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      Effect of Thrombotic Microangiopathy on Clinical Outcomes in Indian Patients With Lupus Nephritis

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          Abstract

          Introduction

          In addition to glomerular lesion, renal vascular lesion is also an important prognostic marker of lupus nephritis (LN). Among patients with various vascular changes, individuals with thrombotic microangiopathy (TMA) present with severe clinical manifestations and have a high mortality. The aim of the present study was to assess the spectrum and impact of TMA on the outcomes of LN. In a prospective observational study of 2.5 years’ duration, clinical and renal histopathological data regarding biopsy-proven LN were noted, and evaluation for antiphospholipid syndrome (APS) as a cause of TMA in LN was also carried out.

          Methods

          Study subjects were followed up actively for 6 months, and various outcomes were noted. Cases were divided into 2 groups as LN with TMA and LN without TMA, and various features were compared between the 2 groups. Outcomes recorded were complete response (CR), partial response (PR), treatment failure, and death.

          Results

          Of the 197 patients with LN, 50 patients (25.4%) were diagnosed with co-existing renal TMA. Five patients (10%) were found to have concomitant APS. As compared to patients without TMA, those with TMA had significantly higher rates of oliguria ( P = 0.035), advanced renal injury, that is, serum creatinine > 3mg/dl ( P = 0.002), fibrocellular and fibrous crescents ( P = 0.01), and tubular atrophy ( P = 0.001). Outcomes included CR in 15 patients (30%), PR in 10 (20%), failure in 19 (38%), and death in 6 (12%). Patients with LN with TMA had higher rates of treatment failure ( P = 0.02) compared to the group without TMA.

          Discussion

          The presence of TMA in patients with LN is associated with adverse clinicopathological presentation and poor outcome.

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          Most cited references11

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          Renal vascular complications of systemic lupus erythematosus.

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            Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan.

            Thrombotic microangiopathy (TMA) co-existing with SLE is rarely reported. This study aimed to investigate the triggering factors, clinical features and outcomes of SLE patients with TMA in Northern Taiwan. Twenty-five TMA cases out of 2461 SLE patients admitted to Taipei Veterans General Hospital, between 2000 and 2010, were enrolled. When TMA occurred, 16 (64.0%) patients had infection; 22 (88.0%) were in an active disease state with a SLEDAI score >10. Among the infection group, 13 (81.3%) had an increase in the SLEDAI score of ≥ 4. We found that older age (≥ 50 years), low platelets (≤ 20,000/nm(3)), presence of infection, acute renal failure (ARF) or four or more TMA features were independent risk factors for persistent haematological abnormalities (P  2%) were the risk factors for persistent renal function impairment (P < 0.05). The overall mortality rate was 52.0% (13 out of 25); older age (≥ 40 years), low complement value, presence of infection (P < 0.001), two or more infection sources, ARF and four or more TMA features were the statistically significant factors contributing to a higher mortality rate. Patients receiving plasma exchange seven times or more had a significantly higher rate of improvement in renal function and haematological abnormalities. Our study showed that infection was one of the major triggers for the flare-up of SLE disease activity and occurrence of TMA in SLE. Infection is also a strong risk factor for outcome in SLE patients with TMA. Plasma exchange can be considered as an adjuvant treatment modality.
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              Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13.

              Thrombotic thrombocytopenic purpura (TTP) is a rare but frequently fatal complication of SLE. It occurs in the context of both active and inactive lupus and carries a worse overall prognosis than idiopathic acquired TTP. Recent advances in the knowledge and treatment of TTP do not seem to have brought similar improvements in the management and outcome of TTP in SLE. The illumination of the role of the von Willebrand factor multimer protease, ADAMTS13 in idiopathic TTP continues to enhance our comprehension of the pathogenesis of the disease and has contributed to improvements in diagnosis and management. We explore the overlap of TTP and SLE, and discuss the current understanding of the involvement of ADAMTS13 and its implications for patients with this uncommon form of microangiopathic haemolytic anaemia.
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                Author and article information

                Contributors
                Journal
                Kidney Int Rep
                Kidney Int Rep
                Kidney International Reports
                Elsevier
                2468-0249
                27 April 2017
                September 2017
                27 April 2017
                : 2
                : 5
                : 844-849
                Affiliations
                [1 ]Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
                [2 ]Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
                [3 ]Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
                Author notes
                [] Correspondence: K.L. Gupta, Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India.Department of Nephrology, Postgraduate Institute of Medical Education and ResearchChandigarh-160012India klgupta@ 123456hotmail.com
                [4]

                NP, HA, and KLG contributed equally to this work.

                Article
                S2468-0249(17)30103-1
                10.1016/j.ekir.2017.04.008
                5733673
                29270491
                98829968-c2d2-4d5a-b2ad-56c4a91a2633
                © 2017 International Society of Nephrology. Published by Elsevier Inc.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 24 January 2017
                : 13 April 2017
                : 17 April 2017
                Categories
                Clinical Research

                antiphospholipid syndrome,lupus nephritis,thrombotic microangiopathy

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