The research was conducted in compliance with the Declaration of Helsinki. Clinical features The clinical picture including symptoms resulting from other organ involvement such as the pancreas, lacrimal and salivary glands, or lungs was noted. Diagnostic Apoptosis inhibitor clues to IgG4-RKD were carefully evaluated, and important items were extracted. Serum IgG, IgG4, IgE, and complement levels were collected

from the clinical data file. Serum creatinine (Cr) levels and any abnormalities of urinalysis including proteinuria and hematuria before corticosteroid therapy were noted in all cases. Urine N-acetyl-β-d-glucosaminidase and urine β2-microglobulin levels were also noted if available. Imaging CT was the most recommended radiographic imaging method for IgG4-RKD. In general, contrast-enhanced CT was needed to make the correct diagnosis; however, the use of contrast medium required careful judgment in patients selleckchem with impaired renal function. Without enhancement, diffuse enlargement of the kidney inconsistent with the degree of renal function was noted. Other modalities including gallium scintigraphy, magnetic resonance imaging, and fluorodeoxyglucose positron emission tomography were additionally used to identify renal lesions. Histology and immunostaining

Renal histology was available in 28 patients. Bouin’s fluid-fixed or formalin-fixed and paraffin-embedded renal specimens of patients with IgG4-RKD were analyzed, and the degree of lymphoplasmacytic infiltration in the interstitium, degree of fibrosis, eosinophilic infiltration, and glomerular lesions were recorded. In immunostaining, immunofluorescence was performed against IgG, IgA, IgM, C3, C1q, and fibrinogen. Immunostaining was performed using mouse monoclonal antibody against human

IgG4 (Zymed Laboratories, San Francisco, CA, USA, or The Binding Site, Birmingham, UK), anti-human IgG (Dako, Glostrup, Denmark), and/or anti-human CD138 (AbD Serotec, Oxford, UK). Diagnostic algorithm and criteria We first analyzed 41 cases of IgG4-RKD, the preliminary diagnosis of which was made based on the clinical decision of observers who had sufficient experience with IgG4-related disease including ZD1839 cell line AIP. To select the most sensitive and specific test for the diagnosis of IgG4-RKD, we referred to the revised clinical diagnostic criteria for AIP proposed by Okazaki et al. [12] and Mayo Clinic criteria for AIP proposed by Chari et al. [13]. On the basis of these analyses, a diagnostic algorithm and criteria were BI 10773 concentration prepared. Results Clinical features Table 1 summarizes clinical and histological characteristics of the 41 patients. The mean age of the 41 patients was 63.7 years (range 27–83). The ratio of male to female patients was 30:11. Eight patients without preceding IgG4-related disease were suspected to have renal disease because of decreased kidney function (n = 4), radiographic abnormalities (n = 2) and/or urinary abnormalities (n = 1).