SESSION TITLE: Critical Care Student/Resident Case Report Posters IV
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Granulomatosis with polyangiitis (GP), formerly known as Wegener’s granulomatosis, is a granulomatous vasculitis affecting the respiratory tract and kidneys. Staphylococcus aureus (SA) colonization is a risk factor for GP relapse but cases presenting with staphylococcal sepsis and pneumonia have not been reported. Here we describe a case of limited form of GP diagnosed after presenting with MRSA bacteremia and pneumonia.
CASE PRESENTATION: A 47 year-old-man presented with fever, respiratory distress and skin rash. He had recently received trimethoprim-sulphamethoxazole for a skin infection. Examination revealed extensive desquamating rash, hypotension, bilateral rales and mild peripheral edema. In the ICU, he was treated with fluid resuscitation, vasopressors, IV antibiotics and ventilatory support. Chest X-ray showed nodular lung infiltrates (Image 1). Chest CT showed bilateral nodular opacities with cavitation (Image 2). Blood and sputum cultures grew MRSA; skin biopsy was suggestive of hypersensitivity drug reaction. Despite appropriate therapy, patient’s respiratory failure and lung lesions worsened. He had a CRP of 165 and serologies including c-ANCA were negative. Lung biopsy showed necrotising pulmonary vasculitis suggestive of GP for which pulse dose steroid therapy was started. Cyclophosphamide therapy was held due to severe sepsis. Patient improved dramatically after pulse steroid therapy, was extubated and subsequently discharged on oral steroid therapy, antibiotics to complete the course and follow-up with Rheumatology.
DISCUSSION: GP is diagnosed with tissue biopsy showing necrotizing vasculitis with granulomas. It can be ANCA negative in 20% cases. Bronchoalveolar lavage fluid in GP patients is trophic for SA growth, nasal carriage of SA has been associated with increased relapses of GP and treatment of SA with cotrimoxazole has been shown to achieve partial or complete resolution of GP in some studies. But its role in the pathogenesis of GP remains unclear. Increased expression of proinflammatory cytokines IL6, IL1 and IL8 due to SA infection have been postulated to be responsible for the relapses. Our case shows that GP can present with SA pneumonia and sepsis though the cause and effect relationship needs to be answered.
CONCLUSIONS: Patients with multifocal pneumonia not responding to adequate treatment and worsening radiological findings should be evaluated for vasculitis. Further studies are needed to determine the role of SA in the pathogenesis of GP.
Reference #1: Langford C et al. The Vasculitis Syndromes; Harrison’s Principles of Internal Medicine, 18th Edition.
Reference #2: Stegeman CA t al: Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener’s granulomatos. Ann Intern Med 1994; 120: 12-17.
Reference #3: Yao L et al: Interleukin-8 gene expression in Staphylococcus aureus-infected endothelial cells. Infect Immun. 1996 Aug;64(8):3407-9.
DISCLOSURE: The following authors have nothing to disclose: Rahul Mutneja, Mamta Shah, D. Datta
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