Case 2018-1 A 38-Year-Old Female with Rapid Progressive Renal Failure ( 董睿哲,李啓弘,徐永勳 )

☆ 年輕女性會想 SLE with lupus nephritis,但是病人沒有 skin rash 或 arthralgia 等症狀,C3 稍掉 C4 正常,ANA 、Anti-dsDNA 等也 negative,故不考慮

☆ 病人沒皮膚上沒看到 vasculitis,無肺部病狀,CXR clear,ANCA及 Anti-GBM disease 不像

☆  病人尿蛋白不多 ( sub-nephrotic),HBV、HCV、Cryoglobulinemia 皆 negative,所以 MPGN 不考慮

☆ 所以最後只剩 IgAN 了,來看看切片

☆  以下切片感謝李啓弘醫師的製作及說明

☆  Severe chronic changes, characterized by diffuse (76%) global glomerulosclerosis (arrow) and severe interstitial fibrosis and tubular atrophy (80-90%). (PAS, 40X)

☆  Minimal to mild mesangial hypercellularity ( 三角箭頭,大於三個細胞) with segmental sclerosis (箭號) in 5 (20%) glomeruli. (PAS, 400X)

☆  Endocapillary hypercellularity (三角箭頭,細胞核較小,外來的發炎細胞) 

☆ 這張 patho 還有一個特別的地方,有看到嗎?

☆  Crescent ( 半月形 ) formation,RPGN 常見 ( 內專考過 )

☆ Moderate arteriosclerosis. (H&E, 200X) ,病人血壓高而不自知

☆  IgA 在 mesangial area deposition,C3 也有一些,說明 IgAN 是走 alternative pathway

☆  An increase in mesangial matrix with scattered electron-dense deposits ( 箭號 ), as well as  endocapillary hypercellularity with endothelial swelling and podocyte foot process effacement ( 箭頭 ). (TEM, 3000X)

☆ Final Diagnosis : IgAN with RPGN

☆ 因為 76% 腎絲球已 global glomerulosclerosis , steroid + immunosuppresant  只是帶來更多風險而無助益,所以在病人透析兩週平順後讓她帶 perm-catheter 回國,並交待必須持續接受透析治療

☆ 如果病人再早一點來,要怎麼治療呢? IgAN 病生理機制為何呢?

請跳轉這裡有答案 ( Link

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