Rapid and Complete Remission of Class IV Lupus Nephritis with Massive Wire Loop Lesions

Class IV lupus nephritis (LN) often has a poorer prognosis than other classes. However, class IV LN has various phenotypes, including not only segmental and global types but also others. We present the case of a 29-year-old woman with class IV-G LN who had an early response to glucocorticoid monotherapy. In addition, multiple lung nodules such as miliary tuberculosis (TB) were detected on computed tomography. All cultures of sputum, gastric fluid, and bone marrow were negative. A kidney biopsy revealed diffuse endocapillary proliferative glomerulonephritis with marked subendothelial deposition. Electron microscopy revealed massive electron-dense deposits in the subendothelial area, mesangium area, and peritubular capillaries. The histological diagnosis was class IV-G (A) LN. We administered high-dose glucocorticoid monotherapy. After treatment, the LN and the lung lesions had complete, rapid remission for 1 month. The lung lesions were associated with an immune complex similar to wire loop lesions, not TB. Thus, it is important to consider class IV-G LN with massive wire loop lesions as a new subtype.

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P0897RISK FACTORS FOR AVASCULAR BONE NECROSIS IN PATIENTS WITH LUPUS NEPHRITIS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0897 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Daniela Monova ◽

Simeon Monov ◽

Assen Kamenov ◽

Vladislava Milenova

Keyword(s):

Risk Factors ◽

Lupus Nephritis ◽

Avascular Necrosis ◽

Immunosuppressive Agents ◽

Individual Risk ◽

Control Group ◽

High Dose ◽

Increased Risk ◽

Avascular Necrosis Of Bone ◽

Class Iv

Abstract Background and Aims Avascular necrosis of bone (AVN) is an important complication of systemic lupus erythematosus (SLE) and often causes serious physical disability. The aim of this study was to investigate the risk factors for symptomatic avascular necrosis of bone (AVN) in lupus nephritis (LN) patients. Method The records of 374 patients (43 males, 331 females) with kidney biopsy-proven LN were reviewed retrospectively. Symptomatic AVN cases were defined as those with at least one diagnosis of AVN. The patients with LN who did not have AVN were evaluated as a control group. To determine risk factors for AVN, clinical, laboratory and therapeutic variables were analyzed by logistic regression. Results Symptomatic AVN was present in 17 patients (4 males, 13 females, mean age of 27,4±6,7 years). Among the 17 patients, 28 joints presented AVN. 12 occurred in hips (2 bilateral), 6-in ankles, 4-in knees, 3-in shoulders and 1- in lumbar spine. In 9 patients AVN involved 2 or more joints. 14 patients were on steroids at the time of presentation of AVN. 2 patients were not on CS and 1 patient did not has documentation of steroid use. Meta-analysis demonstrates a significant increased risk of AVN in patients with high disease activity and class IV LN (p<0,005). LN patients with AVN showed an earlier onset age (p<0,05) and received significantly higher total cumulative corticosteroid dose. AVN was not significantly associated with use of immunosuppressive agents. Serositis, coagulation disorders, vasculitis, cigarette smoking were higher incidence in male with LN and AVN. Raynaud‘s phenomenon, autoimmune thyroiditis, arthritis, Sjögren’s syndrome, IgM anticardiolipin antibodies, antiphospholipid syndrome, Cushingoid body habitus were higher incidence in female with LN and AVN. Conclusion Many risk factors have been involved in the development of AVN in LN patients. AVN is prevalent in class IV LN and in younger patients. Since asymptomatic osteonecrosis may remain undetected, its true prevalence could be much higher than we reported. Multifocal lesions involving more than three anatomical sites are unusual. Corticosteroids are the principal risk factor, although some cases of AVN occur in relatively steroid naïve patients. Early detection of AVN is important because the prognosis depends of the stage and location of the lesion. An individual risk assessment for AVN development should be made prior to and during treatment for LN, especially in patients high dose corticosteroids.

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Lupus Nephritis in Children—A Review of 167 Patients

PEDIATRICS ◽

10.1542/peds.94.3.335 ◽

1994 ◽

Vol 94 (3) ◽

pp. 335-340

Author(s):

Ling-Yoeu Yang ◽

Wei-Perng Chen ◽

Ching-Yuang Lin

Keyword(s):

Risk Factors ◽

Renal Failure ◽

Lupus Nephritis ◽

Renal Biopsy ◽

Proliferative Glomerulonephritis ◽

High Dose ◽

Creatinine Concentration ◽

Initial Biopsy ◽

Class Iv

Background. Relatively few studies have been made of children with lupus nephritis. The prognosis of children with lupus nephritis is ominous for those with diffuse proliferative glomerulonephritis and active interstitial inflammation. Up to now few studies have been made on this subject. Objectives. To evaluate the clinical course, histopathology, and prognosis of lupus nephritis in children, to identify the risk factors for renal failure and mortality, and to share our experience in treating lupus nephritis in children. Methods. Retrospectively, 167 children under 18 years of age with lupus nephritis at Veterans General Hospital-Taipei, Taiwan from 1979 to 1991 were studied. All patients received renal biopsy and follow-up biopsies were performed in 36 children. The clinical and serologic parameters at the time of renal biopsy were recorded. Results. There were 55 (33%) patients with class II, 30 (18%) with class III, 69 (41.3%) with class IV, and 13 (7.8%) with class V nephritis based on initial biopsy. The mean follow-up time was 59 months. Follow-up biopsies were histologically stationary in 29 patients, progressive in five, and regressive in two. The results revealed that those with persistent hypertension, anemia, increased serum creatinine concentration, and decreased creatinine clearance rate at initial biopsy were more prone to develop renal failure. Low titer of CH50 hemolytic assay appeared to be a poor prognostic indicator. The overall renal and patient 5-year survival rates were 93.1% (135/145) and 91.08% (143/157), respectively. They were 87.7% (50/57) and 82% (55/67), respectively, of patients with class IV proliferative glomerulonephritis. Conclusions. The prognosis of children with class IV nephritis in this study was better than that reported previously. All children surviving without renal failure were maintaining their normal lives with little organ dysfunction. The improved results may be due to earlier renal biopsy for precise histopathologic definition, better supportive care, and selective use of aggressive therapy, including methylprednisolone pulse therapy, intravenous cyclophosphamide, intravenous prostaglandin E1 therapy, high-dose intravenous gammaglobulin therapy, and cyclosporin A for those with high risk factors.

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Successful Treatment of Class IV+V Lupus Nephritis with Combination Therapy of High-dose Corticosteroids, Tacrolimus and Intravenous Cyclophosphamide

Internal Medicine ◽

10.2169/internalmedicine.52.9366 ◽

2013 ◽

Vol 52 (10) ◽

pp. 1125-1130 ◽

Cited By ~ 5

Author(s):

Takahiko Kurasawa ◽

Hayato Nagasawa ◽

Eiko Nishi ◽

Hirofumi Takei ◽

Ayumi Okuyama ◽

...

Keyword(s):

Lupus Nephritis ◽

Combination Therapy ◽

Successful Treatment ◽

High Dose ◽

Intravenous Cyclophosphamide ◽

Class Iv

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High-dose diquat poisoning: a case report

Journal of International Medical Research ◽

10.1177/03000605211026117 ◽

2021 ◽

Vol 49 (6) ◽

pp. 030006052110261

Author(s):

Yanxia Huang ◽

Renjing Zhang ◽

Mei Meng ◽

Dechang Chen ◽

Yunxin Deng

Keyword(s):

Renal Failure ◽

Multiple Organ Failure ◽

Organ Failure ◽

Multiple Organ ◽

High Dose ◽

Severe Respiratory Failure ◽

Aggressive Treatment ◽

Lung Lesions ◽

Pulmonary Lesions ◽

New Treatment

Diquat is a widely used herbicide that is substituted for paraquat. With paraquat off the market, cases of diquat poisoning have been gradually increasing. The kidney is the most frequently impaired organ in diquat poisoning. Few cases of multiple organ failure caused by diquat have been reported. We herein describe a 30-year-old man who orally ingested about 160 mL of enriched diquat. Despite aggressive treatment, the patient’s condition progressed to multiple organ failure and death. The pulmonary lesions in this patient were different from those previously reported. This patient did not die of renal failure but of severe respiratory failure. He exhibited three different stages of pulmonary disease. The lung lesions in this case were unique. We hope that doctors will pay more attention to the lung lesions in patients with diquat poisoning in future and find new treatment methods to save the lives of such patients.

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Patients with Primary Central Nervous System Lymphoma Not Eligible for Clinical Trials: Prognostic Factors, Treatment and Outcome

Cancers ◽

10.3390/cancers13122934 ◽

2021 ◽

Vol 13 (12) ◽

pp. 2934

Author(s):

Sabine Seidel ◽

Michelle Margold ◽

Thomas Kowalski ◽

Alexander Baraniskin ◽

Roland Schroers ◽

...

Keyword(s):

Central Nervous System ◽

Clinical Trials ◽

Nervous System ◽

Prognostic Factors ◽

Central Nervous System Lymphoma ◽

Initial Response ◽

Early Response ◽

Multicenter Trial ◽

High Dose ◽

Therapeutic Trials

Patients with primary central nervous system lymphoma (PCNSL) not fulfilling inclusion criteria for clinical trials represent an underreported population. Thirty-four consecutive PCNSL patients seen at our center between 2005 and 2019 with exclusion criteria for therapeutic trials were analyzed (non-study patients) and compared with patients from the G-PCNSL-SG-1 (German PCNSL Study Group 1) study (study patients), the largest prospective multicenter trial on PCNSL, comprising 551 patients. Median follow up was 68 months (range 1–141) in non-study patients and 51 months (1–105) in study patients. Twenty-seven/34 (79.4%) non-study patients received high dose methotrexate (HDMTX), while seven/34 (20.6%) with a glomerular filtration rate (GFR) < 50 mL/min did not. Median overall survival (OS) was six months (95% confidence interval [CI] 0–21 months) in those 34 non-study patients. The 27 non-study patients treated with HDMTX were compared with 526/551 G-PCNSL-SG-1 study patients who had received HDMTX as well. Median OS was 20 months (95% CI 0–45)/21 months (95% CI 18–25) in 27 non-study/526 study patients (p = 0.766). Favorable prognostic factors in non-study patients were young age, application of HDMTX and early response on magnet resonance imaging (MRI). If HDMTX-based chemotherapy can be applied, long-term disease control is possible even in patients not qualifying for clinical trials. Initial response on early MRI might be useful for decision on treatment continuation.

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Initial renal histology and early response predict outcomes of Brazilian lupus nephritis patients

Lupus ◽

10.1177/0961203319890681 ◽

2019 ◽

Vol 29 (1) ◽

pp. 83-91

Author(s):

G Vajgel ◽

C B L Oliveira ◽

D M N Costa ◽

M A G M Cavalcante ◽

L M Valente ◽

...

Keyword(s):

Kidney Disease ◽

Lupus Nephritis ◽

Confidence Interval ◽

Odds Ratio ◽

Estimated Glomerular Filtration Rate ◽

Interstitial Fibrosis ◽

End Stage ◽

Class Iv

Objective We analyzed baseline and follow-up characteristics related to poorer renal outcomes in a Brazilian cohort of admixture race patients with lupus nephritis. Methods Overall, 280 outpatients with a diagnosis of systemic lupus erythematosus and previous kidney biopsy of lupus nephritis were recruited from August 2015 to December 2018 and had baseline laboratory and histologic data retrospectively analyzed; patients were then followed-up and data were recorded. The main outcome measure was the estimated glomerular filtration rate at last follow-up. Secondary analyses assessed the impact of initial kidney histology and treatment in long-term kidney survival. Results Median duration of lupus nephritis was 60 months (interquartile range: 27–120); 40 (14.3%) patients presented progressive chronic kidney disease (estimated glomerular filtration rate <30 and ≥10 ml/min/1.73 m2) or end-stage kidney disease at last visit. Adjusted logistic regression analysis showed that class IV lupus nephritis (odds ratio 14.91; 95% confidence interval 1.77–125.99; p = 0.01) and interstitial fibrosis ≥25% at initial biopsy (odds ratio 5.87; 95% confidence interval 1.32–26.16; p = 0.02), lack of complete or partial response at 12 months (odds ratio 16.3; 95% confidence interval 3.74–71.43; p < 0.001), and a second renal flare (odds ratio 4.49; 95% confidence interval 1.10–18.44; p = 0.04) were predictors of progressive chronic kidney disease. In a Kaplan-Meier survival curve we found that class IV lupus nephritis and interstitial fibrosis ≥25% were significantly associated with end-stage kidney disease throughout follow-up (hazard ratio 2.96; 95% confidence interval 1.3–7.0; p = 0.036 and hazard ratio 4.96; 95% confidence interval 1.9–12.9; p < 0.0001, respectively). Conclusion In this large cohort of admixture race patients, class IV lupus nephritis and chronic interstitial damage at initial renal biopsy together with non-response after 1 year of therapy and relapse were associated with worse long-term renal outcomes.

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Long-term follow-up of the MAINTAIN Nephritis Trial, comparing azathioprine and mycophenolate mofetil as maintenance therapy of lupus nephritis

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2014-206897 ◽

2015 ◽

Vol 75 (3) ◽

pp. 526-531 ◽

Cited By ~ 68

Author(s):

Farah Tamirou ◽

David D'Cruz ◽

Shirish Sangle ◽

Philippe Remy ◽

Carlos Vasconcelos ◽

...

Keyword(s):

Mycophenolate Mofetil ◽

Lupus Nephritis ◽

Positive Predictive Value ◽

Maintenance Therapy ◽

Early Response ◽

Renal Outcome ◽

Proliferative Lupus Nephritis ◽

Long Term Follow Up

ObjectiveTo report the 10-year follow-up of the MAINTAIN Nephritis Trial comparing azathioprine (AZA) and mycophenolate mofetil (MMF) as maintenance therapy of proliferative lupus nephritis, and to test different definitions of early response as predictors of long-term renal outcome.MethodsIn 2014, data on survival, kidney function, 24 h proteinuria, renal flares and other outcomes were collected for the 105 patients randomised between 2002 and 2006, except in 13 lost to follow-up.ResultsDeath (2 and 3 in the AZA and MMF groups, respectively) and end-stage renal disease (1 and 3, respectively) were rare events. Time to renal flare (22 and 19 flares in AZA and MMF groups, respectively) did not differ between AZA and MMF patients. Patients with good long-term renal outcome had a much more stringent early decrease of 24 h proteinuria compared with patients with poor outcome. The positive predictive value of a 24 h proteinuria <0.5 g/day at 3 months, 6 months and 12 months for a good long-term renal outcome was excellent (between 89% and 92%). Inclusion of renal function and urinalysis in the early response criteria did not impact the value of early proteinuria decrease as long-term prognostic marker.ConclusionsThe long-term follow-up data of the MAINTAIN Nephritis Trial do not indicate that MMF is superior to AZA as maintenance therapy in a Caucasian population suffering from proliferative lupus nephritis. Moreover, we confirm the excellent positive predictive value of an early proteinuria decrease for long-term renal outcome.Trial registration numberNCT00204022.

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Magnetic Resonance Imaging during Proton Therapy Irradiation Allows for the Early Response Assessment of Pediatric Chordoma

Diagnostics ◽

10.3390/diagnostics11061117 ◽

2021 ◽

Vol 11 (6) ◽

pp. 1117

Author(s):

Sabina Vennarini ◽

Dante Amelio ◽

Stefano Lorentini ◽

Giovanna Stefania Colafati ◽

Antonella Cacchione ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Proton Therapy ◽

Treatment Plan ◽

Surgical Excision ◽

Response Assessment ◽

Early Response ◽

High Dose ◽

Resonance Imaging ◽

Early Response Assessment

Chordoma in pediatric patients is very rare. Proton therapy has become a gold standard in the treatment of these neoplasms, as high dose escalation can be achieved regarding the target while maximizing the sparing of the healthy tissues near the tumor. The aim of the work was to assess the evolution of morphological sequences during treatment using T1/T2-weighted magnetic resonance imaging (MRI) for the early response assessment of a classic chordoma of the skull base in a pediatric patient who had undergone surgical excision. Our results demonstrated a significant quantitative reduction in the residual nodule component adhered to the medullary bulb junction, with an almost complete recovery of normal anatomy at the end of the irradiation treatment. This was mainly shown in the T2-weighted MRI. On the other hand, the classic component of the lesion was predominantly present and located around the tooth of the axis. The occipital condyles were morphologically and dimensionally stable for the entire irradiation period. In conclusion, the application of this type of monitoring methodology, which is unusual during the administration of a proton treatment for chordoma, highlighted the unexpected early response of the disease. At the same time, it allowed the continuous assessment of the reliability of the treatment plan.

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