open access

Vol 73, No 4 (2022)
Review paper
Submitted: 2022-01-11
Accepted: 2022-03-17
Published online: 2022-07-18
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Optimization of the treatment of moderate to severe and active thyroid orbitopathy considering the recommendations of the European Group on Graves’ Orbitopathy (EUGOGO) [Optymalizacja leczenia umiarkowanej do ciężkiej i aktywnej orbitopatii tarczycowej z uwzględnieniem zaleceń European Group on Graves’ Orbitopathy (EUGOGO)]

Mariusz Nowak1, Bogdan Marek1, Beata Kos-Kudła2, Lucyna Siemińska1, Magdalena Londzin-Olesik2, Joanna Głogowska-Szeląg1, Wojciech Nowak3, Dariusz Kajdaniuk1
·
Pubmed: 36059167
·
Endokrynol Pol 2022;73(4):756-777.
Affiliations
  1. Pathophysiology Division, Department of Pathophysiology and Endocrinology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in Katowice, Poland
  2. Department of Endocrinology and Neuroendocrine Tumours, Department of Pathophysiology and Endocrinology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in Katowice
  3. Science Students’ Association, Pathophysiology Division, Department of Pathophysiology and Endocrinology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in Katowice, Poland

open access

Vol 73, No 4 (2022)
Review Article
Submitted: 2022-01-11
Accepted: 2022-03-17
Published online: 2022-07-18

Abstract

Graves’ disease (GB), also known as Basedow’s disease, is the most common cause of hyperthyroidism, and thyroid orbitopathy (TO) is its most common non-thyroid manifestation with an incidence of 42.2/million people/year. Based on the guidelines of the European Graves’ Orbitopathy Group (EUGOGO), certain management standards presented in our publication should be used to optimize and improve the efficacy of TO treatment. Deciding on the optimal treatment for both hyperthyroidism and TO requires a cooperative team of specialists: endocrinologist, ophthalmologist, radiation therapist, and surgeon, as well as consideration of the risk of relapse and possible complications of the treatment method.

The inflammatory activity and severity of TO should be diagnosed based on the investigator’s own experience and according to standard diagnostic criteria. Assessment of the inflammatory activity of TO can be performed using the clinical activity score (CAS) and using imaging methods — mainly MRI. The severity of TO is assessed using a seven-grade NOSPECS classification and a three-grade EUGOGO scale. In moderate to severe and active TO, i.v. methylprednisolone pulses are the treatment of choice. It is important to maintain the standard and regimen of treatment. The recommended standard as first-line treatment in most patients with moderate to severe and active TO is the combined use of methylprednisolone i.v. (cumulative dose of 4.5 g over 12 weeks) with concurrent administration of mycophenolate sodium 0.72 g per day for 24 weeks.

In more severe forms of moderate to severe and active TO, a higher cumulative dose of methylprednisolone i.v. is recommended as an alternative first-line treatment (7.5 g) as monotherapy starting with a dose of 0.75 g once a week for 6 weeks and 0.5 g for a further 6 weeks. EUGOGO guidelines recommend that in cases of no clinical response after 6 weeks of first-line treatment with i.v. methylprednisolone and mycophenolate, after 3–4 weeks, a second course of i.v. methylprednisolone monotherapy should be started with a higher cumulative dose of 7.5 g. Other second-line treatment options are orbital radiotherapy with or without oral or i.v. systemic glucocorticosteroid therapy, cyclosporine, or azathioprine in combination with p.o. glucocorticosteroid, methotrexate monotherapy, and a group of biologic drugs rituximab, tocilizumab, teprotumumab).

Keeping in mind that TO is a sight-threatening disease, we expect, through the treatment applied, to maintain full visual acuity, pain relief, single vision in the useful part of the visual field, and a positive cosmetic effect.

 

Abstract

Graves’ disease (GB), also known as Basedow’s disease, is the most common cause of hyperthyroidism, and thyroid orbitopathy (TO) is its most common non-thyroid manifestation with an incidence of 42.2/million people/year. Based on the guidelines of the European Graves’ Orbitopathy Group (EUGOGO), certain management standards presented in our publication should be used to optimize and improve the efficacy of TO treatment. Deciding on the optimal treatment for both hyperthyroidism and TO requires a cooperative team of specialists: endocrinologist, ophthalmologist, radiation therapist, and surgeon, as well as consideration of the risk of relapse and possible complications of the treatment method.

The inflammatory activity and severity of TO should be diagnosed based on the investigator’s own experience and according to standard diagnostic criteria. Assessment of the inflammatory activity of TO can be performed using the clinical activity score (CAS) and using imaging methods — mainly MRI. The severity of TO is assessed using a seven-grade NOSPECS classification and a three-grade EUGOGO scale. In moderate to severe and active TO, i.v. methylprednisolone pulses are the treatment of choice. It is important to maintain the standard and regimen of treatment. The recommended standard as first-line treatment in most patients with moderate to severe and active TO is the combined use of methylprednisolone i.v. (cumulative dose of 4.5 g over 12 weeks) with concurrent administration of mycophenolate sodium 0.72 g per day for 24 weeks.

In more severe forms of moderate to severe and active TO, a higher cumulative dose of methylprednisolone i.v. is recommended as an alternative first-line treatment (7.5 g) as monotherapy starting with a dose of 0.75 g once a week for 6 weeks and 0.5 g for a further 6 weeks. EUGOGO guidelines recommend that in cases of no clinical response after 6 weeks of first-line treatment with i.v. methylprednisolone and mycophenolate, after 3–4 weeks, a second course of i.v. methylprednisolone monotherapy should be started with a higher cumulative dose of 7.5 g. Other second-line treatment options are orbital radiotherapy with or without oral or i.v. systemic glucocorticosteroid therapy, cyclosporine, or azathioprine in combination with p.o. glucocorticosteroid, methotrexate monotherapy, and a group of biologic drugs rituximab, tocilizumab, teprotumumab).

Keeping in mind that TO is a sight-threatening disease, we expect, through the treatment applied, to maintain full visual acuity, pain relief, single vision in the useful part of the visual field, and a positive cosmetic effect.

 

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Keywords

Graves’ disease; Basedow’s disease; thyroid orbitopathy; assessment of thyroid orbitopathy; first-line treatment; second-line treatment

About this article
Title

Optimization of the treatment of moderate to severe and active thyroid orbitopathy considering the recommendations of the European Group on Graves’ Orbitopathy (EUGOGO) [Optymalizacja leczenia umiarkowanej do ciężkiej i aktywnej orbitopatii tarczycowej z uwzględnieniem zaleceń European Group on Graves’ Orbitopathy (EUGOGO)]

Journal

Endokrynologia Polska

Issue

Vol 73, No 4 (2022)

Article type

Review paper

Pages

756-777

Published online

2022-07-18

Page views

4262

Article views/downloads

1976

DOI

10.5603/EP.a2022.0040

Pubmed

36059167

Bibliographic record

Endokrynol Pol 2022;73(4):756-777.

Keywords

Graves’ disease
Basedow’s disease
thyroid orbitopathy
assessment of thyroid orbitopathy
first-line treatment
second-line treatment

Authors

Mariusz Nowak
Bogdan Marek
Beata Kos-Kudła
Lucyna Siemińska
Magdalena Londzin-Olesik
Joanna Głogowska-Szeląg
Wojciech Nowak
Dariusz Kajdaniuk

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