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IgG4-Related Disease: A Multispecialty Condition
Iuri Usêda Santana1, Emanuela Pimenta da Fonseca2, Mittermayer Barreto Santiago2
1Serviço de Clínica Médica do Hospital Geral Roberto Santos, Rua Direta do Saboeiro s/n, Cabula, 41180-900 Salvador, BA, Brazil.
Case Reports in Rheumatology
|December 16, 2014
Summary
Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition affecting multiple organs. This case report details a patient with IgG4-RD, emphasizing its inclusion in multisystem disease differential diagnoses.
Area of Science:
- Immunology
- Rheumatology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a recently identified systemic condition.
- It is characterized by organ swelling, IgG4-positive plasma cell infiltration, fibrosis, and elevated serum IgG4.
- IgG4-RD can affect multiple organs and tissues throughout the body.
Purpose of the Study:
- To report a case of IgG4-related disease in a 34-year-old male.
- To highlight the diverse clinical manifestations of IgG4-RD.
- To emphasize the importance of considering IgG4-RD in the differential diagnosis of multisystem diseases.
Main Methods:
- Case report of a 34-year-old male patient.
- Clinical evaluation including history, physical examination, and laboratory tests.
- Immunohistochemical staining of a maxillary biopsy for IgG4-related disease confirmation.
- Assessment of treatment response to corticosteroids.
Main Results:
- The patient presented with diffuse abdominal pain, weight loss, neck stiffness, a history of maxillary mass, palpebral ptosis, and chronic cough.
- Laboratory findings included polyclonal hypergammaglobulinemia and elevated serum IgG4 levels.
- Maxillary biopsy confirmed IgG4-related disease, with excellent response to corticosteroid therapy.
Conclusions:
- IgG4-related disease can present with a wide range of symptoms affecting multiple organ systems.
- Elevated serum IgG4 levels and characteristic histopathology are key diagnostic markers.
- Corticosteroid therapy can be highly effective in managing IgG4-RD.
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