Plasmapheresis for diffuse alveolar hemorrhage in a patient with Wegener's granulomatosis: case report and review of the literature.
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.
Nguyen
Thong
T
University of Texas Medical Branch, Pathology Department Galveston, Texas 77555-0743, USA. thonguye@utmb.edu
Martin
Meri King
MK
Indrikovs
Alexander J
AJ
D000328:Adult; D001992:Bronchoalveolar Lavage Fluid; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000473:pathology / Q000628:therapy; D006470:Hemorrhage / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D006801:Humans; D008297:Male; D009297:Nasal Mucosa / Q000473:pathology; D020556:Neutrophil Infiltration; D010956:Plasmapheresis; D011650:Pulmonary Alveoli / Q000473:pathology; D012867:Skin / Q000473:pathology