| Peer-Reviewed

A Fatal Case of Systemic Lupus Erythematosus Complicated with Diffuse Alveolar Hemorrhage

Received: 22 September 2021    Accepted: 12 October 2021    Published: 28 October 2021
Views:       Downloads:
Abstract

Introduction: SLE is a multisystem autoimmune disease with variable clinical presentation. DAH is a rare but catastrophic manifestation of SLE with high mortality, requiring early, intensive therapy. Case Presentation: A 31-year-old female presented with low-grade fever and joint pains for six weeks associated with alopecia, anorexia, and weight loss. She had pale and had cervical lymphadenopathy, moderate hepatomegaly, and splenomegaly. Hb level was 9.2mg/dL, with a platelet count of 144 × 103/µL. The ESR was 65mm/hour, and the CRP level was 36 mg/L with a UPCR of 332mg/g. She had low C3, C4 levels, positive ANA, and dsDNA titer. A renal biopsy revealed class 3 lupus nephritis. Twenty days after, she was readmitted with acute onset of pleuritic chest pain, cough, and dyspnea, where she was hemodynamically unstable with SpO2 of 85% on air. The Hb level was 8.6 g/dL with a platelet count of 106 × 103/µL. Her condition deteriorated despite standard medical care, where the Hb level and SpO2 dropped to 5.6 d/dL and 65%, respectively, even after blood transfusions, intubation, and artificial ventilation. The chest X-ray revealed bilateral large whitish hazy shadows, and the HRCT scan revealed diffuse bilateral pulmonary hemorrhages. Then she was transferred to the ICU, and there she was started with broad-spectrum antibiotics, methylprednisolone, and plasmapheresis. However, after one week of ICU stay, her renal functions worsened, and she was initiated on CRRT. However, despite all the resuscitation efforts, she succumbed following a cardiac arrest. Conclusion: DAH is a rare catastrophic complication of SLE, which usually presents in patients with an established diagnosis of SLE, even on medical therapy. The diagnosis of DAH is problematic because it mimics a severe pulmonary infection. Early detection and aggressive management are warranted to improve affected patients' outcomes and quality of life.

Published in International Journal of Immunology (Volume 9, Issue 4)
DOI 10.11648/j.iji.20210904.11
Page(s) 68-72
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Systemic Lupus Erythematosus, Diffuse Alveolar Hemorrhage, Pulmonary Hemorrhage

References
[1] A. Fanouriakis et al., "2019 Update of the EULAR recommendations for the management of systemic lupus erythematosus,"Ann. Rheum. Dis., vol. 78, no. 6, pp. 736–745, 2019, doi: 10.1136/annrheumdis-2019-215089.
[2] R. P. S. Virdi, A. Bashir, G. Shahzad, J. Iqbal, and J. O. Mejia, "Diffuse Alveolar Hemorrhage: A Rare Life-Threatening Condition in Systemic Lupus Erythematosus,"Case Rep. Pulmonol., vol. 2012, pp. 1–4, 2012, doi: 10.1155/2012/836017.
[3] L. A. Barile, L. J. Jara, F. Medina-Rodríguez, J. L. García-Figueroa, and J. M. Miranda-Limón, “Pulmonary hemorrhage in systemic lupus erythematosus,” Lupus, vol. 6, no. 5, pp. 445–448, 1997, doi: 10.1177/096120339700600506.
[4] A. R. Lara and M. I. Schwarz, "Diffuse alveolar hemorrhage,"Chest, vol. 137, no. 5, pp. 1164–1171, 2010, doi: 10.1378/chest.08-2084.
[5] A. Narani, "Systemic Lupus Erythematosus (SLE) - a review of clinical approach for diagnosis and current treatment strategies,"Jaffna Med. J., vol. 31, no. 2, p. 9, 2019, doi: 10.4038/jmj.v31i2.73.
[6] A. S. Santos-Ocampo, B. F. Mandell, and B. J. Fessler, "Alveolar hemorrhage in systemic lupus erythematosus: Presentation and management,"Chest, vol. 118, no. 4, pp. 1083–1090, 2000, doi: 10.1378/chest.118.4.1083.
[7] N. K. Al-Adhoubi and J. Bystrom, "Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies, lupus, vol. 29, no. 4, pp. 355–363, 2020, doi: 10.1177/0961203320903798.
[8] M. S. Park, "Diffuse alveolar hemorrhage,"Tuberc. Respir. Dis. (Seoul)., vol. 74, no. 4, pp. 151–162, 2013, doi: 10.4046/trd.2013.74.4.151.
[9] R. Ta, R. Celli, and A. B. West, "Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: Histopathologic Features and Clinical Correlations,"Case Rep. Pathol., vol. 2017, pp. 1–6, 2017, doi: 10.1155/2017/1936282.
[10] G. A. Mahmoud, R. E. Gheith, M. M. Kamel, and R. A. Soliman, "Alveolar hemorrhage in systemic lupus erythematosus: An overview,"Egypt. Rheumatol., vol. 33, no. 1, pp. 1–11, 2011, doi: 10.1016/j.ejr.2010.02.001.
[11] O. Tolaymat and F. Berianu, "Systemic Lupus Erythematosus Presenting with Alveolar Hemorrhage,"Case Rep. Rheumatol., vol. 2018, no. Figure 1, pp. 1–3, 2018, doi: 10.1155/2018/8218904.
[12] S. Di Bartolomeo, A. Alunno, and F. Carubbi, "Respiratory manifestations in systemic lupus erythematosus,"Pharmaceuticals, vol. 14, no. 3, pp. 1–18, 2021, doi: 10.3390/ph14030276.
[13] M. Aringer et al., "2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus,"Ann. Rheum. Dis., vol. 78, no. 9, pp. 1151–1159, 2019, doi: 10.1136/annrheumdis-2018-214819.
[14] N. M. Kazzaz, P. Coit, E. E. Lewis, W. J. McCune, A. H. Sawalha, and J. S. Knight, "Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: Risk factors, therapy and survival,"Lupus Sci. Med., vol. 2, no. 1, 2015, doi: 10.1136/lupus-2015-000117.
[15] C. Ednalino, J. Yip, and S. E. Carsons, "Systematic review of diffuse alveolar hemorrhage in systemic lupus erythematosus: Focus on outcome and therapy,"J. Clin. Rheumatol., vol. 21, no. 6, pp. 305–310, 2015, doi: 10.1097/RHU.0000000000000291.
[16] B. A. De Holanda, I. G. Menna Barreto, I. S. Gomes De Araujo, and D. B. De Araujo, "Alveolar hemorrhage as the initial presentation of systemic lupus erythematosus,"Reumatologia, vol. 54, no. 5, pp. 264–266, 2016, doi: 10.5114/reum.2016.63668.
Cite This Article
  • APA Style

    Parackrama Karunathilake, Ruwanthi Jayasinghe, Thilak Jayalath, Shamali Abeyagunawardena, Udaya Ralapanawa. (2021). A Fatal Case of Systemic Lupus Erythematosus Complicated with Diffuse Alveolar Hemorrhage. International Journal of Immunology, 9(4), 68-72. https://doi.org/10.11648/j.iji.20210904.11

    Copy | Download

    ACS Style

    Parackrama Karunathilake; Ruwanthi Jayasinghe; Thilak Jayalath; Shamali Abeyagunawardena; Udaya Ralapanawa. A Fatal Case of Systemic Lupus Erythematosus Complicated with Diffuse Alveolar Hemorrhage. Int. J. Immunol. 2021, 9(4), 68-72. doi: 10.11648/j.iji.20210904.11

    Copy | Download

    AMA Style

    Parackrama Karunathilake, Ruwanthi Jayasinghe, Thilak Jayalath, Shamali Abeyagunawardena, Udaya Ralapanawa. A Fatal Case of Systemic Lupus Erythematosus Complicated with Diffuse Alveolar Hemorrhage. Int J Immunol. 2021;9(4):68-72. doi: 10.11648/j.iji.20210904.11

    Copy | Download

  • @article{10.11648/j.iji.20210904.11,
      author = {Parackrama Karunathilake and Ruwanthi Jayasinghe and Thilak Jayalath and Shamali Abeyagunawardena and Udaya Ralapanawa},
      title = {A Fatal Case of Systemic Lupus Erythematosus Complicated with Diffuse Alveolar Hemorrhage},
      journal = {International Journal of Immunology},
      volume = {9},
      number = {4},
      pages = {68-72},
      doi = {10.11648/j.iji.20210904.11},
      url = {https://doi.org/10.11648/j.iji.20210904.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.iji.20210904.11},
      abstract = {Introduction: SLE is a multisystem autoimmune disease with variable clinical presentation. DAH is a rare but catastrophic manifestation of SLE with high mortality, requiring early, intensive therapy. Case Presentation: A 31-year-old female presented with low-grade fever and joint pains for six weeks associated with alopecia, anorexia, and weight loss. She had pale and had cervical lymphadenopathy, moderate hepatomegaly, and splenomegaly. Hb level was 9.2mg/dL, with a platelet count of 144 × 103/µL. The ESR was 65mm/hour, and the CRP level was 36 mg/L with a UPCR of 332mg/g. She had low C3, C4 levels, positive ANA, and dsDNA titer. A renal biopsy revealed class 3 lupus nephritis. Twenty days after, she was readmitted with acute onset of pleuritic chest pain, cough, and dyspnea, where she was hemodynamically unstable with SpO2 of 85% on air. The Hb level was 8.6 g/dL with a platelet count of 106 × 103/µL. Her condition deteriorated despite standard medical care, where the Hb level and SpO2 dropped to 5.6 d/dL and 65%, respectively, even after blood transfusions, intubation, and artificial ventilation. The chest X-ray revealed bilateral large whitish hazy shadows, and the HRCT scan revealed diffuse bilateral pulmonary hemorrhages. Then she was transferred to the ICU, and there she was started with broad-spectrum antibiotics, methylprednisolone, and plasmapheresis. However, after one week of ICU stay, her renal functions worsened, and she was initiated on CRRT. However, despite all the resuscitation efforts, she succumbed following a cardiac arrest. Conclusion: DAH is a rare catastrophic complication of SLE, which usually presents in patients with an established diagnosis of SLE, even on medical therapy. The diagnosis of DAH is problematic because it mimics a severe pulmonary infection. Early detection and aggressive management are warranted to improve affected patients' outcomes and quality of life.},
     year = {2021}
    }
    

    Copy | Download

  • TY  - JOUR
    T1  - A Fatal Case of Systemic Lupus Erythematosus Complicated with Diffuse Alveolar Hemorrhage
    AU  - Parackrama Karunathilake
    AU  - Ruwanthi Jayasinghe
    AU  - Thilak Jayalath
    AU  - Shamali Abeyagunawardena
    AU  - Udaya Ralapanawa
    Y1  - 2021/10/28
    PY  - 2021
    N1  - https://doi.org/10.11648/j.iji.20210904.11
    DO  - 10.11648/j.iji.20210904.11
    T2  - International Journal of Immunology
    JF  - International Journal of Immunology
    JO  - International Journal of Immunology
    SP  - 68
    EP  - 72
    PB  - Science Publishing Group
    SN  - 2329-1753
    UR  - https://doi.org/10.11648/j.iji.20210904.11
    AB  - Introduction: SLE is a multisystem autoimmune disease with variable clinical presentation. DAH is a rare but catastrophic manifestation of SLE with high mortality, requiring early, intensive therapy. Case Presentation: A 31-year-old female presented with low-grade fever and joint pains for six weeks associated with alopecia, anorexia, and weight loss. She had pale and had cervical lymphadenopathy, moderate hepatomegaly, and splenomegaly. Hb level was 9.2mg/dL, with a platelet count of 144 × 103/µL. The ESR was 65mm/hour, and the CRP level was 36 mg/L with a UPCR of 332mg/g. She had low C3, C4 levels, positive ANA, and dsDNA titer. A renal biopsy revealed class 3 lupus nephritis. Twenty days after, she was readmitted with acute onset of pleuritic chest pain, cough, and dyspnea, where she was hemodynamically unstable with SpO2 of 85% on air. The Hb level was 8.6 g/dL with a platelet count of 106 × 103/µL. Her condition deteriorated despite standard medical care, where the Hb level and SpO2 dropped to 5.6 d/dL and 65%, respectively, even after blood transfusions, intubation, and artificial ventilation. The chest X-ray revealed bilateral large whitish hazy shadows, and the HRCT scan revealed diffuse bilateral pulmonary hemorrhages. Then she was transferred to the ICU, and there she was started with broad-spectrum antibiotics, methylprednisolone, and plasmapheresis. However, after one week of ICU stay, her renal functions worsened, and she was initiated on CRRT. However, despite all the resuscitation efforts, she succumbed following a cardiac arrest. Conclusion: DAH is a rare catastrophic complication of SLE, which usually presents in patients with an established diagnosis of SLE, even on medical therapy. The diagnosis of DAH is problematic because it mimics a severe pulmonary infection. Early detection and aggressive management are warranted to improve affected patients' outcomes and quality of life.
    VL  - 9
    IS  - 4
    ER  - 

    Copy | Download

Author Information
  • Department of Medicine, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka

  • Teaching Hospital, Peradeniya, Sri Lanka

  • Department of Medicine, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka

  • Department of Medicine, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka

  • Department of Medicine, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka

  • Sections