Periaortitis and periarteritis are one of the clinical features of IgG4-related disease. Such vascular manifestations are reported to be present in 10-30% of overall IgG4-related disease, and they may also appear as an isolated lesion of IgG4-related disease. As other types of large vessel vasculitis, IgG4-related vasculitis may cause significant clinical consequences. On the other hand, the diagnosis of IgG4-related periaortitis/periarteritis seems to have been made on the basis of different diagnostic criteria in reported studies; thus far, difficulty seems to be present in assessing the true frequency of the disease as well as determining the optimal therapeutic strategy. In this review, in addition presenting the clinical and pathological features of IgG4-related periaortitis/periarteirtis, we would like to discuss on what kind of points should be resolved for the better understanding of vascular involvement of IgG4-related disease in terms of diagnosis and treatment.

IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening.

Overview of IgG4-related aortitis and periaortitis. A decade since their first description.

Purpose of reviewTo summarize current evidence regarding the pathophysiology, diagnostic criteria, and management of IgG4 disease in the head and neck.Recent findingsThe anti-CD20 antibody, rituximab is being used increasingly as a primary treatment modality in cases with a definitive diagnosis. Des

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IgG4-related disease in the head and neck.

<h2>Abstract</h2> Immunoglobulin (Ig)G4-related disease (IgG4-RD) with retroperitoneal fibrosis (RPF) is a rare, fibroinflammatory disease involving the soft tissues of the retroperitoneum. A 73-year-old man with IgG4-related RPF affecting the abdominal aorta and iliac arteries was treated with steroids and mycophenolate mofetil. The prevalence of the disease remains unknown because it is often misdiagnosed and can mimic many malignant, infectious, and inflammatory conditions. Autoimmune pancreatitis is a common presenting condition of IgG4-RD. Because As IgG4-RD is responsive to steroids, diagnosing IgG4-related RPF early can prevent the exposure of patients with RPF to unnecessary diagnostic and therapeutic interventions. 

http://www.jvscit.org/article/S2468428722000223/pdf

A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge

Retroperitoneal fibrosis is caused by the replacement of normal retroperitoneal tissue with fibrosis. The majority of the cases are idiopathic, but some secondary causes include malignancy, infection, drugs, and radiotherapy. Immunoglobulin G-4 (IgG-4) related disease is a relatively newer disease and one of the rarer causes of retroperitoneal fibrosis. It usually involves the pancreas, lungs, kidneys, aorta lacrimal and salivary glands, or extrapancreatic bile duct. Elevated serum IgG-4 is the biomarker of the disease and its levels correlate with disease activity. High-dose glucocorticoid is the treatment of choice.

https://journals.sagepub.com/doi/pdf/10.1177/23247096211022487

Immunoglobulin G-4-Related Retroperitoneal Fibrosis.

Retroperitoneal fibrosis (RPF) is a progressive fibroinflammatory disease that can be complicated by urinary obstruction. RPF can be the only manifestation of IgG4-related disease (IgG4-RD). Treatment of IgG4-related RPF is challenging and mostly consists of long-term glucocorticoids leading to significant side effects and treatment intolerance. Recent exploration of the role of rituximab as a B-cell depleting therapy in the treatment of IgG4-RD provides therapeutic potential as a well-tolerated alternative to glucocorticoids. We present a case of IgG4-related RPF for which rituximab was instituted as a steroid-sparing treatment strategy. Following 4 doses, kidney function partially recovered, and the disease went into remission. We discuss the potential merit of rituximab for the treatment of patients with IgG4-related RPF.

/pdf/rituximab-for-retroperitoneal-fibrosis-due-to-igg4-related-4rcn4444up.pdf

Aleksandr Perepletchikov

Papers

Rituximab for retroperitoneal fibrosis due to IgG4-related disease: A case report and literature review.