Seronegative systemic lupus erythematosus: etiology of nephrotic syndrome and acute renal failure in early postpartum period

Systemic lupus erythematosus (SLE) is an autoimmune syndrome that occurs most commonly in women during their reproductive years. Nephritis is known to be one of the most serious complications of SLE. Lupus nephropathy is frequently associated with ANA and anti-dsDNA antibodies. Rarely, serological markers may be initially absent, and in many cases, they become positive after sometime. We present a 28-year old, otherwise healthy female who admitted to our clinic with edema, hypertension, proteinuria and acute renal failure following her fourth delivery. Serum immunological markers were negative and renal biopsy showed histopathological changes consistent with systemic lupus erythematosus as the etiology of nephrotic syndrome. A dramatic therapeutic response was achieved by pulse steroid and cyclophosphamide treatment following oral steroid therapy. In women with new onset nephrotic syndrome or renal function deterioration in postpartum period, even if the patient is asymptomatic or seronegative, it is crucial to exclude SLE for a rapid diagnosis and prompt treatment in the case of lupus nephritis. Renal biopsy is of diagnostic importance in such cases in which there is no other clinical, biochemical and serological evidence of the disease.     

Lupus nephritis: Clinical course as related to morphologic forms and their transitions

An intensive study of the course of lupus nephritis has been undertaken in 88 patients in whom strict morphologic criteria were utilized in classification. All were treated with steroid, and 17 received cytotoxic drugs in addition. Focal proliferative lupus nephritis generally follows a benign course except in the occasional instances when transition to the diffuse proliferative or membranous forms occurs. Membranous lupus nephritis, when characterized by persistent nephrotic syndrome, leads slowly to renal failure, but this progression is aborted in the one-third in whom remission of the nephrotic syndrome can be achieved. A fatal outcome occurs within five years in the majority of those with diffuse proliferative lupus nephritis and the nephrotic syndrome, often in association with necrotizing renal vasculitis, severe hypertension and accelerated renal failure. A small number with the diffuse proliferative form have a remission and then show only mesangial abnormalities, usually, however, with the appearance of glomerular sclerosis. Progressive glomerular sclerosis is observed in some patients and may be a sequel of the remission of the diffuse or focal proliferative lesions, or it may represent still another form of lupus nephritis. Mesangial immune deposits with or without proliferation, at times in the absence of clinical renal disease, are observed early in the course of systemic lupus erythematosus (SLE) and may proceed to the diffuse proliferative or membranous forms.     

Severe Schonlein-Henoch nephritis in adults. A report of twenty cases

Twenty patients with severe Sch?nlein-Henoch nephritis were selected on a histologically basis of diffuse proliferative endo- and/or extracapillary glomerulonephritis during a period of 12 years in Champagne-Ardenne. There were 15 men and 5 women, mean age 44.1 years. An infectious history was found in 40%, an urinary tract cancer in 15%. In all cases there was purpura, in 80% joint pain and in 50% digestive symptoms. Clinical presentation at diagnosis included, in all cases, hematuria (gross in 50%) and proteinuria (of nephrotic range in 80%); there was hypertension in 60% and renal failure in 80%. Histology found, in all cases, mesangial IgA and often C3 deposits, with a diffuse endocapillary proliferation in 10%, extra-capillary proliferation in 30% and both endo-extracapillary in 60%; 45% of the patients had crescents in greater than 50% of glomeruli. The outcome, after steroid and immunosuppressive treatment, was end-stage renal failure in 25%, moderate renal failure in 20%, or normal renal function in 55% with a mean follow-up period of 4.6 years. These severe nephritis were associated with repetitive and often necrotic purpura, frequent joint pain and severe digestive symptoms. The analysis of initial renal presentation confirmed the bad prognosis of nephrotic syndrome, renal failure and especially hypertension, which were well correlated with the severity and diffusion of proliferative lesions. Despite a worse known prognosis, these nephritis responded to an aggressive and early treatment.     

The clinical and epidemiologic features in 140 patients with lupus nephritis in a predominantly black population from one center in Kingston, Jamaica

BACKGROUND: Lupus nephritis has emerged as a major factor in the overall survival of patients and may help to explain the poor prognosis associated with systemic lupus erythematosus (SLE) in black patients. METHODS: The authors reviewed the clinical and epidemiologic features of lupus nephritis in 130 women and 10 men who were mainly of African descent. RESULTS: The mean (standard deviation) age at diagnosis of SLE was 27.9 (10.3) years. The majority of patients (75%) developed renal involvement within 1 year of presentation with SLE. The most frequent extrarenal manifestations were arthritis (67%), malar rash (44%), serositis (41%), and neurologic disorders (30%). Class IV nephritis was the most common glomerular lesion, accounting for 49% of the biopsies, with class II accounting for a further 23%. Proteinuria was a common feature at presentation in all classes. Nephrotic range proteinuria was most common in classes III and IV. Prevalence of nephrotic range proteinuria was similar in classes II (23%) and V (19%). Hematuria occurred in more than one half of the patients with classes II, IV, and V disease. Fifty-nine percent of the patients had renal impairment at the time of renal biopsy. The prevalence of hypertension, the nephritic syndrome, and renal impairment was significantly higher in class IV patients compared with all the other groups. Factors that were significantly associated with classes III and IV disease compared with the other classes on univariate analysis were renal impairment, proteinuria (but not in nephrotic range), low C3 levels, and anemia. CONCLUSIONS: The clinical features of the study patients were similar to those of patients belonging to other ethnic groups, but a high proportion of the study patients had renal impairment at the time of renal biopsy.     

Clinicopathological study of nephropathy in patients with rheumatoid arthritis]

We carried out a retrospective study to investigate the clinical and pathological findings in 31 patients with rheumatoid arthritis (RA). In clinical findings, 17 patients showed nephrotic syndrome, five had isolated proteinuria, two had proteinuria and hematuria and seven had renal failure. In pathological findings, there were 16 patients with membranous nephropathy (MN), two with proliferative glomerulonephritis (DPGN), two with minor glomerular abnormality (MGA), six with amyloidosis, 2 with tubulointerstitial nephritis, and three patients had accompanying lupus nephritis. Eleven of 16 with MGN had been treated with gold, bucillamine or D-penicillamine, so they were diagnosed as drug induced MGN. In the other five patients, we could not decide which drugs induced the nephropathy. The 2 cases of MGA were associated with nephrotic syndrome and acute renal failure, which were caused by non-steroidal antiinflammatory drugs. There were two cases of non-Ig A DPGN, which was regarded as the native nephropathy in RA. The three cases with lupus nephritis were diagnosed as systemic lupus erythematosus by the criteria of the American Rheumatism Association (ARA). In conclusion, the nephropathy in patients with RA was varied and renal biopsy was a useful examination.     

系统性红斑狼疮合并静脉血栓栓塞症临床相关危险因素分析

目的探讨系统性红斑狼疮（SLE）合并发生静脉血栓栓塞症（VTE）的临床相关危险因素。方法回顾性连续收集2008年1月至2012年2月期间在解放军总医院住院治疗的27例SLE并发VTE的患者入血栓组,并募集同期27例与血栓组性别、年龄、体质量指数（BMI）、生活方式等环境因素相匹配的不伴有VTE的SLE患者作为对照组,利用单因素统计学分析两组患者的静脉血栓形成相关临床危险因素（血小板计数、免疫功能、补体、合并低蛋白血症、狼疮肾炎、肾功能不全、肾病综合征、肾性高血压、蛋白尿、血尿等）及实验室诊断指标[C-反应蛋白（CRP）、D-二聚体,白细胞计数、活化部分凝血活酶时间（APTT）、血浆凝血酶原时间（PT）、血浆纤维蛋白原（FIB）1的差异。结果与对照组相比,血栓组合并低蛋白血症（70．37％）、狼疮肾炎（74．07％）、肾功能不全（70．37％）、肾病综合征（55．56％）、肾性高血压（66．67％）的发生率均显著升高（P值分别为0．003,0．000,0．000,0．027,O．029）。血栓组患者的实验室检测指标CRP（7．19±9．23）mg／L和D．二聚体（6．32±5．75）mg／L均显著高于对照组（P值分别为0．004,0．000）。结论低蛋白血症、狼疮肾炎、肾功能不全、肾病综合征及肾性高血压可能是SLE合并VTE的临床相关危险因素;CRP及D-二聚体可能成为SLE合并VTE的实验室诊断指标。     

Successful Treatment of Rapidly Progressive Lupus Nephritis Associated with Anti-MPO Antibodies by Intravenous Immunoglobulins

We report a case of systemic lupus erythematosus (SLE) associated with crescentic glomerulonephritis and myeloperoxidase-specific anti-neutrophil cytoplasmic antibodies (MPO-ANCA). A 34-year-old Japanese female patient diagnosed with SLE developed rapidly progressive renal failure and nephrotic syndrome. Haemodialysis was required to restore renal function. Methylprednisolone pulse therapy followed by plasmapheresis did not suppress the progression of renal failure, so she was treated with high-dose intravenous immunoglobulin (IV-IG) therapy, which was well tolerated and effectively prevented renal failure. A renal biopsy showed diffuse proliferative lupus nephritis (WHO classification IVc) with predominant crescent formation and scant subendothelial immune deposits. These findings indicate that, in addition to lupus nephritis, which usually results from the deposition of circulating or locally formed immune complexes, MPO-ANCA may be involved in the pathogenesis of crescentic glomerulonephritis. Furthermore, we propose that IV-IG is an effective therapy for MPO-ANCA-related renal crisis in lupus nephritis. Received: 10 December 1997 / Accepted: 23 July 1998     

Late development of systemic lupus erythematosus in patients with glomerular "fingerprint" deposits

Two patients presenting with nephrotic syndrome but without evidence of collagen vascular disease had organized glomerular immune deposits with a "fingerprint" pattern. This finding has been previously associated with lupus nephritis and, in our institution, has been seen in 6% of the biopsy specimens from patients with lupus nephritis. Clinical signs and symptoms of systemic lupus erythematosus in these two patients did not develop until 2 and 5 years later, respectively. The cases of these patients suggest that glomerular deposits with a fingerprint pattern may be a specific marker for lupus erythematosus even when overt clinical features of this disease are lacking. Patients with this finding on renal biopsy should have an extended follow-up for possible development of lupus erythematosus.     

Long-term follow-up of patients with lupus nephritis. A study based on the classification of the World Health Organization

The long-term course of 56 patients with systemic lupus erythematosus who had precisely defined renal histology and carefully assessed clinical status at the time of their initial renal biopsy prior to 1976 was evaluated and analyzed by life-table analysis. The average length of follow-up has now been greater than 10 years since initial biopsy. Patients with mesangial lesions (World Health Organization [WHO] classes IIA and IIB) had a more favorable renal and patient survival at five and 10 years than did patients in the other WHO classes (III, IV, and V). Individual renal histologic features of activity and chronicity when combined into an activity index and a chronicity index did not significantly predict renal survival in this population, nor did the presence of hypertension or renal dysfunction at the time of the initial renal biopsy significantly influence renal or patient survival. Patients with the nephrotic syndrome at initial biopsy had a poorer renal survival than did patients without the nephrotic syndrome. However, patients who experienced a remission of the nephrotic syndrome fared better in terms of both renal and patient survival than did those patients without a remission. By life-table analysis, patient survival was significantly better for patients in whom biopsy was performed after 1973 than for those in whom biopsy was performed prior to that time despite similar clinical features and WHO histology in each group interval. Our data suggest that improved survival for patients in recent studies may relate to better supportive care and more selective use of immunosuppressive therapy in patients with milder forms of lupus nephritis.     

Renal biopsy in elderly adults over 75 years of age

The results of 177 renal biopsies (RB) in patients over 75 years of age were analysed. The three most frequent histological types were: Overall: membranous nephritis (MN), minimal change disease (MCD) and IgA Nephropathy (IgAN); In nephrotic syndrome (51% of RB): MN (36%), MCD (33%) and amyloidosis (12%); In chronic renal failure without nephrotic syndrome (25% of RB): chronic interstitial nephritis (17%), benign nephrosclerosis (12%) and IgAN (12%); In acute or progressive renal failure (18% of RB): acute tubular necrosis (36%), crescentic GN (16%) and IgAN (12%). Isolated proteinuria was most frequently associated with IgAN. In only 40% of patients was the medical history relevant, and only in selected cases it allowed for accurate prediction of the histological findings. Our data favor a more liberal use of biopsy in the elderly patients.     

Comparison of renal disease severity and outcome in patients with primary antiphospholipid syndrome, antiphospholipid syndrome secondary to systemic lupus erythematosus (SLE) and SLE alone

OBJECTIVE: To ascertain the clinical presentation, histopathology and outcome of renal involvement in patients with primary antiphospholipid syndrome (PAPS), antiphospholipid syndrome secondary to systemic lupus erythematosus (SAPS) and systemic lupus erythematosus alone. METHOD: A retrospective analysis was undertaken of 20 patients with PAPS, 25 patients with SAPS and 275 patients with systemic lupus erythematosus to ascertain the frequency and pattern of renal involvement. RESULTS: Renal involvement was found most frequently in patients with SAPS, in whom it occurred in 68% of patients. Renal disease was equally common in patients with PAPS and systemic lupus erythematosus alone where it was seen in 30% of patients. Patients with systemic lupus erythematosus most frequently presented with nephrotic syndrome due to glomerulonephritis, whereas those with PAPS and SAPS were more likely to present with hypertension and reduced glomerular filtration rate. No patients with PAPS developed end-stage renal failure compared with 5.9% of patients with SAPS and 16.9% of patients with systemic lupus erythematosus alone; 23.5% of patients with SAPS died compared with 15.7% of patients with systemic lupus erythematosus alone and no patients with PAPS. CONCLUSION: Renal involvement is a major feature of both PAPS and SAPS, where renal thrombosis frequently leads to reduced glomerular filtration rate and hypertension. One-third of patients with systemic lupus erythematosus alone develop glomerulonephritis leading to renal disease which most commonly presents with nephrotic syndrome. Patients with PAPS were less likely to develop end-stage renal failure or die during the follow-up period.     

Clinical and Laboratory Predictors of Distinct Histopathogical Features of Lupus Nephritis

The authors aimed to explore whether distinct clinical, serological, and urinalysis findings are associated with specific histological classes of lupus nephritis. Clinical and laboratory features were recorded at the time of clinical diagnosis from 297 consecutive patients with biopsy-confirmed lupus nephritis. Univariate and logistic regression analyses were performed and a risk score was developed to estimate the risk for developing different classes of lupus nephritis. Variables independently associated with class II included absence of malar rash, negative anti-dsDNA, and ≤5 urine leucocytes/high power field (hpf); with III/IV: age at nephritis diagnosis ≤32 years old, presence of musculoskeletal features, new-onset hypertension, positive anti-dsDNA, >5 urine leucocytes/hpf, creatinine >1.2 mg/dL, cellular casts >1/hpf, and absence of nephrotic range proteinuria; with V: age at nephritis diagnosis >32 years, malar rash, absence of musculoskeletal complaints or serum C3 hypocomplementemia, nephrotic range proteinuria, and ≤9 urine erythrocytes/hpf. A risk predictive score of specific histological classes was calculated for each patient. Associations between 2, 3 or more risk factors with specific histological classes were also revealed [Odds ratios (95% confidence interval) (≥2 risk factors) was 6.7 (2.8–17.4) for class II nephritis, 15.6 (5.1–47.8), and 8.2 (3.6–19.0) for classes III/IV and for class V, respectively (≥3 risk factors)]. The identification of independent factors associated with specific classes of lupus nephritis can provide guidance in selecting specific therapeutic modalities, particularly in cases in which renal biopsy is contraindicated.     

Lesiones renales en el lupus eritematoso diseminado: ausencia de relación entre datos clínicos e histológicos

Background

The existence and type of renal involvement influences the prognosis of systemic lupus erythematosus and this information may be critical when it comes to taking appropriate therapeutic decisions.

DIALYSIS AND TRANSPLANTATION IN PATIENTS WITH RENAL FAILURE DUE TO SYSTEMIC LUPUS ERYTHEMATOSUS. THE AUSTRALIAN AND NEW ZEALAND EXPERIENCE

Abstract Between 1977 and 1985, 5726 patients in Australia and New Zealand entered end stage renal failure programmes. Of these, 63 patients had renal failure due to systemic lupus erythematosus (a prevalence of 1.1% of patients entering renal replacement programmes). When compared with patients with other forms of glomerulonephritis, there was a female preponderance and a younger age distribution in patients with renal failure due to lupus nephritis. Integrated patient, dialysis, and transplant survival data showed that results in patients with renal failure due to lupus nephritis were comparable with those in patients with other forms of glomerulonephritis or in patients with renal failure due to any cause. Age at entry significantly affected survival, with significant differences being found in those patients under as opposed to over 50 years of age. Causes of death in patients with lupus nephritis were similar to those in patients with renal failure due to other causes. It is concluded that dialysis and transplantation are acceptable forms of treatment for patients with end stage renal failure due to systemic lupus erythematosus.     

84例干燥综合征合并肾脏损害的临床与病理分析

目的 结合肾活检及相关实验室检测结果,进一步探讨干燥综合征（SS）肾脏损害的临床及病理特征。方法 对我科1993－1999年间收住的84例SS肾脏损害患者,进行常规免疫学、肾小管功能及部分肾活检检查。结果 55／84例为肾小管性酸中毒（RTA）,5／55例合并肾性尿崩症,3／55例合并低钾性麻痹;表现为肾病综合征及肾小球肾炎（GN）分别为12及10例;14／84例有轻度肾功能衰竭。69．1％的患者为高γ球蛋白血症,64．3％和44．1％的患者抗SS－A和抗SS－B（＋）。肾活体组织检查37例,光镜下21例为慢性间质性肾炎伴间质大量淋巴、浆细胞浸润及小管萎缩,10／37例为狼疮性肾炎（LN）,5例为系膜增生性肾炎。免疫荧光检查大多阴性,部分标本肾小球有免疫球蛋白和补体沉积,1例IgG肾病患者有间质沉积。25例应用泼尼松及环磷酰胺（CTX）冲击治疗。14例肾功能受损者血清肌酐（Cr）水平基本降低正常。结论 SS肾脏损害常见,以RTA和GN为主要表现,泼尼松治疗可以减少间质淋巴细胞和浆细胞浸润,改善肾功能,纠正RTA。     

Systemic lupus erythematosus. III. Observations on clinical renal involvement and follow up of renal function: Dutch experience with 110 patients studied prospectively.

A prospective study of 110 patients with systemic lupus erythematosus (SLE) was undertaken to evaluate the reliability of clinical signs of lupus nephritis, which developed in 39 (35%) patients. Those patients with SLE who showed no clinical signs of lupus nephritis had an excellent survival rate (10 year survival 93%) and retained normal renal function (serum creatinine less than 130 mumols/l); clinical lupus nephritis developed mainly in the first three years after diagnosis of SLE and was associated with a decreased survival rate (10 year survival 62%). Increased mortality was found in male patients with lupus nephritis over 25 years of age and in female patients with lupus nephritis under 25 years of age, while renal failure rates did not differ between these groups. Treatment of lupus nephritis with high dose prednisone alone or in combination with immunosuppressants did not result in differences in patient survival or renal function preservation. It was concluded that clinical variables are a reliable guide in the management of patients with SLE, and routine use of renal biopsy in these patients is rejected.     

Intravenous cyclophosphamide for lupus nephritis in Thai children

OBJECTIVE: To evaluate the efficacy of a 36-month course of intravenous cyclophosphamide therapy for severe lupus nephritis in Thai children between October 1993 and December 2000. METHODS: Intravenous cyclophosphamide combined with oral prednisolone was given for 36 months to patients with systemic lupus erythematosus (SLE) who had severe renal involvement. Serum creatinine (Cr), creatinine clearance (CCr), urinary protein, C3, and complete blood count (CBC) were measured each visit for intravenous cyclophosphamide. Repeated measures ANOVA and Kaplan-Meier survival analysis were used. RESULTS: Of 21 patients enrolled in the study, three died and two were lost to follow-up, leaving 16 patients who completed therapy (13 females and three males) with age at diagnosis 12.1+/-2.3 years (range 7.2-20.6 years). The follow-up period was 6.3+/-2.3 years (range 3.3-13.8 years). Fourteen patients had lupus nephritis WHO classification class IV and two had lupus nephritis WHO classification class II. Hypertension was detected in ten patients. Lowess smoothing curves and repeated measures ANOVA indicated no significant change in Cr and CCr [probability (p) > 0.05)], but significantly increased C3 and haemoglobin and significantly decreased urinary protein and white blood cell count (p < 0.001). Five patients had six episodes of acute renal failure; one died, renal function returned to normal in two patients, two continued to chronic renal failure, and one died of chronic renal failure. The 5-year survival and renal survival were 86.5% and 87.5% (95% CI 55.8-96.5% and 58.6-96.7%), respectively. CONCLUSION: Intravenous cyclophosphamide in severe lupus nephritis in Thai children showed a satisfactory outcome with minimal complications. Further follow-up is needed.     

Outcome and prognostic indicators of diffuse proliferative lupus glomerulonephritis treated with sequential oral cyclophosphamide and azathioprine

OBJECTIVE: To study the outcome and prognostic indicators of diffuse proliferative glomerulonephritis (DPGN) in patients with systemic lupus erythematosus (SLE) treated with sequential oral cyclophosphamide (CYC) and azathioprine (AZA). METHODS: SLE patients with biopsy-proven DPGN treated with sequential oral CYC and AZA were studied. Those who achieved renal remission at 12 months were identified, and the clinical predictors of complete remission were evaluated by regression analysis. All patients were followed up until a relapse of the nephritis or a doubling of the serum creatinine level occurred. The timing and risk factors for flares and creatinine doubling were evaluated by Kaplan-Meier analysis and with the Cox proportional hazards model. RESULTS: We studied 55 patients (47 women, 8 men; mean +/- SD age at renal biopsy 31.1 +/- 10.4 years); 25 (46%) had a serum creatinine level >106 micromoles/liter, and 29 (53%) had nephrotic syndrome. At 12 months posttreatment, 37 (67%) had complete remission and 12 (22%) had partial remission. The initial serum creatinine level was an independent predictor of complete remission. Excluding the 4 patients who were treatment- resistant or died, 21 patients (41%) had renal flares during a median followup of 4 years. The cumulative risk of renal flare was 6% at 1 year, 21% at 3 years, and 32% at 5 years. The median time to relapse was 43 months. The histologic activity score and the mean daily dose of CYC were multivariate predictors of renal flare, by Cox regression. At the last followup visit, 9 of 54 patients (17%) had a doubling of the creatinine level, 6 of whom (11%) underwent dialysis. The cumulative risk of creatinine doubling was 8.4% at 5 years and 18.2% at 10 years. An increasing chronicity index at the time of initial renal biopsy was an independent predictor of deterioration in renal function. CONCLUSION: Sequential therapy with oral CYC followed by AZA appears to be an effective treatment regimen for DPGN in patients with SLE, with 89% of patients achieving complete or partial remission at 12 months, 62.8% remaining in remission after 5 years, and 81.8% having stable renal function after 10 years. Predictors of treatment resistance and relapse include increasing serum creatinine level, higher histologic activity scores, and a lower dose of CYC. Increasing chronicity indices predict a deterioration of renal function.     

Long-term outcome of lupus nephritis in Asian Indians

Objective

There are sparse data on outcome of lupus nephritis from developing countries. This study looks at outcome in Asian Indians.

Methods

This retrospective study included patients at a single center over 20 years. Patients were treated as per standard protocols. The primary outcome measure was chronic renal failure or death; the secondary outcome was end‐stage renal disease or death. The worst‐case scenario was also calculated, considering those lost to followup in the first year as events. Kaplan‐Meier survival curves and the log rank test were used for survival analysis. Data are shown as the mean ± SD.

Results

We included 188 patients with lupus nephritis, with a female:male ratio of 11:1, a mean ± SD age at onset of 23.6 ± 10.5 years, and a median followup time of 6 years (interquartile range 3–9 years). Of 136 patients with a biopsy sample, the distribution was as follows: class II in 22, class III in 36, class IV in 61, class V in 16, and class VI in 1. Survival with normal renal function was 84%, 69%, and 57% at 5, 10, and 15 years, respectively; in the worst‐case scenario, survival was 77%, 63%, and 51%, respectively. There was no difference in survival by histologic class; however, nonbiopsied patients had lower survival. Renal survival was 91%, 81%, and 76% at 5, 10, and 15 years, respectively; in the worst‐case scenario, survival was 79%, 70%, and 66%, respectively. Risk factors for poor outcome were low C3, hematuria, hypertension, creatinine, lack of remission, and occurrence of major infection. There was a high rate of major infections of 42.3%, with tuberculosis at 11.5%. Infections caused one‐half of all deaths.

Conclusion

The outcome of lupus nephritis in Asian Indians with standard immunosuppressive regimens is reasonable, but immunosuppression is associated with a high rate of infection.     

Posterior reversible encephalopathy syndrome--an underrecognized manifestation of systemic lupus erythematosus

OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) is a rare, recently described neurologic condition identifiable by clinical presentation and magnetic resonance image (MRI) appearance. It is associated with renal insufficiency, hypertension, and rheumatologic diseases. Patients present with headache, seizures, loss of vision and altered mental function, and a pattern on imaging studies of predominantly transient, posterior cerebral hyperintensities on T2-weighted MRI. There is a high likelihood of presentation of this syndrome to a rheumatologist. METHODS: Three recent cases of systemic lupus erythematosus (SLE) with PRES, along with 9 previously reported cases, are reviewed. RESULTS: All 3 patients presented with seizures and subacute visual changes in association with lupus nephritis. The first presented with hypertension, complete visual field loss, and status epilepticus 2 weeks after starting oral cyclosporine therapy for refractory lupus nephritis. The second patient was normotensive and presented with seizures and visual symptoms while in hospital with SLE-related pancreatitis and nephritis. The third patient had headache and seizures with severe lupus disease activity including nephritis, pancytopenia, and pulmonary hemorrhage. Cranial MRI showed predominantly posterior signal abnormalities on T2-weighted images, which resolved after cessation of cyclosporine in the first case, treatment with IV cyclophosphamide in the second case, and treatment with cyclophosphamide and plasmapheresis in the final case. Literature review showed that PRES is a manifestation of SLE or a consequence of therapy with calcineurin inhibitors or rituximab. The hallmark features are visual loss and seizures. Severe hypertension (> 170/110 mm Hg) and renal failure were present in the majority of previously identified cases of SLE and PRES. Our second case was normotensive but had marked lupus disease activity. PRES can lead to cerebral infarction. CONCLUSION: With increasing availability of MRI, PRES will be identified more frequently. Swift action to identify potential offending agents, controlling hypertension, and treating active disease can lead to reversal of radiologic and neurologic findings.