Patients With Juvenile Dermatomyositis Treated With IVIG Had No Increased Risk of Thromboembolic Events

There were no differences in the occurrence of thromboembolic events (TE) in patients with juvenile dermatomyositis (JDM) treated with intravenous immunoglobulin (IVIG) compared with those without IVIG exposure, according to study results from a single-institution retrospective cohort trial. The findings demonstrate that there is no significantly increased risk of TE attributed to IVIG.¹

Dermatomyositis can cause muscle weakness and skin rash in adult and pediatric patients. | Image Credit: © Pekr | stock.adobe.com

JDM, an autoimmune disease, can cause inflammation of the skin, muscles, and other organs resulting in skin rash, proximal muscle weakness, and skin ulceration. Patients who are unresponsive to treatments have been observed to have increased mortality and complications compared with those who respond well. Experts have suggested that, in patients with a long course of disease, high levels of tumor necrosis factor inhibitor have been reported.^2,3

Typically, glucocorticoids are the recommended first-line treatment, followed by immunosuppressants and IVIG in further lines. However, investigators in the placebo-controlled ProDERM trial found that IVIG significantly improved symptoms of JDM compared with placebo. The trial enrolled 95 patients, who had all been previously treated and did not demonstrate a strong response. At 16 weeks, 79% of patients in the IVIG group had a score improvement of at least 20, compared with 44% of the placebo group.³

Despite effectiveness in these patients, IVIG carries an FDA warning for thromboembolic events, which has raised concerns of whether IVIG administration can increase the risk of TEs in patients with JDM. The investigators conducted a single-institution, retrospective cohort study evaluating the risk of TE in patients with JDM treated with IVIG. In total, 125 patients with JDM who received IVIG and 110 patients who did not receive IVIG were identified.¹

Notably, there were no incident TEs in either cohort. The investigators ensured that patients had a closely matched prevalence of all measured clotting risk factors, except history of peripherally inserted central catheter line, which was more likely in patients receiving IVIG (8% vs 1%; P = .007). On average, patients received IVIG for 877 days, and the likelihood of requiring a complementary Janus kinase inhibitor was independent of IVIG history.¹

Given the unclear data and lack of robust evidence regarding the true risk of IVIG use in JDM, these results provide much-needed clarity. Results of a prior study from Aggarwal et al contrast with this data, documented many treatment-related adverse events (AEs) in their IVIG patient cohort, including 9 serious AEs that were considered related to IVIG—and 6 TE events. However, the current investigators caution that the study was not powered to evaluate TE risk with IVIG exposure.^1,4

In contrast, the results of this study provide a pediatric lens to evidence, which suggests that despite prior studies showing otherwise, there is no significant TE risk with IVIG patients in adults or juveniles. They also discuss that the absence of TE in either cohort of patients is likely attributable to lower patient baseline clotting risk compared to adult DM patients, noting the lower rates of underlying malignancy and pro-thrombotic lifestyle factors.¹

REFERENCES

1. Gomes LLA, Silva GS, Carol HA, Subhadarshani S, Rutstein BH, and Shaw KS. Thromboembolic risk in juvenile dermatomyositis patients treated with intravenous immunoglobulin. Pediatric Dermatology. 2025. Published online January 9, 2025. doi:10.1111/pde.15865

2. Toich L. Potential new treatment for patients with juvenile dermatomyositis. Pharmacy Times^®. Published June 15, 2016. Accessed January 22, 2025. https://www.pharmacytimes.com/view/potential-new-treatment-for-patients-with-juvenile-dermatomyositis

3. Hunter E. Intravenous immune globulin may improve dermatomyositis symptoms in adults. Pharmacy Times. Published October 11, 2022. Accessed January 22, 2025. https://www.pharmacytimes.com/view/intravenous-immune-globulin-may-improve-dermatomyositis-symptoms-in-adults

4. Aggarwal R, Charles-Schoeman C, Schessl J, et al. Trial of intravenous immune globulin in dermatomyositis. New Engl J Med. 2022;387(14):1264-1278. doi:10.1056/NEJMoa2117912