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

Abstract

IntroductionIn 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.MethodsStudy 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.ResultsOf 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.DiscussionThe presence of TMA in patients with LN is associated with adverse clinicopathological presentation and poor outcome.

Management of lupus nephritis: a systematic literature review informing the 2019 update of the joint EULAR and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations

“…43 Additionally, evidence of thrombotic microangiopathy may be present in 5–25% of patients with LN. Nevertheless, data from observational studies regarding its impact on prognosis are equivocal; six studies have shown association of thrombotic microangiopathy with poor long-term renal outcomes (OR ranging from 2.14 to 5.80) or worse response to treatment, 44– 49 while five studies have failed to show such an association. 50– 54…”

Plasma exchange in lupus nephritis with thrombotic microangiopathy

“…In our prospective series of 197 patients with LN, 50 (25.4%) patients had evidence of TMA. A total of eight (16%) patients received PLEX.…”

“…Thrombotic microangiopathy (TMA) is a rare, life‐threatening disease that involves multiple systems by microvascular occlusion. Recent studies have reported a prevalence of TMA in lupus nephritis (LN) around 25% . The increase in incidence may be related to improved understanding of the disease entity.…”

Management and treatment of glomerular diseases (part 2): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference

“…TMA with LN on kidney biopsy may be due to antiphospholipid antibodies/syndrome ([APS], anticardiolipin antibodies, anti-b2 glycoprotein I, and lupus anticoagulant), thrombotic thrombocytopenic purpura, or atypical hemolytic uremic syndrome. 134,135 Treatment should be guided by the underlying etiology of TMA. 134 Plasma exchange is indicated for thrombocytopenic purpura, but it may also be beneficial in cases of refractory APS.…”