the ninth day of plasmapheresis, his pulmonary status was markedly improved with FiO2 of 60% and PEEP of 8 cm H2O. The patient was later extubated and discharged home in stable condition. Wegener's Granulomatosis with pulmonary hemorrhage is not included in the current guidelines for therapeutic apheresis; therefore, we report this case and, if warranted, propose this condition to be included in the guidelines.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Bronchoalveolar Lavage Fluid
  • Granulomatosis with Polyangiitis / complications*
  • Granulomatosis with Polyangiitis / pathology
  • Granulomatosis with Polyangiitis / therapy
  • Hemorrhage / etiology*
  • Hemorrhage / pathology
  • Hemorrhage / therapy
  • Humans
  • Male
  • Nasal Mucosa / pathology
  • Neutrophil Infiltration
  • Plasmapheresis*
  • Pulmonary Alveoli / pathology
  • Skin / pathology