SESSION TITLE: Miscellaneous Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Wednesday, October 28, 2015 at 11:00 AM - 12:15 PM
INTRODUCTION: Granulomatosis with polyangiitis (GPA) can present with variety of symptoms. Respiratory tract involvement is common and can have variety of presentations including involvement of tracheobronchial tree. GPA involvement of tracheobronchial tree most commonly presents with tracheal stenosis. We present a case of a 22 years old female with treated GPA who presented with 10 day history of shortness of breath and wheezing. After extensive evaluation including inpatient pulmonary function testing(PFT) she was found to have tracheal stenosis.
CASE PRESENTATION: A 22 yo female with GPA presented with 10 day history of worsening shortness of breath (SOB) and wheezing. Her treatment included a recent course of Rituximab and prednisone taper which she was taking at presentation. Physical examination was remarkable for few upper lung zone wheezes, but no stridor. CBC was remarkable for mild anemia with hemoglobin of 12.0 g/dl and leukopenia with white blood cell count of 3.8 thou/cu mm. Chest xray and CT were normal without any evidence of parenchymal or tracheobronchial abnormality. Laryngoscopy done was normal without any supraglottic mucosal abnormality. Given the disproportionate complaints of SOB to exam and normal imaging findings, pulmonary function testing (PFT) was done. PFT was consistent with fixed upper airway obstruction with flattening of the inspiratory and expiratory limb of flow volume loop. Based on this finding, a bronchoscopy was done which revealed inflamed mucosa with subglottic tracheal stenosis with 50% narrowing of the airway. A balloon dilation was done. Patient had resolution of SOB and wheezing post bronchoscopy and follow up PFT revealed normal values with normalization of the flow volume loop.
DISCUSSION: Granulomatosis with polyangiitis can have variety of respiratory presentations including involvement of the tracheobronchial tree which most commonly presents with tracheal stenosis. This case demonstrates a diagnosis of tracheal stenosis made with inpatient PFT testing after extensive work up revealed no cause for the patient’s presentation of SOB and wheezing.
CONCLUSIONS: In patients with GPA presenting with SOB when routine test and imaging dose not reveal a cause for the presentation, before a more invasive testing or alternate work up is pursued, physicians should consider PFT testing.
Reference #1: King, TE. Respiratory tract involvement in granulomatosis with polyangiitis (Wegener's) In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on March 31, 2015)
DISCLOSURE: The following authors have nothing to disclose: Abbas Shahmohammadi, Wael Nasser, Johan Barretto
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