Response to Rituximab after Failure of Cyclophosphamide in the Induction Treatment in a Patient with cANCA-associated Vasculitis and Pachymeningitis: a Case Report
https://doi.org/10.14412/1996-7012-2021-4-68-71
Abstract
A 36-year-old male patient who originally presented with recurrent inflammations in the mastoid, otitis media and peripheral facial paralysis was diagnosed with sterile pachymeningitis, associated with high titres of antineutrophil cytoplasmic antibodies (cANCA) directed against proteinase 3 (PR3). Induction therapy with oral prednisolone 1 mg/kg body weight and cyclophosphamide (CYC) 750 mg/m 2 i.v. every 4 weeks was initiated. Due to side effects, increasing arthralgias and progressive meningitis after 5 doses of CYC, treatment was changed to rituximab (RTX), one cycle comprising two administrations of 1000 mg RTX i.v. two weeks apart, repeated every 6 months. After the very first cycle of RTX, we confirmed subjective improvement of the patient’s fitness as well as radiologic response. Methotrexate (MTX) was added to ease arthralgias. Painful bleeding ulcerations on finger tips were successfully treated with 22 iloprost infusions. Up to date, we have administered 7 cycles of RTX and achieved complete remission of the cANCA-associated vasculitis. After the induction therapy, maintenance treatment with MTX or rituximab will be performed for at least 18–24 months.
We can demonstrate a complete remission with the use of RTX in a patient with cANCA-associated vasculitis and sterile pachymeningitis who failed to respond to the induction treatment with CYC. RTX has been well tolerated.
About the Authors
O. Psenak
Department of Internal Medicine III with Haematology, Medical Oncology, Haemostaseology, Infectiology and Rheumatology, Oncologic Centre; Salzburg Cancer Research Institute – Laboratory for Immunological and Molecular Cancer Research (SCRI-LIMCR), Paracelsus Medical University; Cancer Cluster Salzburg
Austria
Austria
Oskar Psenak, MD, PhD
Salzburger Straβe 520, 5084 Groβgmain
R. Greil
Department of Internal Medicine III with Haematology, Medical Oncology, Haemostaseology, Infectiology and Rheumatology, Oncologic Centre; Salzburg Cancer Research Institute – Laboratory for Immunological and Molecular Cancer Research (SCRI-LIMCR), Paracelsus Medical University; Cancer Cluster Salzburg
Austria
Austria
MD, Prof.
Salzburger Straβe 520, 5084 Groβgmain
References
1. Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016 Jun;29(2):151-59. doi:10.1177/0394632015617063.
2. Tervaert JWC. Trimethoprim-sulfamethoxazole and antineutrophil cytoplasmic antibodies-associated vasculitis. Curr Opin Rheumatol. 2018 Jul;30(4):388-94. doi:10.1097/BOR.0000000000000508.
3. Villa-Forte A, Clark TM, Gomes M, et al. Substitution of methotrexate for cyclophosphamide in Wegener granulomatosis: a 12-year single-practice experience. Medicine (Baltimore). 2007 Sep;86(5):269-77. doi:10.1097/MD.0b013e3181568ec0.
4. Harper L, Morgan MD, Walsh M, et al. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012 Jun;71(6):955-60. doi:10.1136/annrheumdis-2011-200477.
5. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010 Jul 15;363(3):221-32. doi:10.1056/NEJMoa0909905.
6. Aydin Z, Gursu M, Karadag S, et al. Role of plasmapheresis performed in hemodialysis units for the treatment of anti-neutrophilic cytoplasmic antibody-associated systemic vasculitides. Ther Apher Dial. 2011 Oct; 15(5):493-98. doi:10.1111/j.1744-9987.2011.00960.x.
7. Oiwa H, Yamasaki S, Endo K, et al. Experience with seven Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis treated with remissioninduction therapy with intravenous cyclophosphamide according to the CYCLOPS protocol. Intern Med. 2014; 53(20):2291-96. doi:10.2169/internalmedicine.53.2553.
8. Kronbichler A, Jayne DRW, Mayer G. Frequency, risk factors and prophylaxis of infection in ANCA-associated vasculitis. Eur J Clin Invest. 2015 Mar;45(3):346-68. doi:10.1111/eci.12410.
9. Just SA, Knudsen JB, Nielsen MK, Junker P. Wegener's granulomatosis presenting with pachymeningitis: clinical and imaging remission by rituximab. ISRN Rheumatol. 2011;2011: 608942. doi:10.5402/2011/608942.
10. Puechal X, Pagnoux C, Perrodeau E, et al. Long-Term Outcomes Among Participants in the WEGENT Trial of Remission-Maintenance Therapy for Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis. Arthritis Rheumatol. 2016 Mar; 68(3):690-701. doi:10.1002/art.39450.
11. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014 Nov 6;371(19):1771-80. doi:10.1056/NEJMoa1404231.
12. de Joode AAE, Sanders JSF, Puechal X, et al. Long term azathioprine maintenance therapy in ANCA-associated vasculitis: combined results of long-term follow-up data. Rheumatology (Oxford). 2017 Nov 1;56(11): 1894-1901. doi:10.1093/rheumatology/kex281.
13. Qaisar H, Shenouda M, Shariff M, et al. Granulomatosis with Polyangiitis Manifesting as Refractory Otitis Media and Mastoiditis. Arch Iran Med. 2019 Jul 1;22(7):410-13.
14. Koldingsnes W, Nossent JC. Baseline features and initial treatment as predictors of remission and relapse in Wegener's granulomatosis. J Rheumatol. 2003 Jan;30(1):80-8.
15. de Groot K, Adu D, Savage CO; EUVAS (European vasculitis study group). The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant. 2001 Oct; 16(10):2018-27. doi:10.1093/ndt/16.10.2018.
16. Partsch H. Supplementary instrumental examinations in the most frequently occurring peripheral vascular diseases in general practice. Hautarzt. 1985 Apr;36(4):203-11.
17. Foti R, Visalli E, Amato G, et al. Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen. Rheumatol Int. 2017 Feb; 37(2):245-49. doi:10.1007/s00296016-3582-4.
18. Yusof MY, Vital EM, Das S, et al. Repeat cycles of rituximab on clinical relapse in ANCA-associated vasculitis: identifying B cell biomarkers for relapse to guide retreatment decisions. Ann Rheum Dis. 2015 Sep; 74(9):1734-38. doi:10.1136/annrheumdis-2014-206496.
19. Bramsiepe I, Danz B, Heine R, et al. Primary cutaneous manifestation of Wegener's granulomatosis. Dtsch Med Wochenschr. 2008 Jul;133(27):1429-32. doi:10.1055/s-2008-1081089.
20. Schmidt WA, Both M, Reinhold-Keller E. Imaging procedures in rheumatology: imaging in vasculitis. Z Rheumatol. 2006 Nov;65(7):652-56, 658-61. doi:10.1007/s00393-006-0107-7.
Review
For citations:
Psenak O, Greil R. Response to Rituximab after Failure of Cyclophosphamide in the Induction Treatment in a Patient with cANCA-associated Vasculitis and Pachymeningitis: a Case Report. Sovremennaya Revmatologiya=Modern Rheumatology Journal. 2021;15(4):68-71. https://doi.org/10.14412/1996-7012-2021-4-68-71
Views:
446
Email this article 