open access

Vol 67, No 5 (2016)
Review article
Published online: 2016-10-31
Submitted: 2016-01-14
Accepted: 2016-03-14
Get Citation

Immunoglobulin G4 related thyroid disorders: Diagnostic challenges and clinical outcomes

Deep Dutta, Arvind Ahuja, Chitra Selvan
DOI: 10.5603/EP.2016.0061
·
Endokrynologia Polska 2016;67(5):520-524.

open access

Vol 67, No 5 (2016)
Review article
Published online: 2016-10-31
Submitted: 2016-01-14
Accepted: 2016-03-14

Abstract

Ig-G4 related disease (IgG4RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the start of this century only. It is currently one of the hottest areas of clinical and translational research across specialties. Thyroid involvement in IgG4RD is rare, believed to occur in less than 4% of cases, may be isolated or may be associated with other organ involvement. As of today Riedel’s thyroiditis, fibrosing variant of Hashimoto’s thyroiditis, and few patients of Graves’ orbitopathy represent the types of IgG4-related thyroid disease (IgG4RTD). This disorder is frequently confused with malignancy due to the intense sclerosis of thyroid resulting in hard texture on palpation compounded by often-associated compressive symptoms. Diagnosis involves establishing high circulating levels of IgG4 > 135 mg/dL, increased serum IgG4 to IgG ratio of > 8%, immunohistochemistry showing dense lymphoplasmacellular inflammatory infiltrate consisting of IgG4-positive plasma cells with storiform fibrosis and obliterative phlebitis, and increased IgG4 positive plasma cell > 10 cells per high-power field when at least three fields are evaluated. Glucocorticoids are the primary form of therapy in IgG4RD. However, their role in IgG4RTD needs to be evaluated. As of today levothyroxine supplementation for resulting hypothyroidism, appropriate management of Graves’ disease, and surgical excision of thyroid in case of compressive symptoms remain the primary treatment options. (Endokrynol Pol 2016; 67 (5): 520–524)

Abstract

Ig-G4 related disease (IgG4RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the start of this century only. It is currently one of the hottest areas of clinical and translational research across specialties. Thyroid involvement in IgG4RD is rare, believed to occur in less than 4% of cases, may be isolated or may be associated with other organ involvement. As of today Riedel’s thyroiditis, fibrosing variant of Hashimoto’s thyroiditis, and few patients of Graves’ orbitopathy represent the types of IgG4-related thyroid disease (IgG4RTD). This disorder is frequently confused with malignancy due to the intense sclerosis of thyroid resulting in hard texture on palpation compounded by often-associated compressive symptoms. Diagnosis involves establishing high circulating levels of IgG4 > 135 mg/dL, increased serum IgG4 to IgG ratio of > 8%, immunohistochemistry showing dense lymphoplasmacellular inflammatory infiltrate consisting of IgG4-positive plasma cells with storiform fibrosis and obliterative phlebitis, and increased IgG4 positive plasma cell > 10 cells per high-power field when at least three fields are evaluated. Glucocorticoids are the primary form of therapy in IgG4RD. However, their role in IgG4RTD needs to be evaluated. As of today levothyroxine supplementation for resulting hypothyroidism, appropriate management of Graves’ disease, and surgical excision of thyroid in case of compressive symptoms remain the primary treatment options. (Endokrynol Pol 2016; 67 (5): 520–524)

Get Citation

Keywords

immunoglobulin; Hashimoto’s thyroiditis; Riedel’s thyroiditis; Graves disease; IgG4 related disease

About this article
Title

Immunoglobulin G4 related thyroid disorders: Diagnostic challenges and clinical outcomes

Journal

Endokrynologia Polska

Issue

Vol 67, No 5 (2016)

Pages

520-524

Published online

2016-10-31

DOI

10.5603/EP.2016.0061

Bibliographic record

Endokrynologia Polska 2016;67(5):520-524.

Keywords

immunoglobulin
Hashimoto’s thyroiditis
Riedel’s thyroiditis
Graves disease
IgG4 related disease

Authors

Deep Dutta
Arvind Ahuja
Chitra Selvan

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