The new revised classification of glomerulonephritis in systemic lupus erythematosus under the auspice of the International Society of Nephrology and the Renal Pathology Society (ISN/ RPS) was proposed in 2003. The revised classification preserves the simplicity of the original WHO classification, incorporates selective refinements concerning activity and chronicity from the 1982 and 1995 revisions, and adds a number of new modifications. Overall, it bears a strong similarity to the 1974 classification, but introduces several important modifications concerning quantitative and qualitative differences between class III and IV lesions. The new classification provides a clear and unequivocal description of the various lesions and classes of lupus nephritis as well as definitions for diagnostic terms. This review is introduced the ISN/RPS 2003 classification which will facilitates accurate communication between pathologists and clinicians.
Biopsy ; Classification* ; Glomerulonephritis ; Kidney ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Nephrology ; Pathology*

Biopsy ; methods ; Glomerulonephritis, IGA ; pathology ; Humans ; Kidney ; pathology ; Kidney Diseases ; pathology ; Lupus Nephritis ; pathology

OBJECTIVETo study feasibility of Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2000) and British Isles Lupus Assessment Group 2004 (BILAG-2004) scoring systems for assessing renal disease activity in children with lupus nephritis (LN).

METHODSThe clinical data of 159 children with systemic lupus erythematosus (SLE) and LN were collected, and disease activity was assessed by SLEDAI-2000 and BILAG-2004 scoring systems. The correlations between SLEDAI-2000 and BILAG-2004 scores and 24-hour urinary protein excretion and renal pathology index were analyzed. The SLEDAI-2000 and BILAG-2004 scoring systems were evaluated using ROC curve.

RESULTSApproximately one third (31.5%) of the 159 children had a moderate level of 24-hour urinary protein excretion. Among the 37 patients undergoing renal biopsy, 46.0% had diffuse LN (type Ⅳ). 24-hour urinary protein excretion was positively correlated with both SLEDAI-2000 (r=0.36, P<0.05) and BILAG-2004 scores (r= 0.39, P<0.05). Children with types Ⅰ, Ⅱ, Ⅲ, and Ⅳ LN had pathology activity index (AI) which positively correlated with SLEDAI-2000 scores (r=0.86, 0.88, 0.84, 0.77 respectively; P<0.05) and BILAG-2004 scores (r= 0.88, 0.98, 0.86, 0.89 respectively; P<0.05). SLEDAI-2000 score showed the best correlation with AI in patients with type Ⅱ LN, followed by those with type Ⅰ LN. BIILAG-2004 score showed the best correlation with AI in patients with type Ⅱ LN, followed by those with type Ⅳ LN. The BILAG-2004 scoring system had an area under the ROC curve (AUC) of 0.93, and the SLEDAI-2000 scoring system had an AUC of 0.88.

CONCLUSIONSBILAG-2004 and SLEDAI-2000 scoring systems can be used to assess renal disease activity of patients with LN. The BILAG-2004 scoring system can provide more reliable and comprehensive assessment.

Adolescent ; Child ; Feasibility Studies ; Female ; Humans ; Kidney ; pathology ; Lupus Erythematosus, Systemic ; pathology ; Lupus Nephritis ; pathology ; Male ; Proteinuria ; classification ; ROC Curve

BACKGROUNDRelapses occur frequently in patients with lupus nephritis. Renal biopsy is the gold standard for assessing renal activity and hence guiding the treatment. Whether repeat renal biopsy is helpful during flares of lupus nephritis remains inconclusive. In the present study, we retrospectively reviewed the patients with lupus nephritis who had more than one renal biopsy with the hope to find the clinical value of repeat biopsy.

METHODSPatients who had a diagnosis of lupus nephritis and two or more renal biopsies were selected from the database of the patient pathology registration at this renal division. Renal biopsy was evaluated according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification of lupus nephritis. The pathological patterns and treatment regimens were analyzed after a repeat biopsy.

RESULTSWe identified 44 systemic lupus erythematosus patients with serial renal biopsies. In total, there were 94 renal biopsies. Overall, the pathological transition occurred in 64% instances according to the ISN/RPS class. When the transition was analyzed according to proliferative, membranous or mix lesions, it showed different profile: 35% in patients with proliferative lesion, 23.5% patients with mix lesions, 100% in patients with pure membranous lesion. The pathological transition could not be predicted by any clinical characteristics. After the repeat renal biopsy, 34% of patients had a change in their treatment regimens.

CONCLUSIONSThe pathological conversion was very prevalent in patients with lupus nephritis. However, the transitions became less prevalent when they were analyzed according to pure membranous, proliferative, and mix lesion. Repeat biopsy might be helpful to avoid unnecessary increased immunosuppression therapy.

Adult ; Aged ; Biopsy ; Female ; Humans ; Kidney ; pathology ; Lupus Nephritis ; diagnosis ; pathology ; Male

OBJECTIVES: To investigate the clinical characteristics, pathological features, treatment regimen, and prognosis of children with lupus nephritis (LN) and thrombotic microangiopathy (TMA), as well as the treatment outcome of these children and the clinical and pathological differences between LN children with TMA and those without TMA. METHODS: A retrospective analysis was conducted on 12 children with LN and TMA (TMA group) who were admitted to the Department of Nephrology, Children's Hospital of Nanjing Medical University, from December 2010 to December 2021. Twenty-four LN children without TMA who underwent renal biopsy during the same period were included as the non-TMA group. The two groups were compared in terms of clinical manifestations, laboratory examination results, and pathological results. RESULTS: Among the 12 children with TMA, 8 (67%) had hypertension and 3 (25%) progressed to stage 5 chronic kidney disease. Compared with the non-TMA group, the TMA group had more severe tubulointerstitial damage, a higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score at onset, and higher cholesterol levels (P<0.05). There were no significant differences between the two groups in the percentage of crescent bodies and the levels of hemoglobin and platelets (P>0.05). CONCLUSIONS There is a higher proportion of individuals with hypertension among the children with LN and TMA, as well as more severe tubulointerstitial damage. These children have a higher SLEDAI score and a higher cholesterol level.
Child ; Humans ; Lupus Nephritis/complications* ; Kidney/pathology* ; Retrospective Studies ; Thrombotic Microangiopathies/therapy* ; Prognosis ; Hypertension/complications* ; Cholesterol ; Lupus Erythematosus, Systemic

OBJECTIVE: This study was designed to investigate the clinical outcomes of proliferative lupus nephritis and to identify the predictive factors of remission and relapse of proliferative lupus nephritis after intravenous cyclophosphamide (IVCYC) pulse therapy. MEHTODS: Seventy-four patients with proliferative lupus nephritis that had been diagnosed by renal biopsy and treated with IVCYC pulse therapy were studied. Their demographic data, clinical manifestations, laboratory findings, disease activity index, damage index, activity and chronicity indices of renal pathology, and treatment modalities were evaluated. Clinical outcomes of lupus nephritis were assessed by defined criteria. RESULTS: Remission or response were achieved in 79.7% of patients with proliferative lupus nephritis (remission in 32.4% and response in 47.3%, respectively), and 30.5% of those with remission or response experienced relapse or flare of lupus nephritis (relapse in 20.8% of those with remission and flare in 37.1% of those with response) after IVCYC pulse therapy. High creatinine clearance at diagnosis of lupus nephritis, short lag time from diagnosis of lupus nephritis to initiation of immunosuppressive treatment, and long-term cyclophosphamide pulse therapy were the independent predictive factors for remission or response. Long lag time from completion of immunosuppressive treatment to onset of remission or response, and incomplete cyclophosphamide were the independent risk factors for relapse or flare of lupus nephritis. CONCLUSION: Good renal function and early initiation of long-term IVCYC pulse therapy are important in induction of remission or response, while delayed remission or response and incomplete immunosuppressive treatment is strongly associated with poor outcome.
Biopsy ; Creatinine ; Cyclophosphamide* ; Diagnosis ; Humans ; Lupus Nephritis* ; Pathology ; Recurrence* ; Remission Induction ; Risk Factors

OBJECTIVE: To observe the tubulointerstitial damage (TID) in lupus nephritis (LN) and investigate the relationship between autoantibodies and TID in lupus nephritis. METHODS: This cross-sectional study was conducted in a comprehensive tertiary hospital in Peking University Shenzhen Hospital. From March 2012 to July 2021, LN patients who performed renal biopsy were enrolled in the study. Clinical, laboratory and pathology data were collected. We classified the patients into none-or-mild group and moderate-to-severe groups according to the severity of interstitial fibrosis (IF) /tubular atrophy (TA) or tubulointerstitial inflammation (TII). The t test, U test and Chi-square test were used for statistical analysis as appropriate. RESULTS: A total of 226 patients were included, of who 190 (84%) were female with a median age of 32 (26, 39) years. 89% (201/226) of the patients who pathologically proved to be proliferative LN by renal biopsy. The frequency of moderate-to-severe TII and moderate-to-severe IF/TA was 30% (67/226) and 34% (76/226) respectively. For autoantibodies, the patients with moderate-to-severe TII had a lower rate of positive serum anti-ribonucleoprotein (anti-RNP) antibodies than the patients with none-or-mild TII (34% vs. 51%), and moderate-to-severe IF/TA had a lower rate of positive anti-ribosomal P protein (anti-P) antibodies than patients with none-or-mild IF/TA (19% vs. 33%). For other clinical indicators, the patients with moderate-to-severe TII and moderate-to-severe IF/TA were more often combined with proliferative LN, hypertension and anemia than the patients with none-or-mild TII and none-or-mild IF/TA, respectively. The patients with moderate-to-severe TII had higher serum creatinine values and lower glomerular filtration rates than the patients with none-or-mild TII. The patients with moderate-to-severe IF/TA had higher serum creatinine values, and lower glomerular filtration rates than the patients with none-or-mild IF/TA. CONCLUSION In patients with LN in Southern China, anti-RNP antibodies and anti-P antibodies may be potential protective factors for TII and IF/TA, respectively. More studies are needed to identify the risk factors of lupus patients with TID and investigate the correlation between autoantibodies and TID, which are critical for developing better preventive and therapeutic strategies to improve the survival rate of LN.
Humans ; Female ; Male ; Lupus Nephritis ; Kidney/pathology* ; Creatinine ; Cross-Sectional Studies ; Inflammation ; Antibodies, Antinuclear ; Autoantibodies

OBJECTIVETo analyze the clinical and immunological features of children with lupus nephritis (LN).

METHODSChart records of 40 (4 male and 36 female) LN children who were admitted consecutively between January, 2005 and December, 2010 were reviewed. The baseline demographic, pathological and immunological data were analyzed.

RESULTSIn the 40 LN patients analyzed, the mean age of the disease onset was 10.6 ± 2.6 (range from 2.6 to 14.3) years, and 35 cases (88%) were school-age children. Proteinuria was detected in all 40 cases, including nephrotic-range proteinuria in 12 (30%) cases, and isolated proteinuria in 9 (22%) cases. Twenty-six (65%) patients had varying degrees of hematuria. Acute nephritis was the most common sub-type, accounting for 47% of the total cases. Among the 39 cases undergoing renal biopsy, 3 were unclassified and the remaining 36 were classified, respectively, as type IV LN (50%, 18 cases), type II LN (22%, 8 cases). In the histopathologcally classified case, 100% were antinuclear antibody-positive, 61% were anti-dsDNA-positive, and 89% showed varying degrees of decrease in serum C3 and C4 concentrations. Following treatment for 6 months, a high LN remission rate (95%) was achieved; the acute renal activity index remained higher in IV, V+III and V+IV subtypes than in other subtypes, while the chronic index and the degree of tubulointerstitial damage were not different between histopathological subtypes.

CONCLUSIONSThe clinical manifestations of LN children are diverse. Clinically, acute nephritis is the most common form of LN in children. Histopathologically, type IV is the most frequent subtype of LN. Early treatment may result in significant disease remission.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Lupus Nephritis ; drug therapy ; immunology ; pathology ; Male ; Retrospective Studies

BackgroundLupus nephritis (LN) is classified by renal biopsy into proliferative and nonproliferative forms, with distinct prognoses, but renal biopsy is not available for every LN patient. The present study aimed to establish an alternate tool by building a predictive model to evaluate the probability of proliferative LN.

MethodsIn this retrospective cohort with biopsy-proven LN, 382 patients in development cohort, 193 in internal validation cohort, and 164 newly diagnosed patients in external validation cohort were selected. Logistic regression model was established, and the concordance statistics (C-statistics), Akaike information criterion (AIC), integrated discrimination improvement, Hosmer-Lemeshow test, and net reclassification improvement were calculated to evaluate the performance and validation of models.

ResultsThe prevalence of proliferative LN was 77.7% in the whole cohort. A model, including age, gender, systolic blood pressure, hemoglobin, proteinuria, hematuria, and serum C3, performed well on good-of-fit and discrimination in the development chohort to predict the risk of proliferative LN (291 for AIC and 0.84 for C-statistics). In the internal and external validation cohorts, this model showed good capability for discrimination and calibration (0.84 and 0.82 for C-statistics, and 0.99 and 0.75 for P values, respectively).

ConclusionThis study developed and validated a model including demographic and clinical indices to evaluate the probability of presenting proliferative LN to guide therapeutic decisions and outcomes.

Adult ; Biopsy ; Female ; Humans ; Lupus Nephritis ; pathology ; Male ; Nomograms ; Prognosis ; Retrospective Studies ; Risk Factors ; Young Adult

OBJECTIVE: To investigate the influence of chemokine like factor-1(CKLF-1) on the genesis and development of lupus nephritis. METHODS: Immunohistochemistry stain was employed on the 34 lupus nephridial tissues and 10 nephridial tissues of control group. And light microscopy was applied to observe the percentage of CKLF-1 positive cells of renal gromerulus and tubule. RESULTS: The percentages of CKLF-1 positive cells of renal gromerulus and tubule were 10.4%+/-1.5% and 48.9%+/-4.3% in the lupus nephritis group, and the percentages of control group were 4.6%+/-1.1% and 4.3%+/-0.9%. There was significant difference between 2 groups (P<0.01). And there was positive correlation between the percentage of CKLF-1 positive cells of renal gromerulus and tubule and lupus active index (r=0.74 and r=0.53, respectively, P<0.05). CONCLUSION CKLF-1 plays a role in the genesis and development of lupus nephritis.
Adult ; Chemokines ; biosynthesis ; Female ; Humans ; Immunohistochemistry ; Kidney ; chemistry ; pathology ; Lupus Nephritis ; metabolism ; pathology ; MARVEL Domain-Containing Proteins ; Male