Lupus nephritis is a major cause of morbidity and mortality arising from systemic lupus erythematous. It is generally acknowledged that the presence of diffuse proliferative lupus nephritis(DPLN) is highly predictive of a poor prognosis in terms of renal and patient out- come on survival. The objective of this study was to evaluate the clinicopathologic characteristics, renal out- come according to therapeutic regimen, and prognostic factors of biopsy-proven diffuse proliferative lupus nephritis. Among the biopsy-proven lupus nephritis patients who were admitted to Yonsei University Medical Center from January 1986 to June 1997, 36 patents who were diagnosed DPLN by renal biopsy and treated for at least 6 months and regularly followed-up for at least 12 months were included. We retrospec-tively reviewed the medical recorders. Patients were treated with steroid regimen with or without cyclo-phosphamide. According to the therapeutic response, patients were divided into two groups : a therapeutic response group(n=24), and a therapeutic non-response groupLupus nephritis developed at a mean 9.7 months after SLE diagnosis and mean duration of nephritis was 39.2 months. Mean serum creatinine was 1.6mg/ dL, 24 hour proteinuria was 4,873mg, and anti-DNA antibody was positive in 8196 of patients at the time of renal biopsy. Activity index and chronicity index were 10.4 and 2.8, respectively. Overall 5 year renal survival rate was 7596 and no difference between steroid single therapy and cyclophosphamide combination therapy was observed. Factors affecting therapeutic response included delayed development of nephritis(3.1 vs 13.8 months, p<0.05) and elevated serum creatinine level(0.9 vs 1.9mg/dL, p<0.05), which were associated with poor therapeutic response. Other clinicopathologic, biochemical and immunologic parameters were not different between the therapeutic response group and the therapeutic non-response group. In conclusion, delayed development of lupus nephritis and elevated serum creatinine at nephritis presentation are poor prognostic factors of DPLN, but further randomized prospective study{including divided cytoxan intravenous pulse therapy and oral therapy, with long-term follow-up) is necessary.
Academic Medical Centers ; Biopsy ; Creatinine ; Cyclophosphamide ; Diagnosis ; Follow-Up Studies ; Humans ; Lupus Nephritis* ; Medical Records ; Mortality ; Nephritis ; Prognosis ; Proteinuria ; Survival Rate

SLE is an acute and/or chronic disease of a complex autoimmune nature affecting the skin, joints, serous membranes, kidney, central nervous system, cardiovascular system, and other organs of the body. Cardiac abnormalities are one of the most important clinical manifestations of SLE, contributing significantly to the morbidity and morality of the disease. We report a woman, a 30-year-old with SLE, who developed symptoms and echocardiographic signs of dilated cardiomyopathy. She had a history of acute myocardial infartion 7 months ago and has taken cyclophosphamide pulse therapy owing to lupus nephritis. The diagnosis of the condition was based only on clinical and echocardiographic grounds without coronary angiography or endomyocardial biopsy. She received high dose steroid (methylprednisolone pulse therapy 1mg/kg for 3 days) and conservative therapy for heart failure. The association of SLE with dilated cardiomyopathy has not been described with review of previous literatures. For the probable causes of her dilated cardiomyopathy, we suggest that they are due to coronary arteritis or cyclophosphamide-induced myocarditis with regard to her past history of myocardial infartion and cyclophosphamide therapy for lupus nephritis. The incidence of dilated cardiomyopathy, complicated with SLE is rare, but it has ap increased risk for unfavorable outcome. It reserves to pioneer the method of early diagnosis and management which will improved the morbidity and mortality of the disease.
Adult ; Arteritis ; Biopsy ; Cardiomyopathy, Dilated* ; Cardiovascular System ; Central Nervous System ; Chronic Disease ; Coronary Angiography ; Cyclophosphamide ; Diagnosis ; Early Diagnosis ; Echocardiography ; Female ; Heart Failure ; Humans ; Incidence ; Joints ; Kidney ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Morals ; Mortality ; Myocarditis ; Serous Membrane ; Skin

BACKGROUND/AIMS: We investigated whether transthoracic echocardiography-suspected pulmonary hypertension (PH) affects survival in systemic lupus erythematosus (SLE) patients and examined factors associated with PH occurrence and survival. METHODS: This retrospective single-center study included 154 Korean SLE patients fulfilling the American College of Rheumatology criteria (January 1995 to June 2013). Student t test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons. RESULTS: A total of 35 SLE patients with PH (SLE/PH+) and 119 without PH (SLE/PH-) were analyzed. Higher percentages of interstitial lung disease, Raynaud's phenomenon (RP), World Health Organization functional classification III/IV, and cardiomegaly were found in SLE/PH+ compared to SLE/PH-. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was significantly higher in SLE/PH+ (2.46 +/- 1.245 vs. 1.00 +/- 1.235), whereas survival rates were significantly higher in SLE/PH- in log-rank tests (p = 0.001). In multivariate analysis, the adjusted mortality hazard ratio (HR) for SLE/PH+ patients was 3.10. Subgroup analysis demonstrated a higher percentage of lupus nephritis in the SLE/PH+ patients who died (p = 0.039) and low complement-3 levels (p = 0.007). In univariate analysis, the mortality HR for SLE/PH+ patients with lupus nephritis was 4.62, whereas the presence of RP decreased the mortality risk in multivariate analysis; adjusted HR, 0.10. CONCLUSIONS: PH is an independent factor predicting survival in SLE patients. The presence of lupus nephritis resulted in an increased trend for mortality, whereas coexistence of RP was associated with a better survival prognosis in SLE/PH+ patients.
Adolescent ; Adult ; Cardiomegaly/diagnosis/epidemiology ; Chi-Square Distribution ; Female ; Humans ; Hypertension, Pulmonary/diagnosis/*mortality ; Kaplan-Meier Estimate ; Lung Diseases, Interstitial/diagnosis/mortality ; Lupus Erythematosus, Systemic/diagnosis/*mortality ; Lupus Nephritis/diagnosis/mortality ; Male ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models ; Raynaud Disease/diagnosis/epidemiology ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Young Adult

PURPOSE: Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. METHODS: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. RESULTS: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1 (2-18) years old. FANA(95.0%), anti-ds DNA antibody (87.5%), malar rash (80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria (75.0%), nephrotic syndrome (55.0%), and microscopic hematuria alone (15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was 51.8 40.5 months. During 51.8+/-40.5 months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. CONCLUSION: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.
Acute Kidney Injury ; Adult ; Autoimmune Diseases ; Biopsy ; Child* ; Classification ; Cyclophosphamide ; Diagnosis ; DNA ; Exanthema ; Female ; Follow-Up Studies ; gamma-Globulins ; Gonads ; Hematuria ; Humans ; Lupus Nephritis* ; Male ; Mortality ; Nephrotic Syndrome ; Plasma Exchange ; Prednisolone ; Prognosis ; Proteinuria ; Retrospective Studies ; Risk Factors ; Treatment Outcome*

BACKGROUND/AIMS: To compare the clinical characteristics and outcomes of systemic lupus erythematosus between male and female Korean patients. METHODS: A retrospective analysis was performed at a single tertiary hospital from August 1994 to May 2010. Male patients were matched with two to three female patients based on age and disease duration. Organ damage was assessed using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR DI). RESULTS: Fifty-three male patients were compared with 150 female patients. Renal disorders were found more frequently in male patients at disease onset (p < 0.001); the adjusted odds ratio (OR) demonstrated a significant sex preponderance for renal manifestations (OR, 3.26; 95% confidence interval [CI], 1.62 to 6.57). Diffuse proliferative lupus nephritis and end-stage renal disease requiring dialysis during the disease course were more prevalent in male patients (p = 0.025 and p < 0.001, respectively). The risk for requiring long-term dialysis was significantly higher in male than in female patients (OR, 4.02; 95% CI, 1.07 to 15.06), as was the mean SLICC/ACR DI (1.55 +/- 1.35 vs. 1.02 +/- 1.57, respectively; p = 0.028). CONCLUSIONS: Our data demonstrate that Korean patients with lupus have characteristics similar to those of cohorts reported previously. Male patients had significantly higher incidences of renal manifestations and organ damage.
Adult ; *Asian Continental Ancestry Group ; Female ; Humans ; Immunosuppressive Agents/therapeutic use ; Incidence ; Kaplan-Meier Estimate ; Kidney Failure, Chronic/diagnosis/ethnology/therapy ; Lupus Erythematosus, Systemic/diagnosis/*ethnology/mortality/therapy ; Lupus Nephritis/diagnosis/ethnology/therapy ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Prevalence ; Prognosis ; Renal Dialysis ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Sex Distribution ; Sex Factors ; Tertiary Care Centers ; Time Factors ; Young Adult