Acute Respiratory Distress Syndrome in A 32-Year-Old Man with Newly Diagnosed Wegener’s Granulomatosis C-ANCA Positive Pauci Immune Crescentic Glomerulonephritis with Leucopenia Treated Successfully: A Rare Case Report
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Abstract
A 32-year-old man presented with myalgia, polyarthritis, hearing problem, sneezing, erythematous rash, episcleritis for one month. As serum creatinine was high, renal biopsy was done; it was compatible with pauci immune glomerulonephritis. Blood c-ANCA was positive; and, both anti-glomerular basement membrane antibody and p-ANCA were negative. Therefore, he was diagnosed as a case of Wegener’s Granulomatosis with rapidly progressive glomerulonephritis, and treated with corticosteroids, cyclophosphamide and rituximab. Hemodialysis was initiated shortly after immunosuppressive treatment as serum creatinine was rising. He developed dyspnea and low SaO2 due to acute respiratory distress syndrome (ARDS). Total WBC count was low (2.1X109 /L). He was successfully treated with continuous positive airway pressure (CPAP).
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References
I. Ahmad, Y., Morawietz, G., Ksouri, H., Schefold, J. C., & Zuercher, P. (2021a). Granulomatosis with polyangiitis (Wegener’s) complicated by splenic rupture and severe acute respiratory distress syndrome: A case report. Clinical Case Reports, 9(7), e04369. https://doi.org/10.1002/ccr3.4369
II. Ahmad, Y., Morawietz, G., Ksouri, H., Schefold, J., & Zuercher, P. (2021b). Granulomatosis with polyangiitis (Wegener’s) complicated by splenic rupture and severe acute respiratory distress syndrome: A case report. Clinical Case Reports, 9. https://doi.org/10.1002/ccr3.4369
III. Ahmed, S. H., Aziz, T., Cochran, J., & Highland, K. (2004). Use of Extracorporeal Membrane Oxygenation in a Patient With Diffuse Alveolar Hemorrhage. Chest, 126(1), 305–309.
https://doi.org/10.1378/chest.126.1.305
IV. Arora, R. (2014). Massive alveolar haemorrhage: A rare life threatening complication of Wegener’s granulomatosis—Report of a rare case. 2014, 4(6), 509–511.
V. Arunprasath, P., Gobu, P., Dubashi, B., Satheesh, S., & Balachander, J. (2011). Rituximab induced myocardial infarction: A fatal drug reaction. Journal of Cancer Research and Therapeutics, 7(3). https://journals.lww.com/cancerjournal/fulltext/2011/07030/rituximab_induced_myocardial_infarction__a_fatal.20.aspx
VI. Baughman, R. P., Meyer, K. C., Nathanson, I., Angel, L., Bhorade, S. M., Chan, K. M., Culver, D., Harrod, C. G., Hayney, M. S., Highland, K. B., Limper, A. H., Patrick, H., Strange, C., & Whelan, T. (2012). Monitoring of Nonsteroidal Immunosuppressive Drugs in Patients With Lung Disease and Lung Transplant Recipients: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. CHEST, 142(5), e1S-e111S. https://doi.org/10.1378/chest.12-1044
VII. Bonek, K., Brożek-Mądry, E., Wroński, J., Płaza, M., Zielińska, A., Helon, K., Wójcik, K., & Wisłowska, M. (2023). Combination Treatment of Locoregionally Aggressive Granulomatosis with Polyangiitis and Cranial Base Infiltration. Brain Sciences, 13(8).
https://doi.org/10.3390/brainsci13081140
VIII. Boyle, A. J., Holmes, D. N., Hackett, J., Gilliland, S., McCloskey, M., O’Kane, C. M., Young, P., Di Gangi, S., & McAuley, D. F. (2021). Hyperoxaemia and hypoxaemia are associated with harm in patients with ARDS. BMC Pulmonary Medicine, 21(1), 285. https://doi.org/10.1186/s12890-021-01648-7
IX. Capellier, G., Barrot, L., & Winizewski, H. (2023). Oxygenation target in acute respiratory distress syndrome. Journal of Intensive Medicine, 3(3), 220–227. https://doi.org/10.1016/j.jointm.2023.03.002
X. Delvino, P., Monti, S., Balduzzi, S., Belliato, M., Montecucco, C., & Caporali, R. (2019). The role of extra-corporeal membrane oxygenation (ECMO) in the treatment of diffuse alveolar haemorrhage secondary to ANCA-associated vasculitis: Report of two cases and review of the literature. Rheumatology International, 39(2), 367–375.
https://doi.org/10.1007/s00296-018-4116-z
XI. Dolch, M., Irlbeck, M., Wessely, M., Rau, S., Frey, L., & Schönermarck, U. (2013). Acute respiratory distress syndrome (ARDS) as primary manifestation in ANCA-associated vasculitis. In La Presse Médicale (Vol. 42, p. 753).
https://doi.org/10.1016/j.lpm.2013.02.237
XII. ECMO Rescues Patients With Acute Respiratory Failure Related to GPA. (n.d.-a).
XIII. ECMO Rescues Patients With Acute Respiratory Failure Related to GPA. (n.d.-b).
XIV. ECMO Rescues Patients With Acute Respiratory Failure Related to GPA. (n.d.-c).
XV. Emer, J. J., & Claire, W. (2009). Rituximab: A review of dermatological applications. The Journal of Clinical and Aesthetic Dermatology, 2(5), 29–37.
XVI. Gibelin, A., Dumas, G., Valade, S., de Chambrun, M. P., Bagate, F., Neuville, M., Schneider, F., Baboi, L., Groh, M., Raphalen, J.-H., Chiche, J.-D., De Prost, N., Luyt, C.-E., Guérin, C., Maury, E., de Montmollin, E., Hertig, A., Parrot, A., Clere-Jehl, R., & Fartoukh, M. (2021a). Causes of acute respiratory failure in patients with small-vessel vasculitis admitted to intensive care units: A multicenter retrospective study. Annals of Intensive Care, 11(1), 158. https://doi.org/10.1186/s13613-021-00946-x
XVII. Gibelin, A., Dumas, G., Valade, S., de Chambrun, M. P., Bagate, F., Neuville, M., Schneider, F., Baboi, L., Groh, M., Raphalen, J.-H., Chiche, J.-D., De Prost, N., Luyt, C.-E., Guérin, C., Maury, E., de Montmollin, E., Hertig, A., Parrot, A., Clere-Jehl, R., & Fartoukh, M. (2021b). Causes of acute respiratory failure in patients with small-vessel vasculitis admitted to intensive care units: A multicenter retrospective study. Annals of Intensive Care, 11(1), 158. https://doi.org/10.1186/s13613-021-00946-x
XVIII. Granulomatosis with polyangiitis: Clinical characteristics and updates in diagnosis. (n.d.).
XIX. Grieco, D. L., Maggiore, S. M., Roca, O., Spinelli, E., Patel, B. K., Thille, A. W., Barbas, C. S. V., de Acilu, M. G., Cutuli, S. L., Bongiovanni, F., Amato, M., Frat, J.-P., Mauri, T., Kress, J. P., Mancebo, J., & Antonelli, M. (2021). Non-invasive ventilatory support and high-flow nasal oxygen as first-line treatment of acute hypoxemic respiratory failure and ARDS. Intensive Care Medicine, 47(8), 851–866. https://doi.org/10.1007/s00134-021-06459-2
XX. Jung, S. M., Jung, Y. H., Noh, H. J., Woo, I. S., & Han, C. W. (2014). A case of Wegener’s granulomatosis mimicking recurrent hemophagocytic lymphohistiocytosis. The Korean Journal of Internal Medicine, 29(3), 393–397. https://doi.org/10.3904/kjim.2014.29.3.393
XXI. Kanigicherla, D. A. K., Kehagia, A. A., Jamshidi, B., Manounah, L., Barnes, A., Patrick, H., Powell, H., Austin, C., Norton, S., Willcocks, L., Griffith, M., Braddon, F., Steenkamp, R., McKane, W. S., & Khwaja, A. (2024). Prospective study of the effect of rituximab on kidney function in membranous nephropathy. Clinical Kidney Journal, 17(8), sfae179. https://doi.org/10.1093/ckj/sfae179
XXII. Kelesidis, T., Daikos, G., Boumpas, D., & Tsiodras, S. (2011). Does rituximab increase the incidence of infectious complications? A narrative review. International Journal of Infectious Diseases, 15(1), e2–e16. https://doi.org/10.1016/j.ijid.2010.03.025
XXIII. Keogh, K. A., Ytterberg, S. R., Fervenza, F. C., Carlson, K. A., Schroeder, D. R., & Specks, U. (2006). Rituximab for refractory Wegener’s granulomatosis: Report of a prospective, open-label pilot trial. American Journal of Respiratory and Critical Care Medicine, 173(2), 180–187. https://doi.org/10.1164/rccm.200507-1144OC
XXIV. Koppurapu, V., Kakkera, K. S. S., Dunn, A., Subramany, S., Balagoni, H., & Jagana, R. (2017). A Case of Severe ARDS Due to Granulomatosis With Polyangiitis Complicated by Invasive Aspergillosis. CHEST, 152(4), A281. https://doi.org/10.1016/j.chest.2017.08.307
XXV. Lioté, H., Lioté, F., Séroussi, B., Mayaud, C., & Cadranel, J. (2010). Rituximab-induced lung disease: A systematic literature review. European Respiratory Journal, 35(3), 681. https://doi.org/10.1183/09031936.00080209
XXVI. Loscar, M., Hummel, T., Haller, M., Briegel, J., Wiebecke, B., Samtleben, W., Berger, H., Eichhorn, P., & Schelling, G. (1997). [ARDS and Wegener granulomatosis]. Der Anaesthesist, 46(11), 969–973. https://doi.org/10.1007/s001010050494
XXVII. Łosińska, K., Wilk, M., Pripp, A. H., Korkosz, M., & Haugeberg, G. (2022). Long-term drug effectiveness and survival for reference rituximab in rheumatoid arthritis patients in an ordinary outpatient clinic. Scientific Reports, 12(1), 8283. https://doi.org/10.1038/s41598-022-12271-9
XXVIII. McAtee, C. L., Lubega, J., Underbrink, K., Curry, K., Msaouel, P., Barrow, M., Muscal, E., Lotze, T., Srivaths, P., Forbes, L. R., Allen, C., & Bernhardt, M. B. (2021). Association of Rituximab Use With Adverse Events in Children, Adolescents, and Young Adults. JAMA Network Open, 4(2), e2036321–e2036321. https://doi.org/10.1001/jamanetworkopen.2020.36321
XXIX. Mirouse, A., Parrot, A., Audigier, V., Demoule, A., Mayaux, J., Géri, G., Mariotte, E., Bréchot, N., de Prost, N., Vautier, M., Neuville, M., Bigé, N., de Montmollin, E., Cacoub, P., Resche-Rigon, M., Cadranel, J., & Saadoun, D. (2020). Severe diffuse alveolar hemorrhage related to autoimmune disease: A multicenter study. Critical Care, 24(1), 231. https://doi.org/10.1186/s13054-020-02936-0
XXX. Off-label use of rituximab in patients with systemic lupus erythematosus with extrarenal disease activity: A retrospective study and literature review. (n.d.).
XXXI. Ohshimo, S. (2021a). Oxygen administration for patients with ARDS. Journal of Intensive Care, 9(1), 17. https://doi.org/10.1186/s40560-021-00532-0
XXXII. Ohshimo, S. (2021b). Oxygen administration for patients with ARDS. Journal of Intensive Care, 9(1), 17. https://doi.org/10.1186/s40560-021-00532-0
XXXIII. Park, J. A. (2021). Treatment of Diffuse Alveolar Hemorrhage: Controlling Inflammation and Obtaining Rapid and Effective Hemostasis. International Journal of Molecular Sciences, 22(2). https://doi.org/10.3390/ijms22020793
XXXIV. Powers, B., Uppalapati, A., Gogineni, S., & Jamkhana, Z. A. (2013). Rituximab-a drug with many facets and cures: A treatment for acute refractory hypoxemic respiratory failure secondary to severe granulomatosis with polyangiitis. Case Reports in Critical Care, 2013, 123134.
https://doi.org/10.1155/2013/123134
XXXV. Renner, C. (2019). 20 Years of Rituximab Treatment: What have We Learnt? Future Oncology, 15(36), 4119–4121.
https://doi.org/10.2217/fon-2019-0694
XXXVI. Sánchez-Escuredo, A., Núñez, R., Ibernón, M., Martínez, E., López, D., Navarro, M., Bonet, J., Ara, J., & Romero, R. (2011). Rituximab therapy for Wegener’s granulomatosis refractory to conventional treatment. Nefrología (English Edition), 31(4), 502–504.
https://doi.org/10.3265/Nefrologia.pre2011.May.10
XXXVII. Sinha, A., & Bagga, A. (2013). Rituximab therapy in nephrotic syndrome: Implications for patients’ management. Nature Reviews Nephrology, 9(3), 154–169. https://doi.org/10.1038/nrneph.2012.289
XXXVIII. van VOLLENHOVEN, R. F., EMERY, P., BINGHAM, C. O., KEYSTONE, E. C., FLEISCHMANN, R., FURST, D. E., MACEY, K., SWEETSER, M., KELMAN, A., & RAO, R. (2010). Longterm Safety of Patients Receiving Rituximab in Rheumatoid Arthritis Clinical Trials. The Journal of Rheumatology, 37(3), 558. https://doi.org/10.3899/jrheum.090856
XXXIX. van Vollenhoven, R. F., Emery, P., Bingham, C. O., Keystone, E. C., Fleischmann, R. M., Furst, D. E., Tyson, N., Collinson, N., & Lehane, P. B. (2013). Long-term safety of rituximab in rheumatoid arthritis: 9.5-year follow-up of the global clinical trial programme with a focus on adverse events of interest in RA patients. Annals of the Rheumatic Diseases, 72(9), 1496.
https://doi.org/10.1136/annrheumdis-2012-201956
XL. Vigna-Perez, M., Hernández-Castro, B., Paredes-Saharopulos, O., Portales-Pérez, D., Baranda, L., Abud-Mendoza, C., & González-Amaro, R. (2006). Clinical and immunological effects of Rituximab in patients with lupus nephritis refractory to conventional therapy: A pilot study. Arthritis Research & Therapy, 8(3), R83.
https://doi.org/10.1186/ar1954
XLI. Wang, L., Wang, J., Xu, Y., Jiao, J., Xie, L., & Mo, G. (2021a). A novel therapeutic strategy using extracorporeal membrane oxygenation in patients with anti-neutrophil cytoplasmic antibodies-associated vasculitis: A case report and literature review. 2021, 9(15), 1267.
XLII. Wang, L., Wang, J., Xu, Y., Jiao, J., Xie, L., & Mo, G. (2021b). A novel therapeutic strategy using extracorporeal membrane oxygenation in patients with anti-neutrophil cytoplasmic antibodies-associated vasculitis: A case report and literature review. 2021, 9(15), 1267.
XLIII. Yang, L., Xie, H., Liu, Z., Chen, Y., Wang, J., Zhang, H., Ge, Y., & Hu, W. (2018). Risk factors for infectious complications of ANCA-associated vasculitis: A cohort study. BMC Nephrology, 19(1), 138. https://doi.org/10.1186/s12882-018-0933-2
XLIV. Yokoyama, T., & Kondoh, Y. (2022). Noninvasive Ventilation and High-Flow Oxygen Therapy for ARDS: Does Noninvasive Ventilatory Management Improve the Outcome of ARDS Patients? In S. Tasaka (Ed.), Acute Respiratory Distress Syndrome: Advances in Diagnostic Tools and Disease Management (pp. 89–103). Springer Nature Singapore.https://doi.org/10.1007/978-981-168371-8_7