Detection of Serum IgG4 Levels in Patients with IgG4-Related Disease and Other Disorders

Su, Yuying; Sun, Wei; Wang, Chenqiong; Wu, Xiaoqian; Miao, Yi; Xiong, Hui; Bai, Lin; Dong, Lingli

doi:10.1371/journal.pone.0124233

Cited by 30 publications

(23 citation statements)

References 25 publications

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“…8 Serum IgG4 levels are not specific markers for IgG4-RD, as they also increase in cancerous, allergic, and autoimmune disorders. 6,9,10 The specificity and positive predictive value of the serum IgG4 for IgG4-RD are 60 and 34% respectively. 11…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related hypertrophic pachymeningitis

Levraut

Cohen

Bresch

et al. 2019

Neurol Neuroimmunol Neuroinflamm

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ObjectiveMeningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP).MethodsTwo IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.ResultsForty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.Discussion/conclusionIgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.

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Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related hypertrophic pachymeningitis

Levraut

Cohen

Bresch

et al. 2019

Neurol Neuroimmunol Neuroinflamm

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“…As increased serum concentrations of IgG4 have been observed in several diseases with aberrant immunological condition unrelated to IgG4-RD, such as malignant tumours, autoimmune diseases especially rheumatoid arthritis and allergic diseases,9 10 increased IgG4 concentration is not a specific marker for IgG4-RD. In contrast, recent large cohort studies from the UK, Taiwan and Japan showed that serum IgG4 concentration >135 mg/dL had overall sensitivities of 82.8%, 86% and 88%, respectively, in diagnosing IgG4-RD 10–12.…”

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confidence: 99%

How to diagnose IgG4-related disease

et al. 2017

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“…[5–7] However, tissues from deep organs such as pancreas, retroperitoneum, kidney, ocular cavity, and periaorta are difficult to perform biopsy. In addition, IgG4-RD mainly affects middle-aged to elderly men who often have comorbidities, which places them at risk for complications.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related disease involving vital organs diagnosed with lip biopsy

et al. 2016

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Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized new disease entity characterized by elevated serum IgG4 and infiltration of IgG4+ plasma cells in affected tissues. Histological examination is essential for definitive diagnosis, as other pathological conditions can also present with serum IgG4 elevation. However, IgG4-RD frequently involves vital or internal organs that are difficult to perform biopsies. We herein report a unique case of IgG4-RD involving vital organs that could be successfully diagnosed by alternative lip biopsy, an accessible, little invasive procedure, despite no apparent manifestation demonstrating the involvement in labial salivary gland.A 60-year-old man with swelling of both submandibular glands and elevated serum creatinine level visited our hospital. His labial salivary glands appeared normal. His blood test showed high serum IgG4, and positron-emission computed tomography revealed abnormal uptake in submandibular glands, periaorta, and left kidney with hydronephrosis. We suspected him of IgG4-RD; however, the involved organs were difficult to approach for histological examination. Alternatively, we performed lip biopsy and proved massive infiltration of IgG4+ plasma cells leading to the diagnosis with IgG4-RD. Treatment with prednisolone resulted in the remarkable improvement of organ involvements and the normalization of serum IgG4 level after 3 months. Prednisolone was gradually tapered without the relapse of disease.The early recognition and diagnosis of IgG4-RD is clinically important because delay in the treatment initiation leads to fibrosis with irreversible organ damage. Our case highlights the possibility that lip biopsy is a promising option for histological examination in patients with IgG4-RD in whom affected organs are difficult to access, leading to early diagnosis with appropriate treatment.

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Detection of Serum IgG4 Levels in Patients with IgG4-Related Disease and Other Disorders

Cited by 30 publications

References 25 publications

Immunoglobulin G4-related hypertrophic pachymeningitis

Immunoglobulin G4-related hypertrophic pachymeningitis

How to diagnose IgG4-related disease

IgG4-related disease involving vital organs diagnosed with lip biopsy

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