Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveol...

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Main Authors: Yasar Sattar, Ammu Thampi Susheela, Waqas Ullah, Norina Usman, Fnu Zafrullah
Format: Article
Language:English
Published: MDPI AG 2019-07-01
Series:Medicina
Subjects:
Online Access:https://www.mdpi.com/1010-660X/55/7/378
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author Yasar Sattar
Ammu Thampi Susheela
Waqas Ullah
Norina Usman
Fnu Zafrullah
author_facet Yasar Sattar
Ammu Thampi Susheela
Waqas Ullah
Norina Usman
Fnu Zafrullah
author_sort Yasar Sattar
collection DOAJ
description Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH.
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spelling doaj.art-cfe26001c8c74136926d61daa076b90b2023-09-03T02:47:28ZengMDPI AGMedicina1010-660X2019-07-0155737810.3390/medicina55070378medicina55070378Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with PolyangiitisYasar Sattar0Ammu Thampi Susheela1Waqas Ullah2Norina Usman3Fnu Zafrullah4Icahn School of Medicine at Mount Sinai-Elmhurst Hospital, Queens, NY 11373, USABeth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USAAbington Hospital Jefferson Health, Abington, PA 19001, USALasante Health Clinic, Brooklyn, NY 11226, USASteward Carney Hospital, Tufts Medical Center, Boston, MA 02111, USAGranulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH.https://www.mdpi.com/1010-660X/55/7/378diffuse alveolar hemorrhagegranulomatosis with polyangiitisplasmapheresisprednisonecyclophosphamiderituximab
spellingShingle Yasar Sattar
Ammu Thampi Susheela
Waqas Ullah
Norina Usman
Fnu Zafrullah
Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis
Medicina
diffuse alveolar hemorrhage
granulomatosis with polyangiitis
plasmapheresis
prednisone
cyclophosphamide
rituximab
title Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis
title_full Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis
title_fullStr Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis
title_full_unstemmed Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis
title_short Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis
title_sort use of plasmapheresis and immunosuppressants to treat diffuse alveolar hemorrhage in a patient with granulomatosis with polyangiitis
topic diffuse alveolar hemorrhage
granulomatosis with polyangiitis
plasmapheresis
prednisone
cyclophosphamide
rituximab
url https://www.mdpi.com/1010-660X/55/7/378
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