Case Reports

Concurrent herpetic hypertensive anterior uveitis and cytomegalovirus retinitis in a non-HIV patient with melanoma differentiation-associated gene-5 dermatomyositis: intravenous immunoglobulin comes to the rescue

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Published: 21 April 2026
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Melanoma differentiation-associated gene 5 dermatomyositis (MDA-5 DM) is a rare subtype of dermatomyositis. Cytomegalovirus (CMV) retinitis is an opportunistic ocular infection that has historically been linked to HIV infection. Herpes simplex virus (HSV) uveitis is caused by the reactivation of the varicella-zoster virus. We present the case of a 69-year-old woman diagnosed with aggressive MDA-5 DM associated with interstitial lung disease, who received multiple immunosuppressive therapies and subsequently developed left eye CMV retinitis and right eye acute herpetic, hypertensive, anterior uveitis with dendritic keratitis. After the diagnosis of MDA-5 DM, she was treated with glucocorticoids (GC), followed by rituximab and triple therapy (cyclosporine, cyclophosphamide, and GC); however, she did not improve and multiple adverse effects occurred. The patient then started tofacitinib (TOF) and improved. While being on TOF, she developed right eye acute herpetic, hypertensive, anterior uveitis, followed by left eye CMV retinitis. TOF was stopped, and the patient started oral acyclovir and intravenous and intravitreal ganciclovir, which was later changed to maintenance therapy with oral valganciclovir. Cyclical intravenous immunoglobulin (IVIG) administration was started, resulting in an overall improvement in disease activity associated with MDA-5 DM. This case underscores the uncommon link between CMV retinitis in a non-HIV patient and HSV anterior uveitis in a patient undergoing immunosuppressive therapy and the challenge of treating MDA-5 DM with IVIG, which led to favorable results.

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Ruppel T, Forbes Kaprive J, Heydari-Kamjani M, Kesselman MM. Anti-melanoma differentiation-associated gene 5 (Anti-MDA5) dermatomyositis: a case presentation. Cureus 2022; 14: e23102. DOI: https://doi.org/10.7759/cureus.23102
Hirsch S, Pöhler GH, Seeliger B, Prasse A, Witte T, Thiele T. Treatment strategies in MDA5-positive clinically amyopathic dermatomyositis: a single-center retrospective analysis. Clin Exp Med 2024; 24: 37. DOI: https://doi.org/10.1007/s10238-024-01300-8
Mehta P, Machado PM, Gupta L. Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry. Rheumatol Int 2021; 41: 1021-36. DOI: https://doi.org/10.1007/s00296-021-04819-1
Kobayashi R, Hashida N. Overview of cytomegalovirus ocular diseases: retinitis, corneal endotheliitis, and iridocyclitis. Viruses 2024; 16: 1110. DOI: https://doi.org/10.3390/v16071110
Romero-Bueno F, del Campo PD, Trallero-Araguás E, Ruiz-Rodríguez J, Castellvi I, Rodriguez-Nieto M, et al. Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease. Semin Arthritis Rheum 2020; 50: 776-90. DOI: https://doi.org/10.1016/j.semarthrit.2020.03.007
Ge Y, Li S, Tian X, He L, Lu X, Wang G. Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis responds to rituximab therapy. Clin Rheumatol 2021; 40: 2311-7. DOI: https://doi.org/10.1007/s10067-020-05530-5
Chen Z, Wang X, Ye S. Tofacitinib in amyopathic dermatomyositis—associated interstitial lung disease. N Engl J Med 2019; 381: 291-3. DOI: https://doi.org/10.1056/NEJMc1900045
Song MK, Karavellas MP, MacDonald JC, Plummer DJ, Freeman WR. Characterization of reactivation of cytomegalovirus retinitis in patients healed after treatment with highly active antiretroviral therapy. Retina 2000; 20: 151-5. DOI: https://doi.org/10.1097/00006982-200002000-00007
Kuo IC, Kempen JH, Dunn JP, Vogelsang G, Jabs DA. Clinical characteristics and outcomes of cytomegalovirus retinitis in persons without human immunodeficiency virus infection. Am J Ophthalmol 2004; 138: 338-46. DOI: https://doi.org/10.1016/j.ajo.2004.04.015
Munro M, Yadavalli T, Fonteh C, Arfeen S, Lobo-Chan AM. Cytomegalovirus retinitis in HIV and non-HIV individuals. Microorganisms 2019; 8: 55. DOI: https://doi.org/10.3390/microorganisms8010055
Padda IS BR, Parmar M. Tofacitinib. Treasure Island, FL, USA: StatPearls Publishing; 2023.
Winthrop KL, Curtis JR, Lindsey S, Tanaka Y, Yamaoka K, Valdez H, et al. Herpes zoster and tofacitinib: clinical outcomes and the risk of concomitant therapy. Arthritis Rheumatol 2017; 69: 1960-8. DOI: https://doi.org/10.1002/art.40189
Yanagisawa K, Ogawa Y, Hosogai M, Todokoro D, Mitsui T, Yokohama A, et al. Cytomegalovirus retinitis followed by immune recovery uveitis in an elderly patient with rheumatoid arthritis undergoing administration of methotrexate and tofacitinib combination therapy. J Infect Chemother 2017; 23: 572-5. DOI: https://doi.org/10.1016/j.jiac.2017.03.002
Cugley DR, Darby J, Lim LL. Tofacitinib-associated cytomegalovirus retinitis. Rheumatology 2020; 59: e35-7. DOI: https://doi.org/10.1093/rheumatology/kez681
Gupta P, Kharbanda R, Lawrence A, Gupta L. Systemic flare and cutaneous ulceration following cytomegalovirus infection in a patient with anti-melanoma differentiation-associated protein 5 (MDA5) associated myositis: diagnostic challenge during the time of coronavirus disease (COVID-19) pandemic. Egypt Rheumatol 2021; 43: 271-4. DOI: https://doi.org/10.1016/j.ejr.2021.06.001
Tiniakou E, Mecoli CA, Kelly W, Albayda J, Paik J, Adler B, et al. Anti-MDA5-positive dermatomyositis and remission in a single referral centre population. Clin Exp Rheumatol 2023; 41: 309-15. DOI: https://doi.org/10.55563/clinexprheumatol/g4l70r
Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Antimelanoma differentiation-associated gene 5 antibody: expanding the clinical spectrum in North American patients with dermatomyositis. J Rheumatol 2017; 44: 319-25. DOI: https://doi.org/10.3899/jrheum.160682
Allenbach Y, Uzunhan Y, Toquet S, Leroux G, Gallay L, Marquet A, et al. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody. Neurology 2020; 95: e70-8. DOI: https://doi.org/10.1212/WNL.0000000000009727
Bhandari S, Zickuhr L, Baral MR, Bhalla S, Jones H, Bucelli R, Sen D. A review of MDA-5 dermatomyositis and associated interstitial lung disease. Rheumato 2024; 4: 33-48. DOI: https://doi.org/10.3390/rheumato4010004
Pappas DA, Hooper MM, Kremer JM, Reed G, Shan Y, Wenkert D, et al. Herpes zoster reactivation in patients with rheumatoid arthritis: analysis of disease characteristics and disease modifying antirheumatic drugs. Arthritis Care Res 2015; 67: 1671-8. DOI: https://doi.org/10.1002/acr.22628
Tsuji H, Nakashima R, Hosono Y, Imura Y, Yagita M, Yoshifuji H, et al. Multicentre 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 2020; 72: 488-98. DOI: https://doi.org/10.1002/art.41105
Tseng C, Satoh M, Chen Y. AB0697 Dramatic reduction of mortality rate by tofacitinib in anti-MDA-5 antibody-positive patients with rapidly progressive interstitial lung disease. London, UK: BMJ Publishing Group Ltd; 2022. DOI: https://doi.org/10.1136/annrheumdis-2022-eular.3428
Wang LM, Tang QH, Zhang L, Liu SY, Zhang PP, Zhang X, et al. Intravenous immunoglobulin for interstitial lung diseases of anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Rheumatology 2022; 61: 3704-10. DOI: https://doi.org/10.1093/rheumatology/keab928
Shapira Y, Mimouni M, Vishnevskia-Dai V. Cytomegalovirus retinitis in HIV-negative patients – associated conditions, clinical presentation, diagnostic methods and treatment strategy. Acta Ophthalmol 2018; 97: e761-7. DOI: https://doi.org/10.1111/aos.13553
Billet AC, Barba T, Coutant F, Fabien N, Perard L, Sève P, et al. Infection risk in patients with dermatomyositis associated with anti-MDA5 antibodies: a historical cohort study. Biomedicines 2022; 10: 3176. DOI: https://doi.org/10.3390/biomedicines10123176

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1.
Concurrent herpetic hypertensive anterior uveitis and cytomegalovirus retinitis in a non-HIV patient with melanoma differentiation-associated gene-5 dermatomyositis: intravenous immunoglobulin comes to the rescue. Reumatismo [Internet]. 2026 Apr. 21 [cited 2026 Aug. 7];78(2). Available from: https://www.reumatismo.org/reuma/article/view/1826