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증례 : 신이식 환자에서 신부전을 동반한 신장 유육종증 1예
박혜린 ( Hae Lin Park ),반가영 ( Ga Young Ban ),배창범 ( Chang Bum Bae ),임현이 ( Hyun Ee Yim ),김흥수 ( Heung Soo Kim ),박인휘 ( In Whee Park ),신규태 ( Gyu Tae Shin ) 대한내과학회 2013 대한내과학회지 Vol.84 No.2
본 증례는 당뇨병성 신증으로 인한 신부전으로 신이식 후 발생한 신장 유육종증의 예로, 육아종성 간질성 신염에 의해 신기능 악화 소견을 보였으나 스테로이드에 치료 반응을 보였으며, 신이식 후 신기능 저하의 감별진단에 드물지만 신장 유육종증도 포함되어야 하겠다. Sarcoidosis, systemic inflammatory disease characterized by non-caseating granulomas, is rarely associated with renal failure in a kidney transplant. We report a 51-year-old woman with a kidney transplant who was diagnosed to have renal sarcoidosis. After 7 years of renal transplantation, the patient presented with relatively rapid deterioration of renal function and, subsequently, she underwent kidney transplant biopsy. Renal biopsy revealed interstitial nephritis with non-caseating granulomas compatible with granulomatous interstitial nephritis (GIN). She was also found to have granulomatous lymphadenitis and skin lesions. Diagnosis of sarcoidosis was made based on histopathologic findings, the high serum angiotensin converting enzyme level and exclusions of other causes of GIN including tuberculosis, ANCA associated glomerulonephritis and tubulointerstitial nephritis and uveitis syndrome. The patient was started on oral prednisolone, and subsequently her renal function improved. (Korean J Med 2013; 84:290-294)
전신홍반루푸스에 동반된 이차성 혈구포식성 림프조직구증 3예
임은수 ( Eunsoo Lim ),김영건 ( Young Geon Kim ),최원선 ( Won Sun Choi ),정유석 ( Yu Soek Jung ),한재호 ( Jae Ho Han ),배창범 ( Chang Bum Bae ),정주양 ( Ju Yang Jung ),김현아 ( Hyoun Ah Kim ),서창희 ( Chang Hee Suh ) 대한류마티스학회 2015 대한류마티스학회지 Vol.22 No.3
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hyperferritinemia, and phagocytosis of hematopoietic cells in bone marrow, liver, or lymph nodes. HLH can occur during the course of systemic lupus erythematosus (SLE), but can also be a presenting manifestation. Because development of pancytopenia occurs in less than 10 percent of SLE cases, investigation for HLH is necessary when otherwise unexplained pancytopenia persists despite adequate treatment. We experienced three cases of secondary HLH associated with SLE. Among the three patients, two patients developed HLH during the clinical course of SLE. The other patient who presented with pancytopenia was first diagnosed with HLH, and later with SLE. In her case, HLH turned out to be a presenting manifestation of SLE. We report on three successfully treated cases, and discuss the prevalence, characteristics, treatments, and prognosis of secondary HLH associated with SLE. (J Rheum Dis 2015;22:180-185)
혈청양성 류마티스관절염 환자에서 급성 신부전을 동반한 다발성 골수종
이승경 ( Seung Kyung Lee ),황선혁 ( Sun Hyuk Hwang ),박주한 ( Joo Han Park ),송가원 ( Ga Won Song ),박선영 ( Sun Young Park ),김세란 ( Sei Rhan Kim ),정주양 ( Ju Yang Jung ),배창범 ( Chang Bum Bae ),김현아 ( Hyoun Ah Kim ),정성현 대한류마티스학회 2014 대한류마티스학회지 Vol.21 No.5
It is known that rheumatoid arthritis (RA) patients show increased incidence of multiple myeloma (MM), despite its rarity. Only one case of MM with seronegative RA was reported in Korea, thus far. We report a case of MM with seropositive RA. The patient was a 66 year old female who had been diagnosed with seropositive RA 4 years ago. Over the last 1 month, the patient experienced general weakness and weight loss of 10 kg. It was found that her serum creatinine had increased and her urine analysis showed proteinuria. To evaluate renal failure and proteinuria, renal biopsy, bone marrow biopsy and electrophoresis were carried out. A diagnosis of myeloma cast nephropathy was made. We report this rare case of MM represented as acute renal failure during the treatment for RA, and include a review of the literature.