A case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis)

doi:10.1016/j.humpath.2005.11.001

Human Pathology

Volume 37, Issue 2, February 2006, Pages 239-243

https://doi.org/10.1016/j.humpath.2005.11.001 Get rights and content

Summary

There is now increasing evidence that IgG4 is closely involved in idiopathic sclerosing lesions, such as sclerosing pancreatitis and sclerosing sialadenitis. In this report, we describe a case of IgG4-related retroperitoneal and mediastinal fibroses. A 52-year-old man presented with dull back pain and was found to have a continuously surrounding paraaortic mass. A biopsy specimen taken from the retroperitoneum showed a diffuse lymphoplasmacytic infiltration intermixed with fibrosis. Many IgG4-positive plasma cells were demonstrated on immunostaining. His serum IgG4 concentration was 392 mg/dL (reference range, <70). We treated this patient with a corticosteroid, which markedly diminished the paraaortic mass along with lowering of his serum IgG4 concentration. The possible involvement of IgG4 was suggested in the pathogeneses of retroperitoneal and mediastinal fibroses in this patient. IgG4 might be useful in the clinical management of retroperitoneal or mediastinal fibrosis to differentiate them from malignant tumors and predict steroid sensitivity.

Introduction

Idiopathic sclerosing lesions can occur in various organs, such as the retroperitoneum (retroperitoneal fibrosis), mediastinum (mediastinal fibrosis), bile duct (sclerosing cholangitis), salivary gland (sclerosing sialadenitis), and pancreas (sclerosing pancreatitis). Some cases are associated with multiple sclerosing lesions in different organs, suggesting the possible involvement of common etiologies in their pathogenesis. These idiopathic sclerosing lesions have been suggested to be heterogeneous disease entities. Steroid therapy has a beneficial effect in some cases, whereas it is ineffective in others.

In 2001, a close association between IgG4 and the idiopathic sclerosing lesion was first reported with regard to sclerosing pancreatitis [1], which is also called autoimmune pancreatitis or lymphoplasmacytic sclerosing pancreatitis [2], [3]. Patients with sclerosing pancreatitis have a high serum IgG4 concentration, and many IgG4-positive plasma cells are histologically evident in the pancreases of these patients [1], [4]. Sclerosing pancreatitis is effectively treatable with corticosteroids, and the serum IgG4 level is usually lowered, reflecting lesional activity after corticosteroid therapy [1]. In addition, there is increasing evidence that IgG4 is also involved in the pathogenesis of the extrapancreatic sclerosing lesion, such as sclerosing cholangitis or sclerosing sialadenitis [5], [6], [7], [8]. We suggested that IgG4 is involved in the pathogenesis of some idiopathic sclerosing lesions, both in the pancreas and extrapancreatic tissues and that IgG4 might become a useful marker for diagnosis and a predictive marker for corticosteroid sensitivity in these cases.

In this report, we describe a case with IgG4-related retroperitoneal and mediastinal fibroses effectively treated with corticosteroid therapy.

Section snippets

Materials and methods

Formalin-fixed and paraffin-embedded specimens were prepared and used for histopathological and immunohistochemical studies. Sections measuring 4 μm were cut for routine hematoxylin and eosin, elastica van Gieson, and immunohistochemical stainings.

Immunostainings of CD3, CD20, and CD79α were performed by an autostainer (HX System Benchmark, Ventana Medical Systems, Tucson, Ariz), as per the manufacturer's instructions. Primary antibodies of CD3, CD20, and CD79α were from Ventana Medical Systems

Case report

A 52-year-old man was admitted to our hospital with a history of dull back pain and low-grade fever (37.0°C). He had been previously healthy and had no history of serious diseases. On examination, the back pain was located in the perispinal area, without tenderness. On laboratory examination, his serum C-reactive protein was 9.3 mg/dL (reference range, 0.0-0.4); total protein, 8.4 g/dL (6.5-8.2), γ-globulin, 2.0 g/dL (0.7-1.6); IgG, 1765 mg/dL (820-1740); IgA, 250 mg/dL (90-400); and IgM, 57

Discussion

The involvement of IgG4 in the pathogenesis of idiopathic sclerosing lesions was first reported with regard to sclerosing pancreatitis [1]. After this, there has been increasing evidence of the possible involvement of IgG4 in the pathogenesis of extrapancreatic sclerosing lesions. We reported on sclerosing cholangitis, sclerosing sialadenitis (Küttner tumor), and inflammatory pseudotumor of the breast, in all of which, many IgG4-positive plasma cells were histologically evident [5], [6], [7].

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Case reportA case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis)

Summary

Introduction

Section snippets

Materials and methods

Case report

Discussion

Hum Path

Hum Path

J Dermatol Sci

Gastroenterology

J Urol

High serum IgG4 concentrations in patients with sclerosing pancreatitis

N Engl J Med

Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis

Dig Dis Sci

Case report
A case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis)