Skip to main content

Advertisement

SpringerLink
Log in

Lung transplantation for anti-MDA5-positive dermatomyositis-associated rapid progressive interstitial lung disease: report of two cases and review of the literature

  • Case Based Review
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Lung transplantation is an ultimate lifesaving treatment for many patients with end-stage lung disease, whereas whether it is an optional intervention for the anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive dermatomyositis (DM)-associated rapid progressive interstitial lung disease (RP-ILD) remain controversial. We report two patients diagnosed with anti-MDA5-positive DM-associated RP-ILD, who were both bridging to lung transplant with extracorporeal membrane oxygenation (ECMO) after failing to respond to extensive immunosuppressants. The first patient received full rehabilitation, but the second patient died of DM flare at the early-stage post-lung transplantation. Most of the clinical information was parallel in these two patients except the anti-MDA5 antibody level, which gradually decreased and became negative in the first patient but always hovering in high titers in the second patient, although both of the two patients received standard immunosuppressive regimen for prevention of rejection after lung transplantation. A total of 11 patients with anti-MDA5-positive DM-associated RP-ILD who underwent lung transplantation from the literature were identified. Most patients (10/11, 90.1%) were successfully discharged and without DM flare during the follow-up period post-lung transplantation. Nine of them were followed up more than 1 year, and anti-MDA-5 antibody was reported to be negative in four patients, whereas the others were unavailable. Combined with the case series in the literature, our limited experience suggests that lung transplantation is a promising therapeutic option for end-stage patients with anti-MDA5-positive DM-associated RP-ILD, with ECMO as a bridge to lung transplantation, if necessary. However, clearance or a downtrend of anti-MDA5 antibody may be required pre-transplant to avoid DM flare and recurrent RP-ILD post-transplantation.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

Abbreviations

CADM :

clinical amyopathic dermatomyositis

CK :

creatine kinase

CTD :

connective tissue disease

CYC :

cyclophosphamide

DM :

dermatomyositis

ECMO :

extracorporeal membrane oxygenation

FER :

ferritin

GGO :

ground-glass opacity

HRCT :

high-resolution computed tomography

ILD :

interstitial lung disease

LDH :

lactate dehydrogenase

MDA5 :

melanoma differentiation-associated gene 5

MDT :

multidisciplinary team

MMF :

mycophenolate mofetil

mPSL :

methylprednisolone

MSAs :

myositis-specific autoantibodies

MYO :

myohemoglobin

PE :

plasma exchange

RP-ILD :

rapid progressive interstitial lung disease

SpO 2 :

oxygen saturation

References

  1. Sun KY, Fan Y, Wang YX, Zhong YJ, Wang GF (2021) Prevalence of interstitial lung disease in polymyositis and dermatomyositis: a meta-analysis from 2000 to 2020. Semin Arthritis Rheum 51:175–191. https://doi.org/10.1016/j.semarthrit.2020.11.009

    Article  CAS  PubMed  Google Scholar 

  2. Wong VT, So H, Lam TT, Yip RM (2021) Myositis-specific autoantibodies and their clinical associations in idiopathic inflammatory myopathies. Acta Neurol Scand 143:131–139. https://doi.org/10.1111/ane.13331

    Article  CAS  PubMed  Google Scholar 

  3. Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R (2016) Anti-melanoma differentiation-associated gene 5 is associated with rapidly progressive lung disease and poor survival in us patients with amyopathic and myopathic dermatomyositis. Arthritis Care Res (Hoboken) 68:689–694. https://doi.org/10.1002/acr.22728

    Article  CAS  PubMed  Google Scholar 

  4. Romero-Bueno F, Diaz Del Campo P, Trallero-Araguás E et al (2020) Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease. Semin Arthritis Rheum 50:776–790. https://doi.org/10.1016/j.semarthrit.2020.03.007

    Article  CAS  PubMed  Google Scholar 

  5. Ju C, Lian Q, Chen A et al (2021) Outcomes after lung transplantation among chinese patients with connective tissue disease-associated interstitial lung disease and pulmonary hypertension: a retrospective cohort study. Clin Exp Rheumatol. https://doi.org/10.55563/clinexprheumatol/hld9sf

  6. Shoji T, Bando T, Fujinaga T et al (2013) Living-donor lobar lung transplantation for rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis: report of a case. Gen Thorac Cardiovasc Surg 61:32–34. https://doi.org/10.1007/s11748-012-0106-3

    Article  PubMed  Google Scholar 

  7. Leclair V, Labirua-Iturburu A, Lundberg IE (2018) Successful lung transplantation in a case of rapidly progressive interstitial lung disease associated with antimelanoma differentiation-associated gene 5 antibodies. J Rheumatol 45:581–583. https://doi.org/10.3899/jrheum.171047

    Article  PubMed  Google Scholar 

  8. Deitchman AR, Kalchiem-Dekel O, Todd N, Reed RM (2019) Rapidly progressive interstitial lung disease due to anti-melanoma differentiation associated protein-5 requiring a bilateral lung transplant, and complicated by kennel cough. Respir Med Case Rep 28:100886. https://doi.org/10.1016/j.rmcr.2019.100886

    Article  PubMed  PubMed Central  Google Scholar 

  9. Huang K, Levy RD, Avina-Zubieta JA (2020) Successful lung transplant in rapid progressive interstitial lung disease associated with anti-melanoma differentiation associated gene 5. Rheumatology (Oxford) 59:2161–2163. https://doi.org/10.1093/rheumatology/keaa032

    Article  CAS  PubMed  Google Scholar 

  10. Pacot L, Pouchot J, De Prost N et al (2020) Interstitial lung disease-complicated anti-mda5 antibody in clinically amyopathic dermatomyositis patients: report of two cases with distinct clinical features. Front Med (Lausanne) 7:77. https://doi.org/10.3389/fmed.2020.00077

    Article  PubMed  Google Scholar 

  11. Jhajj AS, Shun Yeung JH, To F (2021) Spontaneous pneumomediastinum due to anti-melanoma differentiation-associated protein 5 requiring a bilateral lung transplant. Case Rep Rheumatol 2021:6097183. https://doi.org/10.1155/2021/6097183

    Article  PubMed  PubMed Central  Google Scholar 

  12. Marchiset A, Neuville M, Voiriot G et al (2021) High-emergency lung transplantation for interstitial lung disease associated with anti-mda5 dermatomyositis: a case report. Transplant Proc 53:2613–2615. https://doi.org/10.1016/j.transproceed.2021.08.012

    Article  PubMed  Google Scholar 

  13. Gu Q, Diao M, Hu W, Huang M, Zhu Y (2021) Case report: Extracorporeal membrane oxgenation for rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis in a post-partum woman. Front Med (Lausanne) 8:742823. https://doi.org/10.3389/fmed.2021.742823

    Article  PubMed  Google Scholar 

  14. Sato S, Hirakata M, Kuwana M et al (2005) Autoantibodies to a 140-kd polypeptide, cadm-140, in japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum 52:1571–1576. https://doi.org/10.1002/art.21023

    Article  CAS  PubMed  Google Scholar 

  15. Sato S, Kuwana M, Fujita T, Suzuki Y (2013) Anti-cadm-140/mda5 autoantibody titer correlates with disease activity and predicts disease outcome in patients with dermatomyositis and rapidly progressive interstitial lung disease. Mod Rheumatol 23:496–502. https://doi.org/10.1007/s10165-012-0663-4

    Article  CAS  PubMed  Google Scholar 

  16. Zhang L, Wu G, Gao D et al (2016) Factors associated with interstitial lung disease in patients with polymyositis and dermatomyositis: a systematic review and meta-analysis. PLoS ONE 11:e0155381. https://doi.org/10.1371/journal.pone.0155381

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Xu Y, Zhang Y, Yang H et al (2022) Evaluation and validation of the prognostic value of anti-MDA5 IgG subclasses in dermatomyositis-associated interstitial lung disease. Rheumatology (Oxford). https://doi.org/10.1093/rheumatology/keac229

    Article  PubMed  PubMed Central  Google Scholar 

  18. Tsuji H, Nakashima R, Hosono Y et al (2020) Multicenter prospective study of the efficacy and safety of combined immunosuppressive therapy with high-dose glucocorticoid, tacrolimus, and cyclophosphamide in interstitial lung diseases accompanied by anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Arthritis Rheumatol 72:488–498. https://doi.org/10.1002/art.41105

    Article  CAS  PubMed  Google Scholar 

  19. Park JE, Kim SY, Song JH et al (2018) Comparison of short-term outcomes for connective tissue disease-related interstitial lung disease and idiopathic pulmonary fibrosis after lung transplantation. J Thorac Dis 10:1538–1547. https://doi.org/10.21037/jtd.2018.02.50

  20. Matsushita T, Mizumaki K, Kano M et al (2017) Antimelanoma differentiation-associated protein 5 antibody level is a novel tool for monitoring disease activity in rapidly progressive interstitial lung disease with dermatomyositis. Br J Dermatol 176:395–402. https://doi.org/10.1111/bjd.14882

    Article  CAS  PubMed  Google Scholar 

  21. Endo Y, Koga T, Ishida M et al (2018) Recurrence of anti-mda5 antibody-positive clinically amyopathic dermatomyositis after long-term remission: A case report. Medicine (Baltimore) 97:e11024. https://doi.org/10.1097/MD.0000000000011024

    Article  PubMed  Google Scholar 

  22. Suzuki S, Ikeda K, Yamaji K, Tamura N, Morimoto S (2021) Recurrence in long-term survivor of anti-mda5 antibody-positive clinically amyopathic dermatomyositis: case series and literature review. Mod Rheumatol Case Rep 5:310–316. https://doi.org/10.1080/24725625.2021.1886666

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

The authors would like to acknowledge all who contributed in this case diagnosis, therapy, and decision-making.

Funding

(1) Zhongnanshan Medical Foundation of Guangdong Province (ZNSA-2020013).

(2) Foundation from Guangzhou Institute of Respiratory Health (2019GIRHZ04).

(3) Natural Science Foundation of Guangdong Province (2022A1515012216).

Author information

Author notes

  1. Qun Luo, Jian-xing He, and Chun-rong Ju contributed equally to this paper.

Authors and Affiliations

  1. State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, NO.151, Yanjiang Road, Yuexiu District, Guangzhou, 510120, China

    Qiao-yan Lian, Ao Chen, Jian-heng Zhang, Xin Xu, Dan-xia Huang, Qun Luo, Jian-xing He & Chun-rong Ju

  2. Department of Thoracic Surgery, Guangzhou Institute of Respiratory Disease, First Affiliated Hospital of Guangzhou Medical University, Guangzhou, China

    Xin Xu, Dan-xia Huang & Jian-xing He

Authors
  1. Qiao-yan Lian
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ao Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jian-heng Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Xin Xu
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Dan-xia Huang
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Qun Luo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Jian-xing He
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Chun-rong Ju
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Jian-xing He, Qun Luo, and Chun-rong Ju supervised the study; Qiao-yan Lian collected the data and drafted the manuscript; Chun-rong Ju, Qun Luo, Qiao-yan Lian, Ao Chen, Jian-heng Zhang, Xin Xu, and Dan-xia Huang took care of the patients in clinical setting; Chun-rong Ju revised the manuscript. All authors read and approved the final manuscript.

Corresponding authors

Correspondence to Qun Luo, Jian-xing He or Chun-rong Ju.

Ethics declarations

Ethics approval and consent to participate

The study was conducted in accordance with the Declaration of Helsinki and approved by the Ethics Review Committee of the First Affiliated Hospital of Guangzhou Medical University (NO. K-09, 2022). Patient approval and informed consent were waived because of the retrospective review of patient’s records.

Disclosure

None.

Additional information

Publisher's note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Lian, Qy., Chen, A., Zhang, Jh. et al. Lung transplantation for anti-MDA5-positive dermatomyositis-associated rapid progressive interstitial lung disease: report of two cases and review of the literature. Clin Rheumatol 42, 941–947 (2023). https://doi.org/10.1007/s10067-022-06422-6

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-022-06422-6

Keywords

Search

Navigation