A Fatal Case of Treatment-Related Adverse Effects in Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)
Marina Magrey, Maria Antonelli
.
DOI: 10.4236/ojra.2011.12002   PDF    HTML   XML   8,553 Downloads   14,620 Views   Citations

Abstract

Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.

Share and Cite:

M. Magrey and M. Antonelli, "A Fatal Case of Treatment-Related Adverse Effects in Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)," Open Journal of Rheumatology and Autoimmune Diseases, Vol. 1 No. 2, 2011, pp. 5-9. doi: 10.4236/ojra.2011.12002.

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1] B. V. Maramattom, C. Giannini, E. M. Manno and E. F. Wijdicks, “Wegener’s,” 2005. http://www.ncibi.nlm.nih.gov/pubmed/15980626granulo-matosis http://www.ncibi.nlm.nih.gov/pubmed/15980626
[2] H. A. Rula, R. Silverstein, G. Hoffman, L. Zhang, P. Imrey and C. Langford, “The Correlation between Circulating Microparticles and Platelet Aggregation and Disease Activity in Wegener Granulomatosis,” Arthritis and Rheumatism, Vol. 62, Supplement 10, 2010, p. S856.
[3] M. DeBandt, V. Ollivier, O. Meyer, C. Babin-Chevaye, F. Khechai, D. de Prost, J. Hakim and C. Pasquier, “Induction of Interleukin-1 and Subsequent Tissue Factor Expression by Anti-Proteinase 3 Antibodies in Human Umbilical Vein Endothelial Cells,” Arthritis Rheum, Vol. 40, No. 11, 1997, pp. 2030-2038. doi:10.1002/art.1780401116
[4] P. A. Merkel, G. H. Lo, J. T. Holbrook, et al., “Brief Communication: High Incidence of Venous Thrombotic Events among Patients with Wegener Granulomatosis: The Wegener’s Clinical Occurrence of Thrombosis (WeCLOT) Study,” Annals of Internal Medicine, Vol. 142, No. 8, 2005, pp. 620-626.
[5] G. S. Hoffman, G. S. Kerr, R. Y. Leavitt, C. W. Hallahan, R. S. Lebovics, W. D. Travis, et al., “Wegener Granulomatosis: An Analysis of 158 Patients,” Annals of Internal Medicine, Vol. 116, No. 6, 1992, pp. 488-498
[6] R. Seror, A. Mahr, J. Ramanoelina, C. Pagnoux, P. Cohen and L. Guillevin, “Central Nervous System Involvement in Wegener’s Granulomatosis,” Medicine, Vol. 85, No. 1, 2006, pp. 54-65.
[7] H. Nishino, F. A. Rubino and J. E. Parisi, “The Spectrum of Neurologic Involvement in Wegener’s Granuloma- tosis,” Neurology, Vol. 43, No. 7, 1993, pp. 1334-1337.
[8] W. Zhang, G. Zhou, Q. Shi, X. Zhang, X. F. Zeng and F. C. Zhang, “Clinical Analysis of Nervous System Involvement in ANCA-Associated Systemic Vasculitides,” Clinical and Experimental Rheumatology, Vol. 27, Supplement 52, 2009, pp. S65-S69.
[9] J. U. Holle and W. L Gross, “Neurological Involvement in Wegener’s Granulomatosis,” Current Opinion Rheu- matology, Vol. 23, No. 1, 2011, pp. 7-11. doi:10.1097/BOR.0b013e32834115f9
[10] J. M. Provenzale and N. B. Allen, “Wegener Granulomatosis: CT and MR Findings,” American Journal of Neuroradiology, Vol. 17, 1996, pp. 785-792.
[11] B. A. Cunha, “Nervous System Infections in the Com- promised Host: A Diagnostic Approach,” Infectious Di- sease Clinics of North America Central, Vol. 15, No. 1, 2001, pp. 567-590.
[12] J. U. Holle, W. L. Gross, U. Latza, B. Noelle, P. Ambrosch, M. Heller, R. Fertmann and E. Reinhold-Keller, “Improved Outcome of 445 Wegener’s Granulomatosis Patients in a German Vasculitis Center over Four Decades,” Arthritis & Rheumatism, Vol. 63, No. 1, 2010, pp. 257-266.
[13] R. Luqmani, R. Suppiah, C. J. Edwards, R. Phillip, J. Maskell, D. Culliford, D. Jayne, K. Morishita and N. Arden, “Mortality in Wegener’s Granulomatosis: A Bimodal Pattern,” Rheumatology, Vol. 50, No. 4, 2010, pp. 597-702.
[14] G. S. Hoffman, Y. Drucker, M. F. Cotch, et al., “Wege- ner’s Granulomatosis: Patient-Reported Effects of Disease on Health, Function, and Income,” Arthritis & Rheumatism, Vol. 41, No. 12, 1998, pp. 2257-2262. doi:10.1002/1529-0131(199812)41:12<2257::AID-ART22>3.0.CO;2-K
[15] E. Reinhold-Keller, N. Beuge, U. Latza, et al., “An Inter- disciplinary Approach to the Care of Patients with Wegener’s Granulomatosis: Long-Term Outcome in 155 Patients,” Arthritis & Rheumatism, Vol. 43, No. 5, 2000, pp. 1021-1032. doi:10.1002/1529-0131(200005)43:5<1021::AID-ANR10>3.0.CO;2-J
[16] K. De Groot, L. Harper, D. R. Jayne, L. F. Flores Suarez, G. Gregorinin, W. L. Gross, et al., “Pulse versus Daily Oral Cyclophopshamide for Induction of Remission in Antineu-Trophil Cytoplasmic Antibody-Associated Vasculitis: A Randomized Trial,” Annals of Internal Medi- cine, Vol. 150, 2009, pp. 670-680.
[17] A. D. Booth, M. K. Almond, A. Burns, et al., “Pan- Thames Renal Research Group. Outcome of ANCA- Associated Renal Vasculitis: A Five-Year Retrospective Study,” American Journal of Kidney Diseases, Vol. 41, No. 4, 2003, pp. 776-784. doi:10.1016/S0272-6386(03)00025-8
[18] C. Charlier, C. Henegar, O. Launay, C. Pagnoux, A. Berezné, B. Bienvenu, P. Cohen, L. Mouthon and L. Guillevin, “Risk Factors for Major Infections in Wegener Granulomatosis: Analysis of 113 Patients,” Annals of the Rheumatic Disease, Vol. 68, No. 5, 2009, pp. 658-663. doi:10.1136/ard.2008.088302
[19] M. Little, P. Nightingale, C. A. Verburgh, et al., “Early Mortality in Systemic Vasculitis: Relative Contribution of Adverse Events and Active Vasculitis,” Annals of the Rheumatic Disease, Vol. 69, No. 6, 2010, pp. 1036-1043. doi:10.1136/ard.2009.109389
[20] A. Ibrahim, B. Spellberg and J. Edwards, “Iron Acqui- sition: A Novel Prospective on Mucormycosis Patho- genesis and Treatment,” Current Opinion in Infectious Disease, Vol. 21, No. 6, 2008, pp. 620-625. doi:10.1097/QCO.0b013e3283165fd1
[21] W.-F. Li, H. E. Chao, X.-F. Liu, S.-Y. Wang, J.-L. Qu and Z.-F. Lin, “A Diagnosis Neglected for 6 Years: Report of a Misdiagnosed Case of Pulmonary Mucor- mycosis and Review of the Literature,” Chinese Medical Journal, Vol. 123, No. 17, 2010, pp. 2480-2482
[22] M. F. Liu, F. F. Chen, T. R. Hsiue and C. C. Liu, “Disseminated Zygomycosis Simulating Cerebrovascular Disease and Pulmonary Alveolar Haemorrhage in a Pa- tient with Systemic Lupus Erythematosus,” Clinical Rheu- matology, Vol. 19, No. 4, 2000, pp. 311-314. doi:10.1007/s100670070052
[23] E. Nogueira, P. W. Ind, J. Friedland and A. D. Salama, “Mucormycosis May Mimic Disease Relapse in Wege- ner’s Granulomatosis,” The Journal of Rheumatology, Vol. 37, No. 6, 2010, pp. 1364-1365. doi:10.3899/jrheum.091423
[24] J. D. Finkielman, P. A. Merkel, D. Schroeder, et al., “Antiproteinase 3 Antineutrophil Cytoplasmic Antibodies and Disease Activity in Wegener Granulomatosis,” Annals of Internal Medicine, Vol. 147, No. 9, 2007, pp. 611-619.
[25] R. J. Falk and G. S. Hoffman, “Controversies in Small Vessel Vasculitis,” Current Opinion in Rheumatology, Vol. 19, No. 1, 2007, pp. 1-9.
[26] H. P. McAdams, M. R. de Christenson, D. C. Strollo and E. F. Patz Jr., “Pulmonary Mucormycosis: Radiologic Findings in 32 Cases,” American Journal of Neurora- diology, Vol. 168, No. 6, 1997, pp. 1541-1548.
[27] J. H. Stone, P. A. Merkel, R. Spiera, P. Seo, C. A. Langford, G. S. Hoffman, U. Specks, et al., “RAVE-ITN Research Group,” The New England Journal of Medicine, Vol. 5, No. 363, 2010, pp. 221-232. doi:10.1056/NEJMoa0909905

Copyright © 2024 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.