INTRODUCTION: Muscle and skin involvement are well defined in dermatomyositis but other symptoms contribute significantly to the disease burden and their treatment is not well characterized. This post hoc analysis of ProDERM assessed the effect of intravenous immunoglobulin (IVIg) treatment on other manifestations of dermatomyositis beyond muscular and cutaneous involvement. METHODS: ProDERM was a randomized, placebo-controlled study. For weeks 0-16, patients with dermatomyositis received 2.0 g/kg IVIg (Octagam, 10%) or placebo every 4 weeks. Eligible patients entered the open-label extension, where all received IVIg to week 40. Pulmonary, skeletal, constitutional, gastrointestinal, and cardiovascular disease activity was assessed using the myositis disease activity assessment tool, comprising a visual analog scale (VAS; 0-10 cm) and myositis intention-to-treat activity index. RESULTS: Of 95 patients enrolled, 47 received IVIg and 48 received placebo to week 16. At baseline, 37.9% of patients experienced pulmonary, 64.2% experienced skeletal, 76.8% experienced constitutional, 33.7% experienced gastrointestinal, and 15.8% experienced cardiovascular involvement (VAS > 0.5). Among these patients, for those on IVIg, the following mean VAS scores decreased from baseline to week 16: pulmonary (37.7%; P = 0.001), skeletal (52.6%; P < 0.001), constitutional (44.4%; P < 0.001), and gastrointestinal (49.2%; P = 0.005). No corresponding improvement was seen with placebo except for constitutional VAS. With IVIg, the proportions of patients with arthritis (36.2 to 17.8%; P = 0.01), arthralgia (68.1 to 0.0%; P < 0.001), and fatigue (68.1 to 3.3%; P = 0.008) decreased from baseline to week 16. In the combined cohort, the proportions of patients with dysphonia (20.0 to 8.1%; P = 0.04), arthralgia (66.3 to 39.8%; P < 0.001), weight loss (10.5 to 3.4%; P = 0.04), fatigue (75.8 to 50.0%; P < 0.001), and dysphagia (40.0 to 18.4%; P < 0.001) decreased from baseline to week 40. CONCLUSION: IVIg was effective in treating pulmonary, skeletal, constitutional, and gastrointestinal manifestations of dermatomyositis. We advocate exploring IVIg as treatment for dermatomyositis, beyond muscle and skin manifestations. TRIAL REGISTRATION: ClinicalTrials. gov identifier, NCT02728752.

Department of Dermatology and Allergology University of Szeged Szeged Hungary

Department of Neurology Friedrich Baur Institute Ludwig Maximilians University of Munich Munich Germany

Department of Neurology University of Kansas Medical Center Kansas City KS USA

Division of Clinical Immunology Faculty of Medicine University of Debrecen Debrecen Hungary

Division of Rheumatology and Clinical Immunology University of Pittsburgh School of Medicine Pittsburgh PA USA

Division of Rheumatology Medical College of Georgia at Augusta University Augusta GA USA

Division of Rheumatology University of California 200 Medical Plaza Los Angeles CA 90095 USA

Global Medical and Scientific Affairs Octapharma Pharmazeutika Produktionsges m b H Vienna Austria

Institute of Rheumatology Research Department and Department of Rheumatology 1st Medical Faculty Charles University Prague Czech Republic

Phoenix Neurological Associates Ltd Phoenix AZ USA

Tareev Clinic of Internal Diseases Sechenov 1st Moscow State Medical University Moscow Russia

Zobrazit více v PubMed

Khoo T, Lilleker JB, Thong BY, Leclair V, Lamb JA, Chinoy H. Epidemiology of the idiopathic inflammatory myopathies. Nat Rev Rheumatol. 2023;19(11):695–712. PubMed

Orphanet. Dermatomyositis. Last updated February 2021. https://www.orpha.net/en/disease/detail/221. Accessed Apr 2025.

Kronzer VL, Kimbrough BA, Crowson CS, Davis JM 3rd, Holmqvist M, Ernste FC. Incidence, prevalence, and mortality of dermatomyositis: a population-based cohort study. Arthritis Care Res. 2023;75(2):348–55. PubMed PMC

Chung MP, Paik JJ. Past, present, and future in dermatomyositis therapeutics. Curr Treatm Opt Rheumatol. 2022;8(4):71–90. PubMed PMC

Cho SK, Kim H, Myung J, et al. Incidence and prevalence of idiopathic inflammatory myopathies in Korea: a nationwide population-based study. J Korean Med Sci. 2019;34(8):e55. PubMed PMC

Ikeda S, Arita M, Misaki K, et al. Incidence and impact of interstitial lung disease and malignancy in patients with polymyositis, dermatomyositis, and clinically amyopathic dermatomyositis: a retrospective cohort study. Springerplus. 2015;4:240. PubMed PMC

Marie I, Hachulla E, Cherin P, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002;47(6):614–22. PubMed

Hervier B, Uzunhan Y. Inflammatory myopathy-related interstitial lung disease: from pathophysiology to treatment. Front Med. 2019;6:326. PubMed PMC

Fathi M, Lundberg IE, Tornling G. Pulmonary complications of polymyositis and dermatomyositis. Semin Respir Crit Care Med. 2007;28(4):451–8. PubMed

Joint involvement. In: Dermatomyositis. Berlin: Springer. 10.1007/978-3-540-79313-7_9. Accessed Apr 2025.

Christopher-Stine L, Wan GJ, Kelly W, McGowan M, Bostic R, Reed ML. Patient-reported dermatomyositis and polymyositis flare symptoms are associated with disability, productivity loss, and health care resource use. J Manag Care Spec Pharm. 2020;26(11):1424–33. PubMed PMC

Ricci G, Fontanelli L, Torri F, Schirinzi E, Siciliano G. Fatigue as a common signature of inflammatory myopathies: clinical aspects and care. Clin Exp Rheumatol. 2022;40:425–32. PubMed

Matas-Garcia A, Milisenda JC, Espinosa G, et al. Gastrointestinal involvement in dermatomyositis. Diagnostics (Basel). 2022;12(5):1200. PubMed PMC

Lundberg IE. The heart in dermatomyositis and polymyositis. Rheumatology. 2006;45(Suppl 4):iv18–21. PubMed

Danko K, Ponyi A, Constantin T, Borgulya G, Szegedi G. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. Medicine. 2004;83(1):35–42. PubMed

Marie I. Morbidity and mortality in adult polymyositis and dermatomyositis. Curr Rheumatol Rep. 2012;14(3):275–85. PubMed

Aggarwal R, Charles-Schoeman C, Schessl J, et al. Trial of intravenous immune globulin in dermatomyositis. N Engl J Med. 2022;387(14):1264–78. PubMed

Aggarwal R, Charles-Schoeman C, Schessl J, Dimachkie MM, Beckmann I, Levine T. Prospective, double-blind, randomized, placebo-controlled phase III study evaluating efficacy and safety of octagam 10% in patients with dermatomyositis (“ProDERM Study”). Medicine. 2021;100(1):e23677. PubMed PMC

Aggarwal R, Schessl J, Charles-Schoeman C, et al. Safety and tolerability of intravenous immunoglobulin in patients with active dermatomyositis: results from the randomised, placebo-controlled ProDERM study. Arthritis Res Ther. 2024;26(1):27. PubMed PMC

Myositis disease activity assessment tool (MDAAT), 2005, version 2. https://www.niehs.nih.gov/sites/default/files/2025-05/myositis_disease_activity_assessment_tool_2009_pdfat_508.pdf. Accessed Apr 2025.

Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292(8):403–7. PubMed

Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292(7):344–7. PubMed

Werth VP, Aggarwal R, Charles-Schoeman C, et al. Efficacy of intravenous immunoglobulins (IVIg) in improving skin symptoms in patients with dermatomyositis: a post-hoc analysis of the ProDERM study. EClinicalMedicine. 2023;64:102234. PubMed PMC

Cassard L, Seraly N, Riegert M, Patel A, Fernandez AP. Dermatomyositis: practical guidance and unmet needs. Immunotargets Ther. 2024;13:151–72. PubMed PMC

Charles-Schoeman C, Schessl J, Bata-Csorgo Z, et al. Predictors of response to intravenous immunoglobulin in patients with dermatomyositis: the ProDERM study. Rheumatology (Oxford). 2025;64(6):3767–76. PubMed PMC

Huapaya JA, Hallowell R, Silhan L, et al. Long-term treatment with human immunoglobulin for antisynthetase syndrome-associated interstitial lung disease. Respir Med. 2019;154:6–11. PubMed PMC

Wang LM, Yang QH, Zhang L, et al. Intravenous immunoglobulin for interstitial lung diseases of anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Rheumatology. 2022;61(9):3704–10. PubMed

Labeit B, Pawlitzki M, Ruck T, et al. The impact of dysphagia in myositis: a systematic review and meta-analysis. J Clin Med. 2020;9(7):2150. PubMed PMC

Giannini M, Fiorella ML, Tampoia M, et al. Long-term efficacy of adding intravenous immunoglobulins as treatment of refractory dysphagia related to myositis: a retrospective analysis. Rheumatology. 2020;60(3):1234–42. PubMed

Tavel ME. The placebo effect: the good, the bad, and the ugly. Am J Med. 2014;127(6):484–8. PubMed

Zobrazit více v PubMed

ClinicalTrials.gov
NCT02728752