그레이브스병 환자에서 Propylthiouracil 복용 후 발생한 항중성구 세포질 항체 양성 반월상 사구체신염 1예 = A Case of ANCA-positive Crescentic Glomerulonephritis after Propylthiouracil Treatment in Graves' Disease

다국어 초록 (Multilingual Abstract)

Propylthiouracil (PTU) therapy is commonly used in the treatment of Graves' disease, but often accompanies several side effects, including a mild increase in liver enzymes, leukopenia, skin rash, and arthralgia. ANCA-positive vasculitis and crescentic glomerulonephritis have been rarely reported in patients suffering from (with) Graves' disease and treated with PTU. We experienced a rare case of ANCA-positive crescentic glomerulonephritis presenting rapid progressive renal failure in a 30-year- old woman, suffering from Graves' disease and treated with PTU for 6 years. She was admitted with dyspnea for 1 day and fever, gross hematuria, arthralgia and sore throat for several days. Her chest X-ray revealed moderate cardiomegaly, bilateral pulmonary edema, and bilateral pleural effusion. She had a palpable, firm, diffuse goiter. Anti-myeloperoxidase (anti-MPO) antibody and anti-protease 3 (anti-PR3) antibody were both positive by ELISA. A percutaneous renal biopsy showed crescentic golmerulonephritis showing active cellular crescent formation with some inflammatory cell infiltration and mesangial cell proliferation. Cellular crescents were present in 2 of 3 glomeruli. Immunofluorescence stain showed weak granular deposits of IgG, IgM and C3 in the mesangium and capillary wall. ANCA-positive crescentic glomerulonephritis associated with PTU was diagnosed. The patient was started on intravenous methylprednisolone 250 mg 2 times daily, and then oral prednisolone 100 mg every other day and PTU was discontinued. Her renal function was recovered gradually and anti-MPO antibody and anti-PR3 antibody subsequently fell. Second biopsy, 7 months after first biopsy, showed focal global glomerulosclerosis. 16 months after first biopsy. she had stable renal function with mild renal insufficiency and euthyroid state. (Korean J Nephrol 2005;24(2):305-312)

국문 초록 (Abstract)

Propylthiouracil (PTU)은 Methimazole, Carbimazole과 같이 thionamide 계열의 항갑상선제로서 부작용은 3-5%에서 나타나는데 흔한 부작용은 두드러기, 가려움증, 피부발진, 발열, 관절통 등의 과민성 반응이며, 이외에 미만성 탈모, 중독성 간염, 백혈구 감소증, 무과립구증, 루푸스양 증후군, 재생불량성 빈혈, 혈소판 감소증 등도 나타날 수 있다1).
PTU와 연관된 혈관염과 급성 사구체신염은 비교적 드문 합병증으로 알려져 있다. 1992년 Stankus와 Johnson2)은 PTU와 연관되어 발생한 혈관염에 의해 호흡부전이 초래된 1예를 처음 보고하였고, 1993년 Dolman 등3)은 PTU와 연관되어 혈관염이 발생한 6명 환자의 혈액에서 항중성구 세포질 항체 (anti-neutrophil cytoplasmic antibody, ANCA)를 확인하였다. 또한 1993년 Fujieda 등4)은 PTU와 연관된 ANCA 양성 반월상 사구체신염을 처음으로 보고하였다. 이후 PTU와 연관된 혈관염과 급성 사구체신염의 많은 증례와 Methimazole 또는 Carbimazole과 연관된 적은 수의 증례가 보고되었으며 IgA 신증 환자에서 PTU 복용 후 반월상 사구체신염의 발생이 보고되기도 하였다5). 국내에서는 이 등6)이 PTU 중단과 스테로이드 치료에도 반응하지 않은 P-ANCA 양성 반월상 사구체신염 1예를 보고하였고 이 등7)이 ANCA 양성 혈관염 1예를, 황 등8)이 ANCA 양성 급성 사구체신염 1예를 보고한 바 있다.
기존의 보고들에 따르면 PTU와 연관된 혈관염과 급성 사구체신염은 특징적으로 ANCA 양성반응을 보이며 신 생검에서 괴사성 또는 반월상 사구체신염으로 나타나는데, PTU를 중단하고 스테로이드 치료 또는 스테로이드와 cyclophosphamide의 병합치료를 적용하면 호전되어, 비교적 양호한 예후를 보인다고 하였다9,10).
저자들은 그레이브스병 환자에서 PTU 복용 후 발생한 ANCA 양성 반월상 사구체신염 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

참고문헌 (Reference)

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