First-line treatment is glucocorticoids, which induce a rapid disease response, but frequent flares can occur following discontinuation of glucocorticoid treatment. In cases of recurrent disease or disease refractory to steroids the addition of immunosuppressants, such as rituximab, azathioprine, methotrexate, mycophenolate mofetil or cyclophosphamide, is recommended. Although CD20-targeted B cell depletion is a promising treatment, plasmablast-targeted treatment with monoclonal antibodies and novel therapies against CD4+ CTLs may be more specific and effective future treatment approaches for IgG4-RD. R
Despina Michailidou, MD, PhD, is a first-year rheumatology fellow at the National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Md. She obtained her MD from the National and Kapodistrian University of Athens in Greece. She received her PhD from the Goethe University of Frankfurt in Germany. She completed her residency in internal medicine at Yale New Haven Health System.
Paul J. Cohen, MD, chairs the Bridgeport Hospital Department of Pathology and Laboratory Medicine in Bridgeport, Conn. He spends most of his time in general surgical pathology and has a specialty interest in cardiovascular pathology. He’s an assistant professor of pathology at Yale School of Medicine in New Haven, Conn. He’s also the director of the Yale Pathology residency program at Bridgeport Hospital.
Acknowledgment: This review was supported in part by the Intramural Research Program of the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health.
