| 초록 |
Amyloidosis is a group of disorders in which soluble proteins aggregate and are deposited extracellularly in tissues as insoluble fibrils, causing progressive organ dysfunction. The kidney is one of the most frequent sites of amyloid deposition. Renal amyloidosis is a major cause of morbidity and without treatment, this usually progresses to end-stage renal disease. We report A 56-year-old woman, who was admitted to the nephrology clinic due to leg edema accompanied by nephrotic-range proteinuria, foamy urination, shortness of breath during activity, and ecchymosis on the skin. On physical examinations, her vital signs were stable. However, there was periorbital ecchymosis and decreased vesicular sounds on both lower parts of her lungs. Laboratory examination showed normochromic normocytic anemia, nephrotic-range proteinuria and creatinine levels of 2.2 mg/dL (eGFR 24.3 ml/min/1.73 m2). Renal ultrasound shows chronic renal parenchymal disease. Moreover, the Chest x-ray showed bilateral pleural effusion. Echocardiography showed grade 2 diastolic dysfunction, moderate pericardial effusion, moderate bilateral pleural effusion, and minimal ascites. Results from kidney, fat and pleural biopsies showed histologic features that were all consistent with amyloidosis. Moreover, the results of immunofixation showed IgA lambda monoclonality with free light chain lambda. She was then treated with furosemide, irbesartan 150 mg once daily and planned for administration of bortezomib, cyclophospamide and dexamethasone. In conclusion, amyloidosis is a rare disease with diagnostic difficulties, because of the unspecific early clinical manifestation of the disease. A careful approach, thorough examinations, as well as pathological biopsy expertise, are needed to find this rare disease in the patient, for early optimal management. |