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Asena Gökçay Canpolat Department of Endocrinology and Metabolism, School of Medicine, Ankara University, Ankara, Turkey

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Murat Cinel Department of Endocrinology and Metabolism, School of Medicine, Ankara University, Ankara, Turkey

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Serpil Dizbay Sak Department of Pathology, School of Medicine, Ankara University, Ankara, Turkey

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Işılay Taşkaldıran Department of Endocrinology and Metabolism, Ankara Training and Research Hospital, Ankara, Turkey

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Hakan Korkmaz Department of Endocrinology and Metabolism, School of Medicine, Süleyman Demirel University, Isparta, Turkey

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Özgür Demir Department of Endocrinology and Metabolism, School of Medicine, Ankara University, Ankara, Turkey

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Reyhan Ersoy Department of Endocrinology and Metabolism, School of Medicine, Yıldırım Beyazıt University, Ankara, Turkey

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Selçuk Dağdelen Department of Endocrinology and Metabolism, School of Medicine, Hacettepe University, Ankara, Turkey

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Dilek Berker Department of Endocrinology and Metabolism, Ankara City Hospital, University of Health Sciences, Ankara, Turkey

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Klara Dalva Department of Haematology, School of Medicine, Ankara University, Ankara, Turkey

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Adile Begüm Bahçecioğlu Mutlu Department of Endocrinology and Metabolism, School of Medicine, Ankara University, Ankara, Turkey

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Murat Faik Erdoğan Department of Endocrinology and Metabolism, School of Medicine, Ankara University, Ankara, Turkey

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Background: Riedel thyroiditis (RT) is a rare form of thyroiditis; thus, data about the disease course and treatment options are limited. Therefore, we aimed to assess the clinical, serological, radiological, and histopathological features, as well as short- and long-term follow-up of RT patients under glucocorticoid (GC) and tamoxifen citrate (TMX). Parameters related to IgG4-related diseases (IgG4-RD) were also investigated. Methods: Eight patients with RT diagnosed between 2000 and 2019 were enrolled. Data were collected in a retrospective and prospective manner. The diagnosis was confirmed with histopathological features in all patients. Results of the treatment with GCs on short- to mid-term, followed by TMX in the long term, were evaluated. Results: The mean age at diagnosis was 40.5 ± 6.8 years; female predominance was observed (F/M:7/1). Parameters related to IgG4-RD, like increase in IgG4 serum levels, total plasmablast counts, and IgG4+ plasmablasts, were negative in most of our patients in both active and inactive states of the disease. Likewise, an increased ratio of IgG4/IgG-positive plasma cells >40% could only be observed in 2 cases. GCs followed by TMX were given to the patients with an over-all median follow-up time of 67 (8–216) months. All the patients considerably improved clinically and had a reduction in the size of the mass lesion on GCs, followed by TMX therapy. None of the patients had a recurrence under TMX therapy for a median period of 18.5 (7–96) months. Conclusion: Even though RT is suggested to be a member of IgG4-RD, serologic or histological evidence of IgG4 elevation or positivity is only useful for diagnosis and follow-up of RT. The diagnosis should be based on clinical and radiological evidence and confirmed by histopathology. GCs are effective for initial treatment, and TMX is a successful and safe therapeutic option for long-term maintenance therapy.

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