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IgG4-related disease (IgG4-RD) is a rare fibroinflammatory, multisystemic condition with a relapsing-remitting progression.1 The level of serum IgG4 correlates with inflammatory activity and organ involvement.1 Glucocorticoids are first line for IgG4-RD, but there are numerous adverse effects with chronic use.2 Dupilumab is a monoclonal antibody that acts on the interleukin 4 (IL-4) receptor alpha, shared by the IL-4 and IL-13 receptors.1 IL-4 causes isotype switching from IgM to IgG4 and IL-13 is implicated in fibrosis.3 Thus, it was postulated by the authors to investigate dupilumab as a novel steroid-sparing treatment for IgG4-RD.
A 67-year-old man with no known allergies and a history of sensory neural hearing loss, recurrent bronchitis, spinal stenosis, moderate positional obstructive sleep apnoea, asthma, atopic dermatitis (which caused swelling around his eyes) and allergic rhinoconjunctivitis underwent extensive investigations over the past 2 years due to suspected IgG4-RD.
The patient’s initial complaint was pruritic erythematous lesions on the legs, arms, chest and palms. …
Handling editor Josef S Smolen
Correction notice This article has been corrected since it published Online First. Figure 1 has been updated.
Contributors RSS undertook the primary duties in writing the manuscript, including the case description and discussion as well as submission and editing of the manuscript. SL wrote the introduction of the manuscript and was involved in the editing process. JKL was the physician who identified and managed the case, oversaw the manuscript writing and edited the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests JKL reports receiving research grants, clinical research trial funding and speaker fees from Novartis, Sanofi, Regeneron, Sanofi Genzyme, Astrazeneca, Genentech, Roche and GlaxoSmithKline as well as personal fees from ALK, grants and personal fees from Aralez, and grants and personal fees from Pediapharm. RSS and SL report having nothing to disclose.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.