IgG4-related sclerosing thyroiditis (Riedel-Struma): a review of clinicopathological features and management

• Published in: Virchows Archiv : an international journal of pathology 2023-08, Vol.483 (2), p.133-144
• Main Authors: Czarnywojtek, Agata, Pietrończyk, Krzysztof, Thompson, Lester D. R., Triantafyllou, Asterios, Florek, Ewa, Sawicka-Gutaj, Nadia, Ruchała, Marek, Płazinska, Maria Teresa, Nixon, Iain J., Shaha, Ashok R., Zafereo, Mark, Randolph, Gregory William, Angelos, Peter, Al Ghuzlan, Abir, Agaimy, Abbas, Ferlito, Alfio
• Format: Article
• Language: English
• Subjects: Azathioprine; Cancer; Diagnosis; Etiology; Glucocorticoids; Hashimoto Disease; Humans; Immunoglobulin G; Immunomodulation; Inflammation; Iron; Laboratories; Literature reviews; Medicine; Medicine & Public Health; Methotrexate; Otolaryngology; Pathology; Review and Perspectives; Review and s; Rituximab; Systemic diseases; Thyroid; Thyroid gland; Thyroidectomy; Thyroiditis; Thyroiditis - diagnosis; Thyroiditis - pathology
• ISSN: 0945-6317; 1432-2307
• DOI: 10.1007/s00428-023-03561-2

We present a thorough review of the literature on Riedel thyroiditis (RT) with emphasis on aetiology, diagnosis and management, using the PubMed, Sinomed, and China National Knowledge Infrastructure databases. Although the exact aetiology of RT remains obscure, the histopathological features are consistent with a localized form of IgG 4 -related systemic disease (IgG 4 -RSD). Nevertheless, IgG4-RSD as a systemic fibroinflammatory disorder per se rarely affects the thyroid in the context of multiorgan manifestations. The initial diagnosis of RT is based on clinical history and imaging, but confirmation by histopathological examination is mandatory. In contrast to the historical surgical approach, glucocorticosteroid therapy is currently considered first line therapy, in line with the RT currently being viewed as a manifestation of, or analogous to, IgG4-RSD. For disease relapse, immunomodulatory agents (azathioprine, methotrexate, rituximab) can be used.