Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2024.511
Autopsy and Case Reports
Autopsy Case Report

Anti MDA-5 associated rapidly progressive interstitial lung disease complicated by viral pneumonia - a fatal outcome

Saikat Mitra; Nithye Parvathy; Mandeep Garg; Shritik Devkota; Sandeep Bansal; Inderpaul Singh Sehgal; Kirti Gupta

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Abstract

Dermatomyositis is a heterogeneous systemic disease, with 7% to 10% of the individuals presenting the Anti MDA-5 antibody. This subset of patients has clinically amyotropic dermatomyositis, presenting with cutaneous ulcer and rapidly progressive interstitial lung disease. We report the case of a 22-year-old male with a six-month history of low-grade fever associated with myalgia, polyarthralgia, and marked weight loss. He had a history of shortness of breath and high-grade fever 15 days before admission. His clinical features and imaging workup were consistent with acute respiratory distress syndrome. A nasal swab was positive for H1N1 influenza virus infection. During the disease investigation, he succumbed after nine days of admission. The autopsy examination showed diffuse alveolar damage on a background of non-specific interstitial pattern of injury in the lungs. His postmortem muscle biopsy revealed subtle changes of inflammatory myopathy. The brain showed diffuse subarachnoid hemorrhage. Evaluation of postmortem serum sample revealed positivity for Anti MDA-5 and Ro-52 antibodies. This was a case of Anti MDA-5 and Ro-52 associated dermatomyositis with non-specific interstitial pneumonia pattern of lung injury complicated with H1N1 influenza pneumonia, leading to diffuse alveolar damage and subsequent respiratory failure and death. Serum Anti MDA-5 antibodies represent an important biomarker for diagnosing and predicting prognosis for patients with idiopathic inflammatory myopathies, especially clinically amyopathic dermatomyositis. Anti-Ro-52 has been reported in a wide variety of autoimmune diseases, particularly in myositis, scleroderma, and autoimmune liver diseases. Ro-52 autoantibodies are associated with interstitial lung disease (ILD), and their presence should encourage the clinician's curiosity to search for ILD.

Keywords

Dermatomyositis, Idiopathic Interstitial Pneumonias, Influenza A Virus, H1N1 Subtype, Lung Diseases, Interstitial Respiratory Distress Syndrome

References

1 Mehta P, Machado PM, Gupta L. Understanding and managing Anti MDA-5 dermatomyositis, including potential COVID-19 mimicry. Rheumatol Int. 2021;41(6):1021-36. http://doi.org/10.1007/s00296-021-04819-1. PMid:33774723.

2 Nombel A, Fabien N, Coutant F. Dermatomyositis with Anti MDA-5 antibodies: bioclinical features, pathogenesis and emerging therapies. Front Immunol. 2021;12:773352. http://doi.org/10.3389/fimmu.2021.773352. PMid:34745149.

3 Ger TY, Wang FY, Yang SJ, Yen H, Chen CB. Palmar and lateral digital papules as a distinct feature of fatal anti-melanoma differentiation-associated gene 5 dermatomyositis. Dermatol Sin. 2020;38(2):117-8. http://doi.org/10.4103/ds.ds_42_19.

4 Sato S, Hirakata M, Kuwana M, et al. Autoantibodies to a 140-Kd Polypeptide, CADM-140, in Japanese Patients With Clinically Amyopathic Dermatomyositis. Arthritis Rheum. 2005;52(5):1571-6. http://doi.org/10.1002/art.21023 PMid:15880816.

5 Dias AG Jr, Sampaio NG, Rehwinkel J. A balancing act: MDA5 in antiviral immunity and autoinflammation. Trends Microbiol. 2019;27(1):75-85. http://doi.org/10.1016/j.tim.2018.08.007. PMid:30201512.

6 Chen Z, Cao M, Plana MN, et al. Utility of anti-melanoma differentiation–associated gene 5 antibody measurement in identifying patients with dermatomyositis and a high risk for developing rapidly progressive interstitial lung disease: a review of the literature and a meta-analysis. Arthritis Care Res (Hoboken). 2013;65(8):1316-24. http://doi.org/10.1002/acr.21985. PMid:23908005.

7 Gono T, Kawaguchi Y, Satoh T, et al. Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis. Rheumatology. 2010;49(9):1713-9. http://doi.org/10.1093/rheumatology/keq149. PMid:20498012.

8 Koga T, Fujikawa K, Horai Y, et al. The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatology. 2012;51(7):1278-84. http://doi.org/10.1093/rheumatology/ker518. PMid:22378718.

9 Kim M, Harvey S, Danoff SK, et al. Rapidly progressive interstitial lung disease in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis: serial changes on HRCT. Emerg Radiol. 2022;29(6):961-7. http://doi.org/10.1007/s10140-022-02080-y. PMid:35918568.

10 Chino H, Sekine A, Baba T, et al. Radiological and pathological correlation in Anti MDA-5 antibody-positive interstitial lung disease: rapidly progressive perilobular opacities and diffuse alveolar damage. Intern Med. 2016;55(16):2241-6. http://doi.org/10.2169/internalmedicine.55.5774. PMid:27523002.

11 Kotsiou OS, Daniil Z, Gourgoulianis KI. Pneumomediastinum in MDA-5-associated clinically amyopathic dermatomyositis. Rheumatol Adv Pract. 2017;1(1):rkx003. http://doi.org/10.1093/rap/rkx003. PMid:31431943.

12 Xu A, Ye Y, Fu Q, et al. Prognostic values of anti-Ro52 antibodies in Anti MDA-5-positive clinically amyopathic dermatomyositis associated with interstitial lung disease. Rheumatology. 2021;60(7):3343-51. http://doi.org/10.1093/rheumatology/keaa786. PMid:33331866.

13 Marie I, Dominique S, Janvresse A, Levesque H, Menard JF. Rituximab therapy for refractory interstitial lung disease related to antisynthetase syndrome. Respir Med. 2012;106(4):581-7. http://doi.org/10.1016/j.rmed.2012.01.001. PMid:22280877.

14 Ge Y, Zhou H, Shi J, et al. The efficacy of tacrolimus in patients with refractory dermatomyositis/polymyositis: a systematic review. Clin Rheumatol. 2015;34(12):2097-103. http://doi.org/10.1007/s10067-015-3065-0. PMid:26328518.
 


Submitted date:
06/03/2024

Accepted date:
07/10/2024

Publication date:
08/30/2024

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