In contrast, 33 patients were diagnosed as having IgG4-RKD during the clinical course of IgG4-related disease. Of these, 20 patients were incidentally detected when systemic examination for IgG4-related disease was performed through radiographic examination. Thirteen patients were suspected of having renal disease because of newly noted renal dysfunction. Table 1 Clinical and pathological characteristics of 41 patients Characteristics The number of casesa (%) Age (years) 63.7 ± 12.3 Male sex [no. (%)] 30 (73.2) Patients with preceding IgG4-RD [no. (%)] 33 (80.5) Clue to detect IgG4-RKD with preceding IgG4-RD VX-680 clinical trial [no./total no. (%)] Incidentally detected
during systemic examination for IgG4-RD 20/33 (60.6) Newly noted renal dysfunction 13/33 (39.4) Clue to detect IgG4-RKD without preceding IgG4-RD [no./total no. (%)] Decreased kidney function 4/8 (50.0) Radiographic abnormalities 2/8 (25.0) Urinary abnormalities 1/8 (12.5) Urinalysis and serological features Proteinuria [no./total no. (%)] 3+ 1/36 (2.8) 2+ 6/36 (16.7) 1+ 11/36 (30.6)
± 3/36 (8.3) Hematuria [no./total no. (%)] 3+ 1/36 (2.8) 2+ 2/36 (5.6) 1+ 9/36 (25.0) ± 3/36 (8.3) Elevated serum creatinine [no./total no. (%)] 24/41 (58.5) Serum creatinine level (mg/dl) 1.7 ± 1.5 Elevated serum IgG [no./total no. (%)] 37/41 STK38 (90.2) Serum IgG level (mg/dl) 3467.4 ± 1658.2 Serum IgG levels exceeding Selleck ATM Kinase Inhibitor 3000 mg/dl [no./total no. (%)] 21/41 (51.2) Hypocomplementemia [no./total no. (%)] 22/41 (53.7) Elevated serum IgE [no./total no. (%)] 26/33 (78.8) Serum IgE level (U/ml) 754.3 ± 876.8 Elevated serum IgG4 [no./total no. (%)] 41/41 (100.0) Serum IgG4 level (mg/dl) 991.2 ± 604.9 Imaging (CT) Contrast medium used [no./total no.
(%)] 29/41 (70.7) Multiple low-density lesions on enhanced CT [no./total no. (%)] 19/29 (65.5) Diffuse bilateral renal swelling on enhanced CT [no./total no. (%)] 1/29 (3.4) Diffuse bilateral renal swelling without enhanced CT [no./total no. (%)] 2/12 (16.7) Diffuse thickening of the renal pelvis wall [no./total no. (%)] 6/41 (14.6) Hypovascular solitary nodule [no./total no. (%)] 1/29 (3.4) Histology Patients with tubulointerstitial lesions [no./total biopsied no. (%)] 28/28 (100.0) Patients with glomerular lesions [no./total biopsied no. (%)] 11/28 (39.3) Other organ selleck kinase inhibitor involvement [no. (%)] Pancreas 13 (31.7) Salivary gland 29 (70.7) Lacrimal gland 12 (29.3) Lung 12 (29.3) Lymph node 17 (42.5) Retroperitoneum 4 (9.8) Prostate 3 (7.3) Periaortic area 2 (4.9) Breast, liver, nerve, thyroid gland, peritoneum, bile duct, or jointb 1 (2.4) IgG4-RD IgG4-related disease; IgG4-RKD IgG4-related kidney disease; no.