Intravenous Immunoglobulin Shows Safety and Effectiveness as Treatment of Pyoderma Gangrenosum

Intravenous immunoglobulin (IVIG) was found to be an effective and safe treatment for patients with pyoderma gangrenosum (PG), with a particular role noted for patients who are poor candidates for immunosuppression treatment, according to a systematic review published in the Journal of the European Academy of Dermatology and Venereology.¹

Image credit: freshidea | stock.adobe.com

PG is a rare condition that can cause painful ulcers to develop on the skin, often on the patient’s legs. The exact cause of PG remains unknown, but evidence suggests it is an immune system disorder; people with certain underlying conditions, such as arthritis or inflammatory bowel disease, are at higher risk.²

The condition is most common between 20 and 50 years of age, though it can affect anyone in any age group. Potential complications can include scarring, infection, uncontrolled pain, and the loss of mobility. Treatment for the condition can be challenging due to the lack of literature published on the subject.^1,2

Case reports have been the primary source of research on IVIG treatment for patients with PG. The last systematic review on the subject was published in 2018 and documented a series of PG cases treated with IVIG between 2000 and 2015.^1,3

In total, the review included 49 patients from 26 articles. Since that point, studies with larger cohorts have been completed, allowing for a larger systematic review to be conducted. There were 101 patients identified through 45 papers, both marking a substantial increase from the 2018 review.^1,3

A complete or near-complete resolution of PG following IVIG treatment was observed in 57 cases. There were 18 cases that reported no response. According to a univariate analysis, patients with PG who underwent IVIG treatment after previous or simultaneous corticosteroid use had a 2.85 (95% CI, 1.01-8.08, p-value = 0.049) and 4.41 (95% CI, 1.51-12.86, p-value = 0.007) higher odds of having a partial or complete resolution of PG, respectively.¹

There were also higher odds of complete resolution for patients with a history of pathergy, which is a skin condition that can lead to ulcers. The investigators noted that, in patients with pathergy, the pathogenic mechanisms could be distinct and especially responsive to IVIG therapy, though they noted that more research is required.¹

Interestingly, IVIG was not typically used as a first-line therapy. Systemic steroids (88.0%), cyclosporine (36.0%), mycophenolate (32.0%), infliximab (20%), and adalimumab (20.0%) were used in trials prior to their IVIG treatment.¹

Further, more patients received biologic therapies before IVIG treatment than in the 2018 systematic review. In the current study, 20% of participants trialed infliximab and/or adalimumab prior to IVIG, compared to 14% in the 2018 review.^1,3

In the 2018 review, the investigators found a complete or partial response in 43 (88%) patients, and a complete response in 26 (53%) patients. Only mild adverse events (AEs), such as nausea and headache, were reported in the 2018 review, with similarly limited AEs in the current review. Ultimately, both reviews suggest that there is a role for IVIG as a safe therapy for PG.^1,3

The reviewers noted that their data is likely impacted by publication bias, writing that their univariate analysis should be interpreted with caution as most patients received steroids. More research is necessary on the subject, especially because most of the published literature on treatments for PG is from low-level evidence.¹

“The option of a PG treatment that is not immunosuppressive is significant due to its association with malignancies and other autoimmune conditions,” the investigators concluded.¹

References

1. Gan C, Jobson D, Liu ZF, et al. The efficacy of intravenous immunoglobulin for the treatment of pyoderma gangrenosum: a systematic review and meta-analysis. J Euro Acad Derm Venere. 2024. doi:10.1111/jdv.20274

2. Mayo Clinic Staff. Pyoderma gangrenosum. Mayo Clinic. Updated November 8, 2022. Accessed August 7, 2024. https://www.mayoclinic.org/diseases-conditions/pyoderma-gangrenosum/symptoms-causes/syc-20350386

3. Song H, Lahood N, Mostaghimi A. Intravenous immunoglobulin as adjunct therapy for refractory pyoderma gangrenosum: systematic review of cases and case series. Br J Dermatol. 2018;178(2):363–368. doi:10.1111/bjd.15850