英文摘要 |
This 67-year-old man had been in his usual health until 2 monthsbefore this presentation, when impaired renal function was told inthe health examination, with serum creatinine level 1.7 mg/dL andBUN level 29 mg/dL. The patient reported no fevers, back pain,legs edema, or weight loss. He reported the family history of lungcancer and systemic lupus erythematosus, his father and niece,respectively. Urinalysis yielded no proteinuria, hematuria, cast orcrystal. Chest radiography showed a ill-defined opacities in rightlower lung field. Renal sonography revealed negative finding, andright kidney is 10.14 cm in diameter, left 10.30 cm. Computedtomography (CT) of the brain, neck, chest, abdomen and pelviswith administration of contrast material revealed spiculatedperifissural infiltrative soft tissue in the right lower lobe (Fig. 1)and heterogeneous enhancement at both kidneys. (Fig. 2) SCC andCEA level were both within normal limit. Bronchoscopy yieldedno endobronchial lesion, and endobronchial ultrasound revealedno peribronchial lesion. CT-guided biopsy of lung tumor showedno evidence of malignancy. VATS wedge resection of right lowerlung tumor was performed smoothly. Pathology reported evidentstoriform fibrosis, obliterative phlebitis, and chronic sclerosinginflammation with plasma cell infiltrate. IgG4+ plasma cells wereup to 226/HPF, and IgG4/IgG ratio of plasma cells was more than95%. High serum IgG4 level of 2390 mg/dL was noted. Highlipase level of 381 U/L and high amylase level of 300 U/L werefound. MRI of abdomen with administration of contrast materialrevealed marked swelling of pancreatic head with decreasedenhancement. A diagnosis of IgG4-related disease with lung,kidney and pancreas involvement was made. Oral prednisolone,hydroxychloroquine, and azathioprine were administered.Improvement of renal function was noted in the outpatientdepartment follow-up.
|