IgG4 disease and rituximab – pro
IgG4‐related disease (IgG4‐RD) is an immune‐mediated fibroinflammatory condition that can affect multiple organs and lead to tumefactive, tissue‐destructive lesions and organ failure 1. Over the last decade, the disease has become recognized as a unified systemic disorder that links many individual organ conditions once considered to be unrelated, Multiple approaches to the management of IgG4‐RD have been reported, including surgical resection of affected tissues and treatment with systemic glucocorticoids, “steroid‐sparing” immunosuppressive drugs, or biologic agents, Since there are no randomized clinical trials. experts have provided a guideline (Khosposhani et al).
The guideline recommends early treatment. , even when the disease is to symptomatic: “Subclinical disease can lead to severe, irreversible sequelae in the biliary tree, kidney, aorta, mediastinum, retroperitoneum, mesentery, and other organs 43. However, not all manifestations of IgG4‐RD require immediate treatment. “Watchful waiting” may be appropriate, for example, in patients with asymptomatic lymphadenopathy or mild submandibular gland enlargement.
Involvement of certain organs may be relatively asymptomatic until the late stages of disease, by which time chronic inflammation and fibrosis may have caused irreversible damage. Patients with AIP who are not treated with induction immunosuppression are less likely to achieve remission and more likely to experience disease complications. The importance of early intervention to prevent complications related to progressive fibrosis in the salivary glands has also been demonstrated “. IT also recommend rituximab and maintenance with rituximab, when stroids ahve nto been successffuol.
Zachary S. Wallace Hamid Mattoo Vinay S. Mahajan Maria Kulikova Leo Lu Vikram Deshpande Hyon K. Choi Shiv Pillai John H. Stone, Predictors of disease relapse in IgG4-related disease following rituximab Rheumatology, Volume 55, Issue 6, 1 June 2016, Pages 1000–1008,
A. Khosroshahi Z. S. Wallace J. L. Crowe T. Akamizu A. Azumi M. N. Carruthers S. T. Chari E. Della‐Torre L. Frulloni H. Goto P. A. Hart T. Kamisawa S. Kawa M. Kawano M. H. Kim Y. Kodama K. Kubota M. M. Lerch M. Löhr Y. Masaki S. Matsui T. Mimori S. Nakamura T. Nakazawa H. Ohara K. Okazaki J.H. Ryu T. Saeki N. Schleinitz A. Shimatsu T. Shimosegawa H. Takahashi M. Takahira A. Tanaka M. Topazian H. Umehara G. J. Webster T. E. Witzig M. Yamamoto W. Zhang T. Chiba J. H. Stone, International Consensus Guidance Statement on the Management and Treatment of IgG4‐Related Disease. Arthritis adn Rehumatology Volume67, Issue7 July 2015 Pages 1688-1699