Abstract
Wegeners granulomatosis (WG) is an autoimmune, antineutrophil cytoplasmic antibody mediated necrotizing vasculitis involving renal, and upper and lower respiratory systems. Treatment relies on a combination of immunosuppressive drugs and tapering regimen of glucocorticoids. Therapeutic plasma exchange (TPE) has been recognized as a second line treatment. We report the successful use of TPE in combination with rituximab in achieving remission in a patient with WG (dialysis independent) not responding to conventional therapy.
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References
Deyer G, Fan S (2009) Therapeutic implications of coexisting severe pulmonary hemorrhage and pulmonary emboli in a case of Wegener granulomatosis. Am J Kidney Dis 53:5–8
Reinhold-Keller E (2012) Diagnostics and therapy of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides. Curr Pharm Des 18:4537–4541
Antoniu SA (2007) Treatment options for refractory Wegener’s granulomatosis: a role for rituximab? Curr Opin Investig Drugs 8:927–932
Lee RW, D’Cruz DP (2008) Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis. Drugs 68:747–770
Nguyen T, Martin MK, Indrikovs AJ (2005) Plasmapheresis for diffuse alveolar hemorrhage in a patient with Wegener’s granulomatosis: case report and review of the literature. J Clin Apher 20:230–234
Schwartz J, Winters JL, Padmanabhan A, Balogun RA, Delaney M, Linenberger ML et al (2013) Guidelines on the use of therapeutic apheresis in clinical practice—evidence-based approach from the writing committee of the american society for apheresis: the sixth special issue. J Clin Apher 28:145–284
Klein I, Vervoort G, Steenbergen E, Wetzels J (2008) Progressive renal disease despite immunosuppressive therapy in a patient with Wegener’s granulomatosis. J Med 66:125–127
Aydin Z, Gursu M, Karadag S, Uzun S, Tatli E, Sumnu A et al (2011) Role of plasmapheresis performed in hemodialysis units for the treatment of anti-neutrophilic cytoplasmic antibody-associated systemic vasculitides. Ther Apher Dialy 15:493–498
Sugimoto T, Deji N, Kume S, Osawa N, Sakaguchi M, Isshiki K et al (2007) Pulmonary-renal syndrome, diffuse pulmonary hemorrhage and glomerulonephritis, associated with Wegener’s granulomatosis effectively treated with early plasma exchange therapy. Int Med 46:49–53
Jayne DRW, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L (2007) Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 18:2180–2188
Casian A, Jayne D (2011) Plasma exchange in the treatment of Wegener’s granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome and renal limited vasculitis. Curr Opin Rheumatol 23:12–17
Szpirt WM, Heaf JG, Petersen J (2011) Plasma exchange for induction and cyclosporine a for maintenance of remission in Wegener’s granulomatosis—a clinical randomized controlled trial. Nephrol Dial Transpl 26:206–213
Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. NEJM 363:221–232
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Communicated by Haraprasad Pati.
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Malhotra, S., Dhawan, H.K., Sharma, R.R. et al. Successful Management of Refractory Dialysis Independent Wegener’s Granulomatosis with Combination of Therapeutic Plasma Exchange and Rituximab. Indian J Hematol Blood Transfus 32 (Suppl 1), 332–334 (2016). https://doi.org/10.1007/s12288-015-0507-4
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DOI: https://doi.org/10.1007/s12288-015-0507-4