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

Robert Ali,Anwer Siddiqi,Candice Baldeo,Roshan Lala,Jesse Onyenekwe,Cristian Landa

doi:10.1155/2015/513602

Abstract

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.

Highlights

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, including systemic necrotizing vasculitis, necrotizing granulomatous inflammation, and necrotizing glomerulonephritis [1]
Multiple triggers have been postulated for the development of GPA in genetically susceptible individuals, with noted increased susceptibility to proteinase-3 ANCA associated vasculitis (AAV)
The inaugural lesions are thought to commence as a localized airway granulomatosis that subsequently transforms to a vasculitis [5]

Summary

Introduction

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, including systemic necrotizing vasculitis, necrotizing granulomatous inflammation, and necrotizing glomerulonephritis [1]. She had a leukocytosis of 15,000/mm and CXR showed stable bilateral pulmonary infiltrates (see Figure 2(a)) Her creatinine was elevated from a normal baseline at 1.38 mg/dL and the urinalysis was positive for 54 red blood cells/HPF. Repeat chest CT scan nearly four months later revealed a significant improvement/resolution of the diffuse opacities throughout the bilateral lungs but commented on the stable 3 cm endobronchial mass of the left lower lobe bronchus, with slightly worsened distal consolidation of the left lower lobe. She subsequently underwent snare cautery, followed by argon photocoagulation debulking of the tumor. Her death was believed to be as a result of respiratory failure secondary to extensive pulmonary hemorrhage in a background of interstitial lung disease and a clinical history of granulomatosis with polyangiitis

Findings

Discussion

Conclusion