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Pol Merkur Lekarski. 2017 Sep 29;43(255):129-132.

[IgG4-related disease - a case report].

[Article in Polish]

Author information

1
Medical University of Warsaw: Department of Internal Medicine and Endocrinology.
2
Medical University of Warsaw: Department of Pathology.
3
Department and Out- Patients Clinic of Systemic Diseases of Connective Tissue, National Institute of Geriatrics, Rheumatology and Rehabilitation in Warsaw.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a comparatively new condition that may involve more than one organ. The lack of characteristic, pathognomonic clinical symptoms may delay the diagnosis of this disease. The diagnosis is based upon clinical manifestation, elevated serum levels of IgG4 and histopathologic examination with immunohistochemical staining to reveal infiltration of IgG4-positive plasma cells. The first line treatment is oral glucocorticoids.

A CASE REPORT:

38-year-old woman with Hashimoto disease, chronic sinusitis and chronic hepatitis of unknown etiology was admitted to the Department of Endocrinology because of moderate eyelids swelling accompanied by redness for 3 years. Graves' orbitopathy and systemic vasculitis were suspected, however both were excluded (negative antibodies results: anty-TSHR, ANCA, ANA). Serologic investigation of Sjögren's syndrome was also negative. In Magnetic Resonance Imaging (MRI) of orbits there were described bilateral mild extension of lateral rectus muscles, normal signal of adipose tissue and bilateral lacrimal glands enlargement. Moreover, increased IgG4 serum levels were detected. The material derived from perinasal sinuses surgery was analyzed in histopathology examination with immunohistochemical staining, which revealed characteristic features of chronic inflammatory process and increased numbers of IgG4 - positive plasma cells (>50 in a large field of view). The diagnosis of IgG4-RD was established. Because of non-effective oral methylprednisolone therapy in the past, the patient was referred to Clinic of Rheumatology for further treatment. After the therapy with methylprednisolone and azathioprine there were observed the significant reduction of symptoms.

CONCLUSIONS:

Because of lack of characteristic symptoms of IgG4- RD, it should be always considered in differential diagnosis of chronic inflammatory diseases of various organs.

KEYWORDS:

Hashimoto disease; IgG4-related disease; chronic sinusitis; inflammation of lacrimal glands; orbitopathy

PMID:
28987046
[Indexed for MEDLINE]

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