| Objective:To analyze and compare the clinical signs, pathological data, treatment and prognosis in patients of MHT, to show the full view over this kind of disease; to detect the plasma Adamts 13 and complement factors, to investigate the pathogenesis of MHT.Methods : Enroll the total 92 cases of patients with clincal diagnosis of MHT(including 81 cases of MHT without TMA and 11 cases of MHT with TMA), who admitted in the department of Nephrology in Rujin Hospital of Shanghai Jiaotong university, and analyze the clinical data, treatment and prognosis of these cases;analyze pathological data in 74 cases of the patients mentioned above(including 68 cases of MHT without TMA and 6 cases of MHT with TMA); detect the plasma Adamts 13 activity, anti-Adamts 13 antibody plasma concentration, CFH(Complement Factor H) plasma concentration and MAC(Membrane Attack Complex)plasma concentration among 50 cases of MHT and 28 cases of TMA, by means of ELISA.Results:In all 92 cases of MHT, there were 81 cases(88.04%) of MHT without TMA and 11 cases(90.12%) of MHT with TMA. In both group of MHT without TMA and group of MHT with TMA, essential MHT account for the majority(90.12% and81.82%), most of patients had the previous hypertension history(50.62% and45.45%), signes of renal dysfunction were most common manifestation(67.90% and54.54%), the blood pressures were very high at the onset of the disease(SBP: 221.70 mm Hg and 216.27 mm Hg, DBP: 140.00 mm Hg and 141.00 mm Hg). In group of MHT without TMA, the age of patients was higher(35.00 years old vs 30.00 years old), percentage of male patients was higher(83.95% vs 54.54%), BMI was higher (26.40 kg/m2 vs 23.91 kg/m2). In comparison with group of MHT, group of MHT with TMA, patients presented much more serious renal inefficiency(Scr: 669.00μmol/l vs 287.00 μmol/l, e GFR-EPI: 8.10 ml?min-1?1.73m-2 vs 22.20ml?min-1?1.73m-2), lower level of platelet count(93.00×109/L vs 203.00×109/L), lower level of hemoglobin(82.73 g/L vs 120.07 g/L), higher level of VWF(230.86% vs163.65%), with mass proteinuria(proteinuria≥2+ in urine dipstick)(100.00% vs79.01%). The core of MHT treatment is the combination of different anti-hypertensive drugs, with higher dialysis rate(hemodialysis or peritoneal dialysis)in the group of MHT with TMA.After the timely and effective therapy, patients’ prognosis was good in both group of MHT without TMA and group of MHT with TMA,with high cure rate(64.20% and 81.82%), low recurrence(6.17% and 10.00%)but mortality of latter was a little bit of higher than that of former(0.00% vs 18.18%).Multivariate analysis showed that female(HR=2.730, P=0.017), elevated serum creatinine(HR=1.003,P<0.001) were the independent risk factors of renal survival,and application of ACEI/ARB could protect the renal function and slow down its deterioration in MHT patients.The rate of renal biopsy were high in our study, with that of 83.94% in the group of MHT without TMA and that of 54.54% in the group of MHT with TMA, The pathological characteristics in two groups were moderate nephrosclerosis, with moderate to serious tubular-interstitial lesions, most presenting renal arterio-arterial fibroproliferation(“onion skin” lesions) and hyalinosis, negative in immunofluorescence. And formation of vascular thrombosis was quite common in the group of MHT and TMA(50.00%). Besides, there existed a positive correlation between the severity of tubular-interstitial lesions and the level of serum creatinine.The results of blood detection in 50 blood samples of MHT showed, 32 cases(64.00%) with plasma Adamts 13 activity deficiency(enzyme activity<5%), 50 cases(50.00%) were negative in anti-Adamts 13 antibody. Plasma concentration of CFH was low(462.13 μg/m L) in 44 cases of MHT without TMA, while plasma concentration of CFH was normal(636.88 μg/m L) in 6 cases of MHT with TMA. Plasma concentration of MAC was high in both group(81.83 ng/m L and 288.89ng/m L)Conclusions: This is a large study about clinic data, pathological data and experimental results for MHT and its related TMA. MHT is a clinical emergency,presenting serious symptoms and signs, could progress rapidly, young and middle-aged patients often involved, with eyes, kidneys, heart and brain as the typical and common target organs. MHT is usually complicated with TMA, while those with TMA complication having the much more serious renal dysfunction. After timely and effectively intervention, patients’ prognosis is good, with high survival, high cure rate,low recurrence. In our study, we also found that the plasma Adamts 13 activity deficiency and activation of complement alternative pathway were involved in the pathogenesis of MHT. The joint detection of plasma Adamts 13 activity and complement factor would be able to help the diagnosis and differential diagnosis of MHT and its related TMA. |
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