INTRODUCTION: Wegener's Granulomatosis (WG) is a disorder characterized by granulomatous inflammation and necrotizing vasculitis in various organs while the limited form is typically confined to the lower respiratory tract. Patients with Wegener's Granulomatosis usually present with multiple bilateral nodular infiltrates on imaging, C-ANCA positivity and other systemic involvement. We report a case of Limited Wegener's Granulomatosis in a patient who presented with a solitary cavitary lung mass and a positive Perinuclear Anti-Neutrophil Cytoplasmic Antibody (P-ANCA) titer.
CASE PRESENTATION: A 61-year-old male with a fifty-pack year history of smoking presented to his primary care physician with complaints of a productive cough, right-sided pleuritic chest pain and intermittent episodes of hemoptysis. The hemoptysis had subsided after the primary care physician prescribed him a course of antibiotics but the cough and chest pain persisted prompting the patient to visit the emergency room. The patient's physical examination and laboratory data, including kidney function and urinalysis, were unremarkable. A Chest Roentgenogram showed a right lower lobe opacity. Computed Tomography (CT) revealed a right lower lobe cavitary mass. Positron Emission Tomography (PET) measured the Standard Uptake Value (SUV) of the mass to be 3.3. A bronchoscopy was performed and the cultures were negative for bacterial infection while the cytology did not detect any malignant cells. The autoimmune work-up was entirely negative aside from a P-ANCA titer of 1:80. The patient was referred to cardiothoracic surgery after pulmonary function tests revealed the patient had adequate lung function. A right middle lobe and right lower lobe lobectomy was performed. Histologic examination revealed pulmonary abscess surrounded by pallisading granulomatous inflammation and chronic vasculitis consistent with Wegener's Granulomatosis. The patient was discharged with follow-up in the pulmonary and ear, nose and throat clinics as an outpatient. He remains asymptomatic.
DISCUSSION: Limited Wegener's Granulomatosis was first described by Carrington and Liebow in sixteen patients with pulmonary lesions histologically identical to those seen in Wegener's Granulomatosis who did not go on to develop renal disease. Limited Wegener's Granulomatosis tends to have a more favorable prognosis compared to the systemic form but patients do need to be monitored for systemic complications. Most patients with Wegener's Granulomatosis present with bilateral, multiple nodules on imaging and while many of these nodules do cavitate, the presentation of a solitary cavitary mass is rare. Moreover, anti-neutrophil cytoplasmic antibodies with cytoplasmic staining pattern (C-ANCA) have been found to have a higher degree of sensitivity and specificity in Wegener's Granulomatosis. The sensitivity and specificity of C-ANCA in active Wegener's Granulomatosis has been reported to be as high as 91% and 99%, respectively. These patients usually have a multisystem illness that affects the upper and lower respiratory tracts and the kidneys. On the other hand, patients with Wegener's Granulomatosis, who are P-ANCA positive, have fewer organ involvement. In fact, P-ANCA is found in fewer than 5% of patients with Wegener's Granulomatosis.
CONCLUSIONS: This case emphasizes that Wegener's Granulomatosis must be considered when assessing solitary cavitary masses on chest radiography. Furthermore, noninvasive tests such as anti-neutrophil cytoplasmic antibody titer should be more frequently utilized in evaluating cavitary masses as a manifestation of Wegener's Granulomatosis. While Wegener's Granulomatosis is a pathological diagnosis, the clinical suspicion must be high when a patient presents with a cavitary mass and a positive anti-neutrophil cytoplasmic antibody (ANCA) titer, as this case illustrates.
Reference #1 Rao JK, Weinberger M, Oddone EZ et al. The role of antineutrophil cytoplasmic antibody testing in the diagnosis of Wegener's Granulomatosis. A literature review and meta-analysis. Ann Intern Med 1995; 123 (12): 925-32.
Reference #2 Schonermarck U, Lamprecht P, Csernok E, Gross WL. Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies and myeloperoxidases-ANCA. Rheumatology 2001; 40; 178-84.
DISCLOSURE: The following authors have nothing to disclose: Rupesh Vakil, Walter Strauss, Frank Italiano
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