A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

Higashioka, Kazuhiko; Yoshida, Kenji; Oryoji, Kensuke; Kamada, Kohei; Mizuki, Shinichi; Tsukamoto, Hiroshi; Yokota, Eisuke; Akashi, Koichi

doi:10.1155/2015/392893

Cited by 8 publications

(6 citation statements)

References 25 publications

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“…Although most patients present before multi-organ involvement is evident, Higashioka et al . reported a case of a patient who had 10 organs involved in IgG4-related disease at the time of his diagnosis 16 .…”

Section: Clinical Presentationmentioning

confidence: 99%

Recent advances in understanding and managing IgG4-related disease

Wolfson

Hamilos

2017

F1000Res

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IgG4-related disease was only recently discovered, so its description, management, and new discoveries related to its etiology are rapidly evolving. Because IgG4 itself is a unique antibody which is intimately related to the diagnosis of the disease, the role of plasmablasts in the pathophysiology remains an active area of discussion. Recent studies have uncovered a possible role for CD4-positive cytotoxic T lymphocytes, T follicular helper cells, and M2 macrophages. The clinical presentation is variable and can be vague, as this disease affects many organs and new presentations are continuing to be described. The diagnosis depends on clinical and histopathological assessment. The mainstay of treatment is with glucocorticoids, but rituximab has recently shown promise. Monitoring disease activity using imaging modalities (including positron emission tomography) and serum markers is imperative, as relapses are common. IgG4-related disease spans many medical disciplines but is a treatable condition with which all clinicians should be familiar.

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Section: Clinical Presentationmentioning

confidence: 99%

Recent advances in understanding and managing IgG4-related disease

Wolfson

Hamilos

2017

F1000Res

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“…Nodular lesions on CT dubbed as inflammatory pseudotumors may interfere with the correct differential diagnosis. We reviewed current literatures discussing IgG4-related multiple pseudotumors involvement [ 7 , 8 , 9 , 10 , 11 , 12 ], and discovered that the confusing metastatic-like appearance in the initial CT images were often the misleading reason for these reports (Table 1 ). PET/CT was not implemented in single case because they got final results depending on organ biopsies, fortunately.…”

Section: Discussionmentioning

confidence: 99%

IgG4-Related Pseudotumors Mimicking Metastases in Liver and Lungs

Yueh

Tung

2021

Case Rep Gastroenterol

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A 47-year-old man complained intermittent dull pain at the right upper quadrant abdomen and right neck swelling for 3 months. Blood tests revealed leukocytosis with mild eosinophilia. Computed tomography (CT) showed the presence of multiple nodules in the liver and both lung fields. Positron emission tomography/CT (PET/CT) scans found increased uptake at lymph nodes of the right neck, in the lung, liver, and prostate. The patient was diagnosed with IgG4-related disease (IgG4-RD) based on the biopsy findings from the right neck lymph nodes, showing enriched IgG4-positive lymphoplasmacytic cells. It is often difficult to distinguish IgG4-RD from malignancy, especially in presentations with multiple pseudotumors. This case serves as a reminder that IgG4-RD should be considered in earlier diagnosis, since pseudotumors in multiple organs may imitate tumor metastases.

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“…This diverse entity can affect nearly every organ and systemic manifestations usually occur [6] . Few cases of extensive multiorgan involvements have been published [7] , [8] . Isolated IgG4-pleuritis has been described in only two cases [9] , [10] , both with pleural effusion.…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related pleuritis – A case report

Gajewska

Rychwicka-Kielek

Sørensen

et al. 2016

Respiratory Medicine Case Reports

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Immunoglobulin G4-related disease (IgG4-RD) is a chronic disease that presents with inflammation and fibrosis of involved tissue. It encompasses several disorders previously described using different terms, but all disorders are characterised by IgG4-positive plasma cells and lymphocytes infiltration of tissues. We report a rare case of a 58-year-old man with IgG4-related pleural disease without other systemic manifestations. The diagnosis was based on characteristic changes on PET-CT and typical histopathology in a pleural specimen. The patient’s condition improved following immunosuppressive therapy.

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A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

Cited by 8 publications

References 25 publications

Recent advances in understanding and managing IgG4-related disease

Recent advances in understanding and managing IgG4-related disease

IgG4-Related Pseudotumors Mimicking Metastases in Liver and Lungs

Immunoglobulin G4-related pleuritis – A case report

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