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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Format: | Article |
Language: | English |
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Wiley
2015-01-01
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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. |
format | Article |
id | doaj-art-765c7ea885c14f11a4acdeb5da258a86 |
institution | Kabale University |
issn | 2090-6889 2090-6897 |
language | English |
publishDate | 2015-01-01 |
publisher | Wiley |
record_format | Article |
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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