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Rapid Clinical and Radiological Improvement of ANCA-Negative Limited Granulomatosis With Polyangiitis Induced by Rituximab: Is the B Cell Depletion? FREE TO VIEW

Lorenzo Zaffiri, MD; Shafik Boyaji, MD; Imad Bagh, MD; Mark Loehrke, MD
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MSU/KCMS, Kalamazoo, MI

Chest. 2013;144(4_MeetingAbstracts):961A. doi:10.1378/chest.1701151
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomas and vasculitis, involving upper and lower respiratory tract, lungs and kidneys. It is typically associated with anti-neutrophil cytoplasmatic antibodies (ANCA). However, a cluster of patients presents with limited disease and absence of ANCA antibodies. Despite recent studies demonstrated rapid improvement in patients with GPA, the role of rituximab in treatment of ANCA-negative disease remains uncertain. We present a characteristic case of limited GPA with negative serology for ANCA that promptly responded to treatment with corticosteroids and rituximab.

CASE PRESENTATION: A 24-years-old woman presented with severe headache, otalgia, cough and hemoptysis. She described a 4-month history of sinus congestion. Serology was negative for ANCA, rheumatoid factor and anti-nucleus antibody. CT head showed severe pan-sinus disease. CT chest revealed a thick-walled cavitary lesion in left upper lobe and multiple pulmonary nodules. During hospitalization, the patient developed seventh-nerve palsy and mononeuritis multiplex. Histopathological analysis of sinus biopsy demonstrated granuloma with extensive neutrophil infiltration and vasculitis confirming the diagnosis of GPA. Initial pulse therapy of high-dose methylprednisolone, followed by daily prednisone and weekly rituximab treatment was initiated with rapid clinical and radiological improvement.

DISCUSSION: Up to 40% of patients with GPA can present with involvement only of ear, nose, throat and lungs. Characteristically this cluster of patients is younger at disease onset, likely to be female and less likely to have ANCA antibodies in the serum. The pathogenesis of GPA is complex and still unclear. It involves a strong interaction between neutrophil intra-cytoplasmatic antigens and immune adaptive cells such as Th17 and B lymphocytes. Despite c-ANCA are strongly associated with the development of GPA, discordance between ANCA and disease activity is common. Given the involvement of B lymphocytes in the pathogenesis of the vasculitis, the efficacy of rituximab, a monoclonal anti-CD20 antibody, have been recently demonstrated. The use of rituximab could induce a reduction of auto-antibodies production, attenuation of antigen presentation and decrease inflammatory response

CONCLUSIONS: Our case shows the clinical efficacy of treatment with rituximab in a patient presenting with limited GPA and lack of ANCA antibodies.

Reference #1: Gómez-Puerta JA, Quintana LF, Stone JH, et al. . B-cell depleting agents for ANCA vasculitides: a new therapeutic approach.Autoimmun Rev. 2012;11(9):646-52.

Reference #2: Cartin-Ceba R, Golbin JM, Keogh KA, et al. Rituximab for remission induction and maintenance in refractory granulomatosis with polyangiitis (Wegener's): ten-year experience at a single center. Arthritis Rheum. 2012 ;64(11):3770-8.

Reference #3: Kallenberg CG. Pathophysiology of ANCA-associated small vessel vasculitis. Curr Rheumatol Rep. 2010;12(6):399-405

DISCLOSURE: The following authors have nothing to disclose: Lorenzo Zaffiri, Shafik Boyaji, Imad Bagh, Mark Loehrke

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