Abstract
BackgroundThe aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan.MethodsVarious specialists retrospectively evaluated IgG4-RD patients diagnosed between 1996 and 2015 in five hospitals by analyzing their baseline clinical features, laboratory, imaging, and pathological test findings, and treatment.ResultsOf the 334 patients listed, 205 were male and median age at diagnosis was 65 years. The mean number of organs involved was 3.2 at diagnosis. The most frequently affected organs were the salivary glands, followed by the lacrimal glands, lymph nodes, pancreas, retroperitoneum/periaorta, kidneys, and lungs. The mean serum level of IgG4 was 755 mg/dl, and more than 95% of patients had elevated serum IgG4 levels. The median serum level of C-reactive protein (CRP) was 0.1 mg/dl and the level was less than 1 mg/dl in 90% of patients. A total of 34.7% of patients had low serum levels of C3. Serum levels of C3 and non-IgG4 IgG, calculated as the total IgG minus IgG4, showed an inverse correlation in patients with kidney lesions, while serum IgG4 levels were not correlated with serum C3 levels. Corticosteroid was administered in 78.0% of patients, and was effective in all.ConclusionsThe serum CRP level is generally low and the serum IgG4 level is elevated in most Japanese IgG4-RD patients, in contrast to western patients. These original findings suggest that these two parameters in IgG4-RD differ in some interesting ways from those hitherto reported in western populations. Additional studies, especially international comparative ones, are needed to elucidate the extent and significance of these differences between populations. Attention will also have to be paid to whether the existence of such differences requires consideration when devising international classification criteria.
Highlights
The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of Immunoglobulin G4 (IgG4)-RD in Japan
Inclusion criteria To minimize bias regarding the organs affected by IgG4RD, the diagnosis of IgG4-RD was made by specialists of IgG4-RD in each institution, supported by the comprehensive diagnostic criteria (CDC) [12] and/or criteria of each organ including type 1 autoimmune pancreatitis, IgG4-related kidney disease, IgG4-related Mikulicz’s disease (IgG4-related sialadenitis and dacryoadenitis), and IgG4-related sclerosing cholangitis [6, 13,14,15]
Colon, prostate, and renal cancers were frequently seen both before and after the diagnosis of IgG4-RD, whereas gastric cancer and malignant lymphoma tended to appear after the diagnosis of IgG4-RD (Table 2)
Summary
The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan. Several larger cohort studies have sought to characterize the clinical characteristics of IgG4-RD of over 100 patients [9,10,11] These reports documented the baseline clinical features of IgG4-RD, differences were seen in the frequency of the affected organs and some laboratory data possibly due to different inclusion criteria, authors’ specialty, study design, and racial biases. This prompted us to conduct a large-scale multicenter study with well-experienced physicians of IgG4-RD including rheumatologists, gastroenterologists, pulmonologists, and nephrologists to clarify the baseline clinical and laboratory features of IgG4-RD in a large-scale cohort of 334 IgG4-RD patients
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