Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (GPA), previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical...

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Main Authors: Robert Ali, Candice Baldeo, Jesse Onyenekwe, Roshan Lala, Cristian Landa, Anwer Siddiqi
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2015/513602
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author Robert Ali
Candice Baldeo
Jesse Onyenekwe
Roshan Lala
Cristian Landa
Anwer Siddiqi
author_facet Robert Ali
Candice Baldeo
Jesse Onyenekwe
Roshan Lala
Cristian Landa
Anwer Siddiqi
author_sort Robert Ali
collection DOAJ
description Granulomatosis with polyangiitis (GPA), previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.
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institution Kabale University
issn 2090-6889
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publishDate 2015-01-01
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series Case Reports in Rheumatology
spelling doaj-art-765c7ea885c14f11a4acdeb5da258a862025-02-03T01:32:43ZengWileyCase Reports in Rheumatology2090-68892090-68972015-01-01201510.1155/2015/513602513602Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with PolyangiitisRobert Ali0Candice Baldeo1Jesse Onyenekwe2Roshan Lala3Cristian Landa4Anwer Siddiqi5Department of Internal Medicine, University of Florida, Jacksonville, USADepartment of Internal Medicine, University of Florida, Jacksonville, USADepartment of Internal Medicine, University of Florida, Jacksonville, USADepartment of Internal Medicine, University of Florida, Jacksonville, USADepartment of Internal Medicine, University of Florida, Jacksonville, USADepartment of Pathology, University of Florida, Jacksonville, USAGranulomatosis with polyangiitis (GPA), previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.http://dx.doi.org/10.1155/2015/513602
spellingShingle Robert Ali
Candice Baldeo
Jesse Onyenekwe
Roshan Lala
Cristian Landa
Anwer Siddiqi
Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis
Case Reports in Rheumatology
title Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis
title_full Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis
title_fullStr Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis
title_full_unstemmed Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis
title_short Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis
title_sort atypical endobronchial carcinoid with postobstructive pneumonia obscuring the diagnosis of granulomatosis with polyangiitis
url http://dx.doi.org/10.1155/2015/513602
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