硬皮症腎臟危機併發乳房惡性腫瘤：一案例報告

Scleroderma Renal Crisis and Breast Cancer in a Male Patient: Case Report

黃于恬(Yu-Tien Huang)；洪冬哲(Tung-Che Hung)；丁瑞聰(Jui-Tsung Ting)；白日東(Jih-Tung Pai)；王淳峻(Chun-Chun Wang)；盧彥文(Yen-Wen Lu)

硬皮症 ； 硬皮症腎臟危機 ； 乳癌 ； 雙重置換血漿分離術 ； Scleroderma ； Scleroderma renal crisis ； Breast cancer ； Double filtration plasmapheresis

醫學與健康期刊

9卷1期（2020 / 03 / 01）

125 - 131

繁體中文

硬皮症（scleroderma）是以微血管病變為主的自體免疫疾病，為一侵犯全身的結締組織疾病。而其引起的嚴重的腎臟侵犯，也是最常見的死亡原因為硬皮症腎臟危機（scleroderma renal crisis），病患突發惡性高血壓，隨即發生快速進行性腎衰竭。本案例為44歲男性，之前有憂鬱症和全身性硬化症病史，此次因突發性喘促及嚴重高血壓入院，因在住院期間腎功能持續惡化，進行腎臟切片檢查後確診為硬皮症腎臟危機，後經過雙重置換血漿分離術及使用captopril控制血壓，病人仍因尿毒症狀開始進行透析治療。九個月後不幸再發生乳癌。

Scleroderma (or systemic sclerosis; SSc) is an autoimmune condition with connective tissue disorders involving small vessels. Scleroderma renal crisis, which has a high mortality rate, occurs with sudden onset of malignant hypertension and rapid deterioration of renal function. Herein, we presented a case involving a 44-year-old male patient, who had a past history of depression and systemic sclerosis, being hospitalized for sudden onset of breathing difficulty. The patient was diagnosed as having a hypertension crisis and heart failure related acute decompensation. Renal biopsy showed intimal proliferation and thickening, narrowing and obliteration of the vascular lumen with concentric ＂onion-skin＂ hypertrophy. The patient received double filtration plasmapheresis and captopril treatment. Under this cyclophosphamide treatment, the patient's condition stabilized for three months. However, renal function deteriorated until he needed long-term hemodialysis. Unfortunately, he also developed breast cancer nine months later.

1. Cardoso, F,Bartlett, JMS,Slaets, L(2018).Characterization of male breast cancer: results of the EORTC10085/TBCRC/BIG/NABCG International Male Breast Cancer Program.Ann Oncol,29,405-417.
2. Cheta, J,Rijhwani, S,Rust, H(2017).Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity.J Investig Med High Impact Case Rep,5,2324709617734012.
3. Colaci, M,Giuggioli, D,Vacchi, C(2014).Breast cancer in systemic sclerosis: results of a cross-linkage of an Italian Rheumatologic Center and a population-based Cancer Registry and review of the literature.Autoimmun Rev,13,132-137.
4. Denton, CP,Lapadula, G,Mouthon, L,Muller-Ladner, U(2009).Renal complications and scleroderma renal crisis.Rheumatol,48,32-35.
5. Forbes, AM,Woodrow, JC,Verbov, JL,Graham, RM(1989).Carcinoma of breast and scleroderma: four further cases and a literature review.Br J Rheumatol,28,65-69.
6. Giordano, SH,Buzdar, AU,Hortobagyi, GN(2002).Breast cancer in men.Ann Intern Med,137,678-687.
7. Guillevin, L,Bérezné, A,Seror, R(2012).Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls.Rheumatology,51,460-467.
8. Khalil, AMM,Iftikhar, N,Hussain, SA,Tan, J(2013).Scleroderma renal crisis in a newly diagnosed mixed connective tissue disease resulting in a dialysis-dependent chronic kidney disease despite angiotensin-converting enzyme inhibition.CEN Case Rep,2,41-45.
9. Maria, ATJ,Partouche, L,Goulabchand, R(2019).Intriguing Relationships Between Cancer and Systemic Sclerosis: Role of the Immune System and Other Contributors.Front Immunol,9,3112.
10. Montanelli, G,Beretta, L,Santaniello, A,Scorza, R(2013).Effect of dihydropyridine calcium channel blockers and glucocorticoids on the prevention and development of scleroderma renal crisis in an Italian case series.Clin Exp Rheumatol,31,135-139.
11. Mouthon, L,Mehrenberger, M,Teixeira, L(2011).Endothelin‑1 expression in scleroderma renal crisis.Hum Pathol,42,95-102.
12. Nagaraja, V(2019).Management of scleroderma renal crisis.Current Opinion in Rheumatology,31,223-230.
13. Pearson, JE,Silman, AJ(2003).Risk of cancer in patients with scleroderma.Ann Rheum Dis.,62,697-699.
14. Penn, H,Howie, AJ,Kingdon, EJ(2007).Scleroderma renal crisis: patient characteristics and long-term outcomes.QJ Med,100,485-494.
15. Steen, VD,Costantino, JP,Shapiro, AP,Medsger, TA, Jr(1990).Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors.Ann Intern Med,113,352-357.
16. Steen, VD,Medsger, TA, Jr(2000).Long-term outcomes of scleroderma renal crisis.Ann Intern Med,133,600-603.
17. Woodworth, TG,Suliman, YA,Furst, DE,Clements, P(2016).Scleroderma renal crisis and renal involvement in systemic sclerosis.Nat Rev Nephrol,12,678-691.