Wegener’s granulomatosis (WG) is characterized by small vessel vasculitis that affects the upper respiratory tract, lungs and kidneys. Renal involvement may occur between 40 and 100% of cases. The disease varies from asymptomatic to fulminant rapidly progressive glomerulonephritis forms, leading to terminal renal failure in days.
We present a case of a patient diagnosed with WG in the Hospital de San José in Bogotá, Colombia, and a literature review focused on renal involvement. Literature search was conducted in the following databases: PubMEed, LILAC, SciELO and Cochrane. Articles in english and spanish were selected and no restriction date was made, but at least 70% of selected references were published after 2010.
Renal involvement in patients with WG is common and can be lethal. A high percent may even require renal replacement therapy, although patients can return to normal renal function. Factors associated with return to normal renal function are unclear, but it depends on early therapy and high doses of immunomodulators. Rituximab has shown greater effectiveness and fewer side effects than conventional treatments, so it’s a valid therapeutic option for the management of patients with WG and renal involvement.
2. Iannella G, Greco A, Granata G, Manno A, Pasquariello B, Angeletti D, et al. Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis. Autoimmun Rev. 2016 Jul;15(7):621-31.
3. Liu X, Cui Y, Li Y, Wang C, Zhao H, Han J. Using inpatient data to estimate the prevalence of Wegener’s granulomatosis in China. Intractable Rare Dis Res. 2016 Feb;5(1):31-5.
4. Wallace ZS, Lu N, Unizony S, Stone JH, Choi HK. Improved survival in granulomatosis with polyangiitis:A general population-based study. Semin Arthritis Rheum. 2016 Feb;45(4):483-9.
5. Romeu M, Couchoud C, Delarozière JC, Burtey S, Chiche L, Harlé JR, et al. Survival of patients with ANCA-associated vasculitis on chronic dialysis: data from the French REIN registry from 2002 to 2011. QJM. 2014 Jul;107(7):545-55.
6. Holle JU, Gross WL. Treatment of ANCA-associated vasculitis (AAV). Autoimmun Rev. 2013Feb;12(4):483-6.
7. Kronbichler A, Kerschbaum J, Mayer G. The Influence and Role of Microbial Factors in Autoimmune Kidney Diseases: A Systematic Review. J Immunol Res. 2015;ID:858027.
8. Guidelli GM, Tenti S, Pascarelli NA, Galeazzi M, Fioravanti A. Granulomatosis with polyangiitis and intravenous immunoglobulins: a case series and review of the literature. Autoimmun Rev. 2015 Aug;14(8):659-64.
9. Wojciechowska J, Krajewski W, Krajewski P, Kr?cicki T. Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge. Clin Exp Otorhinolaryngol. 2016 Mar;9(1):8-13.
10. Shafiei K, Luther E, Archie M, Gulick J, Fowler MR. Wegener granulomatosis: case report and brief literature review. J Am Board Fam Pract. 2003 Nov-Dec;16(6):555-9.
11. Lee T, Gasim A, Derebail VK, Chung Y, McGregor JG, Lionaki S, et al. Predictors of treatment outcomes in ANCA-associated vasculitis with severe kidney failure. Clin J Am Soc Nephrol. 2014May;9(5):905-13.
12. Cavoli GL, Ferrantelli A, Bono L, Tortorici C, Passantino R, Rotolo U. Kidney involvement in a wegener granulomatosis case. Indian J. Med Sci. 2012 Sep-Oct;66(9-10):238-40.
13. Sada K-e, Yamamura M, Harigai M, Fujii T, Takasaki Y, Amano K, et al. Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study. Arthritis Res Ther. 2015;17(1):1-12.
14. Latimer N, Carroll C, Wong R, Tappenden P, Venning M, Luqmani R. Rituximab in Combination with Corticosteroids for the Treatment of Anti Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A NICE Single Technology Appraisal. Pharmacoeconomics. 2014 Dec;32(12):1171-83.
15. Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010 Jul;363(3):211-20.
16. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010; Jul;363(3):221-32.
17. Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, Cohen P, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014 Nov;371(19):1771-80.
18. Vázquez V, Fayad A, González G, Quevedo AS, Sindín JR. Vasculitis asociada a ANCA con compromiso renal. Guía de Práctica Clínica. Medicina (Buenos Aires). 2015;75(Suplemento I):1-38.
19. Fortin PM, Tejani AM, Bassett K, Musini VM. Intravenous immunoglobulin as adjuvant therapy for Wegener’s granulomatosis. Cochrane Database Syst Rev. 2013 Jul;(3): CD007057.
20. Schwartz J, Winters JL, Padmanabhan A, Balogun RA, Delaney M, Linenberger ML y col. 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. 2013 Jul;28(3):145–284.
21. Malhotra S, Dhawan HK, Sharma RR, Marwaha N, Sharma A. Successful Management of Refractory Dialysis Independent Wegener’s Granulomatosis with Combination of Therapeutic Plasma Exchange and Rituximab. Indian J Hematol Blood Transfus. 2016 Jun;32(Suppl 1):332-4.
22. Tarabishy AB, Schulte M, Papaliodis GN, Hoffman GS. Wegener’s granulomatosis: Clinical manifestations, differential diagnosis, and management of ocular and systemic disease. Surv Ophthalmol. 2010; 55:429-44.