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Spectrum of Disorders Associated with Elevated Serum IgG4 Levels Encountered in Clinical Practice.

Ryu JH, Horie R, Sekiguchi H, Peikert T, Yi ES - Int J Rheumatol (2012)

Bottom Line: Twenty-nine patients (18.4%) had definite or possible IgG4-RD.Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed.We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Gonda 18 South, 200 First St. SW, Rochester, MN 55905, USA.

ABSTRACT
IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice. Among 3,300 patients who underwent IgG subclass testing over a 2-year period from January 2009 to December 2010, 158 (4.8%) had an elevated serum IgG4 level (>140 mg/dL). IgG4 subclass testing was performed for evaluation of suspected IgG4-RD or immunodeficiency. Twenty-nine patients (18.4%) had definite or possible IgG4-RD. Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed. We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

No MeSH data available.


Related in: MedlinePlus

Serum IgG4 levels for patients with single-organ and multiple-(two or more) organ involvement. Median and interquartile range (25% to 75%) are depicted.
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fig3: Serum IgG4 levels for patients with single-organ and multiple-(two or more) organ involvement. Median and interquartile range (25% to 75%) are depicted.

Mentions: Pattern of organ involvement for these patients with IgG4-RD is outlined in Table 3. Pancreas, bile ducts, and orbital structures were most commonly involved. However, these patients exhibited involvement in a number of other organs including the salivary glands, retroperitoneal region, lymph nodes, kidney, lung, pleura, sinuses, gastrointestinal tract, and testis. Patients with two or more organ involvements (n = 13) had a higher mean serum IgG4 level (840 ± 914) compared to those with single organ involvement (296 ± 250) (P < 0.01) (Figure 3).


Spectrum of Disorders Associated with Elevated Serum IgG4 Levels Encountered in Clinical Practice.

Ryu JH, Horie R, Sekiguchi H, Peikert T, Yi ES - Int J Rheumatol (2012)

Serum IgG4 levels for patients with single-organ and multiple-(two or more) organ involvement. Median and interquartile range (25% to 75%) are depicted.
© Copyright Policy - open-access
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC3368193&req=5

fig3: Serum IgG4 levels for patients with single-organ and multiple-(two or more) organ involvement. Median and interquartile range (25% to 75%) are depicted.
Mentions: Pattern of organ involvement for these patients with IgG4-RD is outlined in Table 3. Pancreas, bile ducts, and orbital structures were most commonly involved. However, these patients exhibited involvement in a number of other organs including the salivary glands, retroperitoneal region, lymph nodes, kidney, lung, pleura, sinuses, gastrointestinal tract, and testis. Patients with two or more organ involvements (n = 13) had a higher mean serum IgG4 level (840 ± 914) compared to those with single organ involvement (296 ± 250) (P < 0.01) (Figure 3).

Bottom Line: Twenty-nine patients (18.4%) had definite or possible IgG4-RD.Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed.We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Gonda 18 South, 200 First St. SW, Rochester, MN 55905, USA.

ABSTRACT
IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice. Among 3,300 patients who underwent IgG subclass testing over a 2-year period from January 2009 to December 2010, 158 (4.8%) had an elevated serum IgG4 level (>140 mg/dL). IgG4 subclass testing was performed for evaluation of suspected IgG4-RD or immunodeficiency. Twenty-nine patients (18.4%) had definite or possible IgG4-RD. Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed. We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

No MeSH data available.


Related in: MedlinePlus