Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis

Sah, Raghuwansh P.; Chari, Suresh T.

doi:10.1097/bor.0b013e3283413469

Cited by 307 publications

(236 citation statements)

References 82 publications

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“…stated that histological data are the mainstay for diagnosis, since both elevated numbers of IgG4-positive plasma cells and IgG4/IgG ratios have been described in other inflammatory conditions and malignancies (11). A high serum IgG4 concentration is often present but approximately 20-30% of patients with classic histopathological and immunochemical findings of the disease have normal serum levels (12). Therefore, it is not mandatory for the diagnosis of IgG4-related conditions.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related Hashimoto’s thyroiditis – A new variant of a well known disease

Luiz

Gonçalves

Silva

et al. 2014

Arq Bras Endocrinol Metab

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SUMMARYHashimoto's thyroiditis (HT) has been characterized for many years as a well-defined clinicopathologic entity, but is now considered a heterogeneous disease. IgG4-related HT is a new subtype characterized by thyroid inflammation rich in IgG4-positive plasma cells and marked fibrosis. It may be part of the systemic IgG4-related disease. We report a case of a 56-year-old Portuguese man who presented with a one-month history of progressive neck swelling and dysphagia. Laboratory testing revealed increased inflammatory parameters, subclinical hypothyroidism and very high levels of thyroid autoantibodies. Cervical ultrasound (US) demonstrated an enlarged and heterogeneous thyroid gland and two hypoechoic nodules. US-guided fine needle aspiration cytology was consistent with lymphocytic thyroiditis. The patient was submitted to total thyroidectomy and microscopic examination identified typical findings of HT, marked fibrosis limited within the thyroid capsule and lymphoplasmacytic infiltration, with > 50 IgG4-positive plasma cells per high-power field and an IgG4/ IgG ratio of > 40%. After surgery, serum IgG4 concentration was high-normal. Symptoms relief and reduction in laboratory inflammatory parameters were noticed. Thyroid function is controlled with levothyroxine. To our knowledge we report the first case of IgG4-related HT in a non-Asian patient. We also perform a review of the literature regarding IgG4-related disease and IgG4-related HT. Our case highlights this new variant of the well known HT, and helps physicians in recognizing its main clinical features, allowing for proper diagnosis and treatment. Arq Bras Endocrinol Metab. 2014;58(8):862-8 SUMÁRIO A tireoidite de Hashimoto (TH) foi caracterizada durante muitos anos como uma entidade clinicopatológica bem definida, mas é atualmente considerada uma patologia heterogênea. A TH associada a IgG4 apresenta-se como um novo subtipo, sendo caracterizada por inflamação da tireoide com numerosos plasmócitos IgG4-positivos e fibrose extensa. É possível que pertença ao espectro da doença sistêmica associada a IgG4. Relatamos o caso de um homem português de 56 anos que se apresentou com aumento progressivo do volume cervical e disfagia, com um mês de evolução. A avaliação laboratorial revelou elevação dos parâmetros inflamatórios, hipotireoidismo subclínico e níveis muito elevados de autoanticorpos tireoidianos. Por ultrassonografia cervical demonstrou-se tireoide aumentada, heterogênea, com dois nódulos hipoecoicos. Foi realizada citologia aspirativa com agulha fina guiada por ultrassom, compatível com tireoidite linfocítica. O doente foi submetido à tireoidectomia total e o exame histológico revelou achados típicos de TH, extensa fibrose localizada dentro da cápsula tireoidiana e infiltrado linfoplasmocitário, com > 50 plasmócitos IgG4-positivos por campo de grande ampliação e uma relação IgG4/IgG > 40%. Após cirurgia, a concentração sérica de IgG4 encontrava-se no limite superior do normal. Ocorreu melhoria sintomática e redução dos parâmetros inflamatór...

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Section: Discussionmentioning

confidence: 99%

IgG4-related Hashimoto’s thyroiditis – A new variant of a well known disease

Luiz

Gonçalves

Silva

et al. 2014

Arq Bras Endocrinol Metab

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“…IgG4 is a rare subclass of IgG, normally accounting for 3% to 6% of total serum IgG [2]. Increase in serum IgG4 levels (>135 mg/dl) is typically associated with a pathologic process, though serum elevation is not independently specific for IgG4-related systemic disease [5], nor do all patients with IgG4-related disease demonstrate increased serum IgG4 [3]. Elevated IgG4 is often accompanied by other markers, such as increased serum IgE and the presence of antinuclear antibodies.…”

Section: Introductionmentioning

confidence: 99%

Perifollicular granulomatous inflammation in reactive lymph nodes: a possible morphologic marker for IgG4 plasmacytosis

et al. 2011

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“…Most patients with IgG4-RD have elevated serum IgG4 concentrations, but the levels vary widely. In Japan, 95% of patients exhibit elevated serum IgG4 concentrations (Hamano et al 2001), while approximately 30% of patients in the United States have normal concentrations, despite exhibiting characteristic histopathological and immunohistochemical findings (Sah and Chari 2011). Our patient initially had a slightly elevated IgG4 concentration (115 mg/ dL) postoperatively, but this increased gradually over time.…”

Section: Discussionmentioning

confidence: 86%

Chronic Sclerosing Sialadenitis of the Submandibular Gland as the Initial Symptom of IgG4-Related Disease: A Case Report

Tanaka

Harada

Kayamori

et al. 2015

Tohoku J. Exp. Med.

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Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition accompanied by tumefactive lesions, dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis in various organs, and, frequently, elevated serum IgG4 levels. Chronic sclerosing sialadenitis (also termed Küttner's tumor) is thought to be a lesion of IgG4-RD; thus, IgG4-related sialadenitis may be the initial symptom of IgG4-RD. We herein report a 64-year-old Japanese female with IgG4-related chronic sclerosing sialadenitis of the right submandibular gland and retroperitoneal fibrosis, who subsequently developed tubulointerstitial nephritis and pancreatitis. She was referred to our Department for treatment of swelling of the right submandibular gland; preoperative imaging studies suggested a malignant tumor. We extirpated the submandibular glands bilaterally and diagnosed IgG4-related chronic sclerosing sialadenitis pathologically. Subsequently, the patient's serum IgG4 concentration increased, and lesions in the retroperitoneum, kidney, and pancreas were confirmed by imaging. Although the radiological characteristics of these lesions mimicked malignancy, steroid treatment was commenced based on the pathology of the submandibular gland and elevated serum IgG4 level. This caused the lesions to disappear, indicating that the patient had experienced IgG4-related retroperitoneal fibrosis, tubulointerstitial nephritis, and pancreatitis. No relapse was detected for 4 years 8 months after surgery. A pathological diagnosis is crucial to exclude the possibility of malignancy and to make treatment decisions when lesions are evident in other organs. In addition, periodic evaluation of the serum IgG4 concentration and imaging of the whole body are warranted in long-term follow-up.

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Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis

Abstract: No single serologic marker is diagnostic of ISD. Serum IgG4 elevation has convincing diagnostic utility when combined with other disease features although its value in disease monitoring may be limited.

Cited by 307 publications

References 82 publications

IgG4-related Hashimoto’s thyroiditis – A new variant of a well known disease

IgG4-related Hashimoto’s thyroiditis – A new variant of a well known disease

Perifollicular granulomatous inflammation in reactive lymph nodes: a possible morphologic marker for IgG4 plasmacytosis

Chronic Sclerosing Sialadenitis of the Submandibular Gland as the Initial Symptom of IgG4-Related Disease: A Case Report

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