SESSION TYPE: Airway Student/Resident Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Granulomatosis with polyangiitis (GPA or Wegener’s) is a form of antineutrophil cytoplasmic antibody (ANCA) - associated vasculitis. It is far more common in older adults with racial predominance among the white population. We report the case of a 29-year-old African American male diagnosed with GPA following recurrent hemoptysis and treatment for recurrent pneumonia.
CASE PRESENTATION: A 29-year-old African American male with no past medical history presented with a 2 month history of several bouts of cough, occasionally productive of yellowish sputum, hemoptysis, and nasal congestion. He also reported subjective fevers, dyspnea on exertion, fatigue and a 23 pound unintentional weight loss. The patient was tachypneic (RR: 24), tachycardic (HR: 122) and hypertensive (BP: 184/73) on initial evaluation. His laboratory studies revealed leukocytosis (WBC: 17,200) with a left shift (Neutrophil count: 91%), anemia (Hb/Hct: 7.2/25.5), thrombocytosis (Platelet count: 899,000) and normal renal indices with trace blood on urinalysis. The patient had been treated symptomatically on several occasions for respiratory tract infection without resolution. Chest radiography revealed diffuse cavitary lesions with air-fluid levels. The patient was admitted to a negative pressure room and initiated on broad-spectrum antibiotics with concern for tuberculosis. Tuberculosis was ruled out; however, his C-ANCA (PR-3) test came back positive (59.96 EU/mL, normal is < 4), suggestive of vasculitis. The patient underwent intranasal biopsy which revealed acute necrotizing inflammation with vasculitis diagnostic of GPA. Induction therapy with intravenous methylprednisone and cyclophosphamide was initiated. The patient improved symptomatically and the hemoptysis resolved. However, after discharge he had a relapse of hemoptysis requiring hospitalization and an increase in cyclophosphamide dosing.
DISCUSSION: This case illustrates the uncommon occurrence of GPA in a young African American adult. Symptoms of GPA overlap a variety of cardiopulmonary diseases including; pneumonia, tuberculosis, heart failure and fungal infections. Cavitary lung lesions are a known feature of this disease and should prompt clinicians to include GPA in their differential diagnosis. Screening tests such as the ANCAs are valuable but a biopsy showing vasculitis is the gold standard for detection. Treatment is most effective when given early and with a combination of immunosuppressive agents.
CONCLUSIONS: The evaluation of patients with any of the following symptoms: recurrent respiratory tract infections, hemoptysis, and cavitary lung lesions has a broad differential diagnosis that must include ANCA associated vasculitis. Once recognized, this disease is treatable though outcomes and levels of success do vary.
1) Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116:488.
DISCLOSURE: The following authors have nothing to disclose: Amaka Ezimora, Oluwafisayo Adebiyi, Taopheeq Mustapha, Jason Martin
No Product/Research Disclosure InformationMeharry Medical College, Nashville, TN