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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/IgG ratios for IgG4-RD (definite and possible IgG4-RD) and non-IgG4-RD subgroups. Median and interquartile range (25% to 75%) are depicted.
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fig2: Serum IgG4/IgG ratios for IgG4-RD (definite and possible IgG4-RD) and non-IgG4-RD subgroups. Median and interquartile range (25% to 75%) are depicted.

Mentions: Twenty-nine patients (18.4%) met the criteria for definite or possible IgG4-RD (Table 2). The mean age of those with IgG4-RD was older compared to those without IgG4-RD (58.3 ± 16.9 versus 49.9 ± 20.8, P < 0.05) but the gender distribution was not different (P = 0.29). The serum IgG4 level was significantly higher in those with IgG4-RD compared to those with non-IgG4-RD diagnoses (P < 0.001) (Table 1). Furthermore, mean serum IgG4 level was higher for those with definite IgG4-RD (940 ± 990) compared to possible IgG4-RD (329 ± 318) which, in turn, was higher compared to non-IgG4-RD subgroup (226 ± 127) (P < 0.05) (Figure 1). The mean serum IgG4/IgG ratio was significantly higher in patients with IgG4-RD (definite and possible) compared to non-IgG4-RD patients (0.263 ± 0.239 versus 0.148 ± 0.061, P < 0.01), but there was substantial overlap in individual values between the two groups as shown in Figure 2. Among 29 patients with IgG4-RD, 10 patients met the criteria for definite IgG4-RD which included supportive histopathologic findings on tissue biopsy. Of 19 patients with possible IgG4-RD, 12 had undergone biopsy procedures but the biopsy findings did not meet the criteria for definite or probable IgG4-RD diagnosis.


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/IgG ratios for IgG4-RD (definite and possible IgG4-RD) and non-IgG4-RD subgroups. Median and interquartile range (25% to 75%) are depicted.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3368193&req=5

fig2: Serum IgG4/IgG ratios for IgG4-RD (definite and possible IgG4-RD) and non-IgG4-RD subgroups. Median and interquartile range (25% to 75%) are depicted.
Mentions: Twenty-nine patients (18.4%) met the criteria for definite or possible IgG4-RD (Table 2). The mean age of those with IgG4-RD was older compared to those without IgG4-RD (58.3 ± 16.9 versus 49.9 ± 20.8, P < 0.05) but the gender distribution was not different (P = 0.29). The serum IgG4 level was significantly higher in those with IgG4-RD compared to those with non-IgG4-RD diagnoses (P < 0.001) (Table 1). Furthermore, mean serum IgG4 level was higher for those with definite IgG4-RD (940 ± 990) compared to possible IgG4-RD (329 ± 318) which, in turn, was higher compared to non-IgG4-RD subgroup (226 ± 127) (P < 0.05) (Figure 1). The mean serum IgG4/IgG ratio was significantly higher in patients with IgG4-RD (definite and possible) compared to non-IgG4-RD patients (0.263 ± 0.239 versus 0.148 ± 0.061, P < 0.01), but there was substantial overlap in individual values between the two groups as shown in Figure 2. Among 29 patients with IgG4-RD, 10 patients met the criteria for definite IgG4-RD which included supportive histopathologic findings on tissue biopsy. Of 19 patients with possible IgG4-RD, 12 had undergone biopsy procedures but the biopsy findings did not meet the criteria for definite or probable IgG4-RD diagnosis.

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