The Comparison of the Associations of Lipoprotein(a) and the Atherogenic Index of Plasma With Coronary Artery Calcification in Patients Without High LDL-C: A Comparative Analysis

Objective@#Lipoprotein(a) (Lp[a]) and the atherogenic index of plasma (AIP) have been reported as predictive markers of coronary artery calcium (CAC). However, previous studies demonstrated that the cardiovascular risk associations with Lp(a) are attenuated in patients with low-density lipoprotein cholesterol (LDL-C) levels ≤135 mg/dL. However, few articles have identified the risk factors of CAC in patients without high LDL-C. Therefore, we performed this study to investigate the association of Lp(a) and AIP with CAC in patients with LDL-C levels ≤135 mg/dL. @*Methods@#This study included 625 lipid-lowering agent naive patients with LDL-C levels ≤135 mg/dL who underwent coronary computed tomographic angiography. We performed multivariate logistic regression analysis to evaluate the risk factors for a coronary artery calcium score (CACS) >0, CACS ≥400, and CAC ≥90th percentile. @*Results@#The mean age of the patients was 55.0±7.9 years and their mean LDL-C level was 94.7 ±23.3 mg/dL. Multivariate regression analysis showed that age, male sex, diabetes, hypertension, Lp(a), and AIP were independent predictors of CAS>0. Age, male sex, and diabetes were independent predictors of CACS≥400. Diabetes, hypertension, and AIP were independent predictors of CAC ≥90th percentile (all p<0.05). Unlike Lp(a), higher AIP tertiles were associated with significantly higher CAC percentiles and greater proportions of patients with CACS ≥400 and CAC ≥90th percentile. @*Conclusion@#In patients without high LDL-C, AIP could be a more reliable predictor of CAC than Lp(a).

Prognostic Value of Procalcitonin in Pneumonia among Patients Admitted to Intensive Care Unit / 고신대학교의과대학학술지

OBJECTIVES: Pneumonia is one of the leading causes of death in the intensive care unit (ICU). Many biomarkers for predicted prognosis have been suggested; among these, procalcitonin (PCT) is known to increase in cases of bacterial infection. However, there have been many debates regarding whether PCT is an appropriate prognostic marker for pneumonia. Therefore, we investigated whether PCT can serve as a biomarker for pneumonia, and compared it with CURB-65, which is a known tool for predicting the prognosis of pneumonia. METHODS: Levels of PCT and CURB-65 scores were compared between 30-day non-survival (n = 30) and survival (n = 101) patients. Relationships between PCT and CURB-65 were determined by using linear regression analysis, as well as by using receiver operating characteristic (ROC) curve analysis and calculation of the area under the curve (AUC). High and low PCT groups were compared. RESULTS: High PCT and high CURB-65 score were positively associated with 30-day mortality. For the prediction of 30-day mortality, initial PCT and CURB-65 exhibited AUCs of 0.63 and 0.66; these were not significantly different (P = 0.132). We found that the high PCT group had a higher rate of initial treatment failure (91%, P = 0.004). CONCLUSIONS: Initial PCT can be a prognostic biomarker for mortality in severe pneumonia, similar to the CURB-65 score. Initial high PCT was positively associated with initial treatment failure.

Retrospective Observational Dose-Titration Study of Subjects with Type 2 Diabetes Mellitus with Inadequate Glycemic Control on 15 mg of Pioglitazone / 임상당뇨병

BACKGROUND: The 30 mg pioglitazone tablet was recently introduced in Korea; no study has yet compared its glucose-lowering or weight gain effects to the 15 mg tablet in Korean patients with type 2 diabetes mellitus (T2DM). METHODS: The electronic medical records of 45 patients with T2DM with glycated hemoglobin (HbA1c) levels > 7.0%, despite taking 15 mg/day pioglitazone and a stable dose of other diabetes drugs for 3 months, were retrospectively reviewed. RESULTS: After dose up-titration, HbA1c levels decreased at 3- and 6-month follow-ups compared with baseline (8.5% at baseline vs. 8.2% at 3 months vs. 7.9% at 6 months; baseline vs. 3 months, P = 0.106; baseline vs. 6 months, P = 0.005; 3 months vs. 6 months, P = 0.096). In the subgroup analysis of 36 patients taking pioglitazone, sulfonylurea, and metformin, HbA1c levels also decreased at 3- and 6-month follow-ups compared with baseline (8.5 % vs. 8.2 % vs. 7.9%; baseline vs. 3 months, P = 0.289; baseline vs. 6 months, P = 0.014; 3 months vs. 6 months, P = 0.232). There was no significant body weight change (70.8 kg vs. 70.7 kg vs. 71.0 kg). CONCLUSION: Up-titrating from 15 mg to 30 mg of pioglitazone in patients with inadequate glycemic control (HbA1c > 9%) who were also taking sulfonylurea and metformin showed additive glucose-lowering effects without significant weight gain in Korean patients with T2DM.

Flecainide-Induced Torsade de Pointes Successfully Treated with Intensive Pharmacological Therapy

Flecainide acetate is a potent class IC anti-arrhythmic drug with a major sodium channel blocking effect. Flecainide toxicity can cause myocardial impairment and precipitate circulatory collapse. It may also result in life-threatening arrhythmia, although cases of flecainide-induced torsades de pointes are rare. Furthermore, the electrical and hemodynamic deteriorations observed during flecainide toxicity may not respond to conventional treatments. In the present study, we report the case of a 20-year-old Korean man with flecainide poisoning, who presented with hypotension. The patient was successfully treated with sodium bicarbonate, amiodarone, MgSO₄, and lidocaine, with no recourse to extracorporeal therapy. Although there is no standard therapy for flecainide toxicity, this report demonstrates that intensive pharmacological treatment is beneficial in cases of flecainide overdose.

Effect of autologous platelet-rich plasma application on cutaneous wound healing in dogs

This study was conducted to identify the effectiveness of platelet-rich plasma (PRP) and efficacy of intralesional injection as a method of application to acute cutaneous wounds in dogs. Healthy adult beagles (n = 3) were used in this study. Autologous PRP was separated from anticoagulant treated whole blood in three dogs. Cutaneous wounds were created and then treated by intralesional injection of PRP in the experimental group, while they were treated with saline in the control group on days 0, 2 and 4. The healing process was evaluated by gross examination throughout the experimental period and histologic examination on day 7, 14 and 21. In PRP treated wounds, the mean diameter was smaller and the wound closure rate was higher than in the control. Histological study revealed that PRP treated wounds showed more granulation formation and angiogenesis on day 7, and faster epithelialization, more granulation formation and collagen deposition were observed on day 14 than in control wounds. On day 21, collagen deposition and epithelialization were enhanced in PRP treated groups. Overall, PRP application showed beneficial effects in wound healing, and intralesional injection was useful for application of PRP and could be a good therapeutic option for wound management in dogs.

A study of experimental autoimmune encephalomyelitis in dogs as a disease model for canine necrotizing encephalitis

In the present study, the use of dogs with experimental autoimmune encephalomyelitis (EAE) as a disease model for necrotizing encephalitis (NE) was assessed. Twelve healthy dogs were included in this study. Canine forebrain tissues (8 g), including white and grey matter, were homogenized with 4 mL of phosphate-buffered saline for 5 min in an ice bath. The suspension was emulsified with the same volume of Freund's complete adjuvant containing 1 mg/mL of killed Mycobacterium tuberculosis H37Ra. Under sedation, each dog was injected subcutaneously with canine brain homogenate at four sites: two in the inguinal and two in the axillary regions. A second injection (booster) was administered to all the dogs using the same procedure 7 days after the first injection. Clinical assessment, magnetic resonance imaging, cerebrospinal fluid analyses, necropsies, and histopathological and immunohistochemical examinations were performed for the dogs with EAE. Out of the 12 animals, seven (58%) developed clinically manifest EAE at various times after immunization. Characteristics of canine EAE models were very similar to canine NE, suggesting that canine EAE can be a disease model for NE in dogs.

Canine model of ischemic stroke with permanent middle cerebral artery occlusion: clinical features, magnetic resonance imaging, histopathology, and immunohistochemistry

The purpose of this study was to identify time-related changes in clinical, MRI, histopathologic, and immunohistochemical findings associated with ischemic stroke in dogs. Additionally, the association of cerebrospinal fluid (CSF) and tissue levels of interleukin (IL)-6 with clinical prognosis was assessed. Ischemic stroke was induced by permanent middle cerebral artery occlusion (MCAO) in nine healthy experimental dogs. The dogs were divided into three groups according to survival time and duration of the experimental period: group A (survived only 1 day), group B (1-week experimental period), and group C (2-week experimental period). Neurologic status was evaluated daily. Magnetic resonance imaging (MRI) was performed according to a predetermined schedule. Concentration of IL-6 in CSF was measured serially after ischemic stroke. Postmortem examination was performed for all experimental dogs. During histopathological examination, variable degrees of cavitation and necrosis due to neuronal cytopathic effects, such as pyknotic nuclei and cytoplasmic shrinkage, were observed on the affected side of the cerebral cortex in all dogs. Immunohistochemistry specific for IL-6 showed increased expression in the ischemic lesions. CSF IL-6 concentrations and ischemic lesion volumes 1 day after ischemic stroke were significantly higher in group A compared to groups B and C.

A Case of Metastatic Low-Grade Endometrial Stromal Sarcoma Treated with Letrozole after Ovarian Ablation by Radiotherapy / Journal of the Korean Cancer Association, 대한암학회지

A 50-year-old woman was admitted to our hospital due to multiple lung nodules detected incidentally on a chest X-ray. A video-assisted thoracoscopic lung biopsy revealed low-grade endometrial stromal sarcoma (LG-ESS). She had undergone a simple hysterectomy 1 year earlier owing to a diagnosis of adenomyosis. A review of her previous hysterectomy specimen showed not endometriosis but LG-ESS. According to the patient's levels of serum follicle stimulating hormone and estradiol, she was in the premenopausal state with retained and normally functioning ovaries. She then underwent ovarian ablation by radiotherapy, after which she was administered 2.5 mg of letrozole once per day. Three months later, the size of the metastatic nodules in both lungs had decreased. The patient was followed up for 24 months while continuing on letrozole, and maintained a partial remission. We report herein on a case of metastatic LG-ESS treated with letrozole after ovarian ablation by radiotherapy.

Acute lymphoblastic leukemia and chronic lymphocytic leukemia in dogs / 동물의과학연구지

An Australian cattle dog (case 1: 6-year-old castrated male) and a Shih-Tzu dog (case 2: 8-year-old castrated male) were referred to the Gyeongsang Animal Medical Center due to anorexia and depression. Physical examinations, complete blood counts, serum chemical analysis, radiography, ultrasonography, and bone marrow biopsy were performed. Upon physical examinations of cases 1 and 2, enlargement of superficial lymph nodes was not identified. Hematologic findings in these dogs included leukocytosis with severe lymphocytosis, anemia, and thrombocytopenia. Upon radiography, both dogs showed splenomegaly. Upon examination of a peripheral blood smear in case 1, immature lymphoid cells, featuring decreased nuclear chromatin condensation and nuclear pleomorphism, were present. Biopsy samples of the bone marrow in case 1 revealed hypercellularity as well as a large number of immature lymphoblastic cells similar in shape to cells in the peripheral blood. The characteristic morphological features of peripheral blood and bone marrow samples in case 2 were small lymphocytes. Thus, the dogs were tentatively diagnosed with acute lymphoblastic leukemia (ALL) and chronic lymphocytic leukemia (CLL), respectively. After diagnosis, the CLL patient was administered chlorambucil and prednisolone therapy. Due to its similarity to human leukemia, the canine leukemia model provides a valuable model for research into human leukemia.

Hematologic and hemodynamic findings during hemodialysis in 13 beagle dogs / 동물의과학연구지

Hemodialysis is an effective therapy for renal failure in veterinary practice. To evaluate hematologic and hemodynamic changes during hemodialysis, 13 dogs were treated with hemodialysis, after which complete blood cell counts (CBC), serum chemistry, and mean systolic blood pressure were analyzed. For CBC, white blood cells (WBC) and platelets underwent significant changes. In serum chemistry, there were significant differences in blood urea nitrogen (BUN), creatinine, total protein, albumin, globulin, amylase, calcium, potassium, and phosphorus contents. Further, mean systolic blood pressure suddenly increased in early hemodialysis and decreased significantly thereafter. During hemodialysis, adverse effects were observed in some dogs as follows: bleeding (1 dog), anemia (2 dogs), leukopenia (8 dogs), thrombocytopenia (2 dogs), and hypotension (1 dog). This study demonstrates hematologic and hemodynamic effects during hemodialysis as well as complications similar to human medicine. Before applying the commercialized human hemodialysis system to canine renal failure patients, we monitored hematologic and hemodynamic findings during hemodialysis in healthy beagle dogs.

Canine model of ischemic stroke with autologous thrombus in three dogs; Magnetic resonance imaging features and histopathological findings / 동물의과학연구지

Ischemic stroke is the most common type of stroke in humans. The purpose of this study was to evaluate the diagnostic value of magnetic resonance imaging (MRI) in a canine model of stroke. Ischemic stroke was induced by using prepared autologous thrombus. The dogs were placed in lateral recumbency on the operation table and the cervical area of each dog was sterilized by using alcohol. After making a cervical incision, the common carotid artery and internal carotid artery (a branch of the common carotid artery that supplies an anterior part of the brain) were exposed. A 200 microL injection of the autologous thrombus prepared 24 hr prior to surgery was delivered with a 20 gauge venous catheter through an internal carotid artery. After successful delivery of the autologous thrombus, the venous catheter was removed, and the cervical incision was sutured. Neurologic signs including generalized seizures, tetraparesis, and altered mental status, were observed in all 3 dogs after induction of ischemic stroke and the signs manifested immediately after awakening from anesthesia. T1- and T2-weighted images and fluid-attenuated inversion recovery (FLAIR) images of the brain were acquired 1 day before and 1 day after surgery. On the day following ischemic stroke induction, MRI revealed multifocal lesions in the cerebral cortex and subcortex such as T1 hypointensity, T2 hyperintensity, FLAIR hyperintensity, and diffusion-weighted hyperintensity in all 3 dogs. Upon postmortem examination, ischemic lesions were found to be consistent with the MRI findings and they were unstained with 2% triphenyltetrazolium chloride. Histologic features of the earliest neuronal changes such as cytoplasmic eosinophilia with pyknotic nuclei were identified. Neuropil spongiosis and perivascular cuffing were also prominently observed at the infarcted area. The present study demonstrated the features of MRI and histopathologic findings in canine ischemic stroke models.

Evaluation of the Clinical Significance of Ketonuria

BACKGROUND: Urine ketone test is commonly used to screen for diabetic ketoacidosis (DKA). Ketonuria also develops in patients with disease conditions other than DKA. However, the prevalence of DKA in patients with ketonuria is not known. We investigated the prevalence of ketonuria and characteristics of patients with ketonuria and estimated the prevalence of DKA among them to study the clinical significance of ketonuria as an indicator of DKA. METHODS: We studied 1,314 adult and 1,027 pediatric patients who underwent urinalysis. The prevalence of ketonuria in the different groups of patients, classified according to the types of their visits to the institution, was investigated, and the relationships between ketonuria and albuminuria, glycosuria, and bilirubinuria were evaluated. RESULTS: The overall prevalence of ketonuria was 9.1%. The prevalences of ketonuria in adult and pediatric patients were 4.3% and 15.2%, respectively. The prevalences of ketonuria were the highest in the adult (9.7%) and pediatric (28%) patients in the group that had visited the emergency department. Among patients with ketonuria, 7% adult and 3.8% pediatric patients showed glycosuria. CONCLUSIONS: This study showed that the prevalence of DKA in patients with ketonuria, defined as the simultaneous presence of ketone bodies and glucose in urine, was only 7%. Therefore, we concluded that ketonuria might be clinically significant as an indicator of acute or severe disease status rather than of DKA.

The Comparison between Particle Agglutination Assay and Enzyme Immunoassay in the Detection of Mycoplasma pneumoniae-Specific Antibody

BACKGROUND: The two common serological test methods used for initial diagnosis of acute Mycoplasma pneumoniae (MP) pneumonia are particle agglutination assay (PA) and enzyme immunoassay (EIA). We compared the differences between the two methods and suggest a test method more suitable for clinical laboratories. METHODS: A total of 35 patients (18 adult and 17 pediatric) performed MP specific antibody test using PA (Serodia-Myco II, Fujirebio, Japan) and EIA (Ani Labsystems, Finland) methods. IgM and IgG antibodies were measured separately by EIA method. PA and both IgM and IgG EIA were tested in 26 patients and PA and IgG-EIA were tested in 9 patients. RESULTS: The concordance rates between PA and EIA were 57.7% for IgM and 65.7% for IgG antibodies. Positive PA results showed better agreement with IgG (77.8%) than IgM (38.9%), while negative PA results showed better agreement with IgM (100%) than IgG EIA results (25%). In adult patients, the correlation between PA titers and IgM (r=0.852, P <0.01) and IgG values (r=0.517, P <0.05) were statistically significant. In pediatric patients, the correlation between PA titers and IgG values (r=0.842, P <0.01) was statistically significant. CONCLUSIONS: In this study, we observed that PA and EIA may not be used alternatively. Therefore, we suggest that use of both PA and IgM-EIA will be the optimal choice for laboratories. However, when laboratories are required to select one from PA or EIA, PA may be more useful to diagnose MP infection.

A Case of Idiopathic Retroperitoneal Fibrosis Associated with Rapidly Progressive Glomerulonephritis with Anti-myeloperoxidase Antineutrophil Cytoplasmic Antibodies / 대한신장학회지

Idiopathic retroperitoneal fibrosis is a rare disease characterized by the presence of retroperitoneal fibroinflammatory tissue, which often surrounds the abdominal aorta, the ureters, or other abdominal organs. There have been sporadic reports of an association with autoimmune diseases, although the pathogenesis of idiopathic retroperitoneal fibrosis is unclear. However, there are very few reports ofidiopathic retroperitoneal fibrosis associated with rapidly progressive glomerulonephritis. We report a case with idiopathic retroperitoneal fibrosis associated with rapidly progressive glomerulonephritis, and anti-myeloperoxidase antineutrophil cytoplasmic antibodies (anti MPO-ANCA), that was improved by the combination therapy of steroids and cyclophosphamide.

The Clinical Outcome of Acute Renal Failure Requiring Hemodialysis after Coronary Intervention in Patients with Chronic Kidney Disease / 대한신장학회지

PURPOSE: Acute renal failure requiring dialysis (ARFD) after coronary intervention is a rare but serious complication, and it is associated with poor prognosis. This study investigated the clinical characteristics and risk factors of ARFD after coronary intervention in patients with chronic kidney disease (CKD). METHODS: This study included 178 adult patients with CKD (baseline serum creatinine of >1.3 mg/dL) undergoing coronary intervention at Samsung Medical Center from April 2003 through June 2007. We retrospectively evaluated the incidence, clinical outcome, and risk factor of ARFD via medical records. ARFD was defined as a decrease in renal function necessitating hemodialysis in the first 7 days after percutaneous coronary intervention. The estimated glomerular filtration rate (eGFR) was obtained using the Levey Modification of Diet in Renal Disease (MDRD) formula. RESULTS: Ten of the 178 patients (5.6%) developed ARFD with a subsequent in-hospital mortality rate of 10% (n=1). According to CKD stage, incidence of ARFD was 0/136 patients (baseline eGFR 30 to 90 ml/min/1.73m2 4/32 (15 to 29,) and 7/10 (<15) respectively (0% vs 9.4% vs 70%, p<0.0001). Multivariate analysis found eGFR (OR=0.5, 95% CI 0.3 to 0.9, p=0.02) to be only independent predictor of ARFD. Of those who developed ARFD (n=10), 2 patients stopped dialysis, 8 had required permanent dialysis. CONCLUSION: Incidence of ARFD increased according to the severity of renal dysfunction. The majority of ARFD patients had required permanent dialysis.

The Patency Rate of Hemodialysis Vascular Access and the Analysis of Patency-related Factors: Comparision of Native Arteriovenous Fistula with Arteriovenous Graft, Single Center Study / 대한신장학회지

PURPOSE: As the proportion of patients with diabetes and old age increases, the use of arteriovenous graft (AVG) is increasing. However, there are few data about the comparison of the patency rate of native arteriovenous fistula (AVF) to that of AVG in Korea. We compared the outcome of native AVF to that of AVG with analysis of the factors affecting the patency of the permanent vascular access in use. METHODS: A retrospective database of all vascular access related procedures performed from January 1, 2003 to December 31, 2003 was established. We evaluated the primary unassisted and cumulative access patency rate with analysis of patency-related factors. We also evaluated the primary failure rate of AVF and AVG. RESULTS: 196 new vascular access surgeries were performed during the period. 14 cases were excluded due to loss of follow-up. 142 native arteriovenous fistulas (78%) and 40 grafts (22%) were constructed. The primary failure rate was similar between AVF and AVG group. The primary unassisted (78%, 72%, 68% vs. 62%, 41%, 22% at 1, 2, 3 year; p<0.001) and cumulative patency rate (93%, 88%, 85% vs. 84%, 74%, 73% at 1, 2, 3 year; p=0.087) were better in AVF group than in AVG group. The factors affecting the patency of vascular access were the type of vascular access and planned construction of permanent vascular access. CONCLUSION: Patency of native AVF as the permanent vascular access was better than that of AVG. Planned construction of permanent vascular access improved the patency of the access.

Risk Factors of Ileus following Radical Cystectomy / 대한비뇨기과학회지

PURPOSE: Ileus is the most common complication following radical cystectomy. Ileus causes prolonged fasting, significant patient discomfort and prolongation of the hospital stay. We retrospectively examined the risk factors for ileus following radical cystectomy MATERIALS AND METHODS: Between January, 2000 and June, 2007, 59 patients underwent radical cystectomy with urinary diversion at our institution. 30 patients underwent creation of an ileal conduit and 29 patients underwent orthotopic bladder substitution with using ileum. Ileus was defined as the persistent absence of flatus on postoperative day 7 or the generalized findings of ileus on simple abdominal film. The potential risk factors were analyzed between the ileus and non ileus groups, including the patient's age and gender, the American Society of Anesthesiologists score, the preoperative serum albumin level, prior abdominal surgery, the operative time, combined operation, the estimated blood loss, the type of urinary diversion, use of N2O, use of intestinal stapler, complications, the ICU stay and the pathologic stage and grade. RESULTS: 24%(14/59) of patients had postoperative ileus. The difference of the ASA score between the two groups was statistically significant (p=0.03). Patients with ileus(67.5+/-7.2) were older than the non-ileus patients(63.0+/-9.4)(p=0.103). Ileus was more frequent in men(29.5%, 13/ 44) than in women(6.6%, 1/15)(p=0.09). CONCLUSIONS: Ileus following radical cystectomy was more frequent in patients with a high ASA score. Older aged men tend to have ileus after radical cystectomy.

Clear Cell Papillary Cystadenoma of the Epididymis Associated with Multiple Renal and Pancreatic Cysts / 대한남성과학회지

Papillary cystadenoma of the epididymis is a rare benign tumor that accounts for only 5% of all epididymal tumors. A case of epididymal cystadenoma was presented in a 25 year old man. The patient was hospitalized initially because of infertility and bilateral painless palpable scrotal masses. He had multiple renal cysts and pancreatic cysts. He had no renal, adrenal, cerebellar or retinal lesion as cardinal manifestation of the Von Hippel-Lindau Syndrome (VHL). The VHL gene mutation in this case was not identified. For evaluation and management, we planned left epididymovasostomy and left testicular biopsy. However, due to a cystic change on the left epididymis and left tunica albuginea, we instead performed a left partial epididymectomy for the cystic lesion, excision of the cystic mass from the left tunica albuginea, and diagnostic left testicular biopsy. The pathologic evaluation revealed a papillary cystadenoma of the epididymis and tunica albuginea, but with normal spermatogenesis.

Regional Citrate Anticoagulation in Continuous Venovenous Hemodiafiltration: Report of Two Cases / 대한신장학회잡지

Continuous renal replacement therapy (CRRT) has been used increasingly for the management of renal failure in hemodynamically unstable and critically ill patients. CRRT requires anticoagulation, usually with heparin, to prevent clotting in the extracorporeal circuit. Systemic heparinization is associated with a high rate of bleeding when used during CRRT in critically ill patients. We applied regional citrate anticoagulation for CRRT to two critically ill patients with high bleeding risk using calcium containing commercial solutions. We conclude that regional citrate anticoagulation with commercial calcium containing solution can be used alternative to heparin for CRRT in patients with high bleeding risk.

Cyclophosphamide treatment in a patient with thrombotic thrombocytopenic purpura and lupus nephritis: report of one case / 대한내과학회지

Thrombotic thrombocytopenic purpura is a rare but fatal complication of systemic lupus erythematosus. The diagnosis of thrombotic thrombocytopenic purpura as a syndrome distinct from systemic lupus erythematosus may be challenging particularly when thrombotic thrombocytopenic purpura is presented concomitantly with systemic lupus erythematosus. Early diagnosis and aggressive treatment including plasmapheresis would be required. However, recent reports have suggested that the use of cyclophosphamide may have a role. We describe a patient with systemic lupus erythematosus who was first presented with severe thrombotic thrombocytopenic purpura. Diagnosis was based on typical clinical features of thrombotic thrombocytopenic purpura and laboratory findings of active lupus nephritis. Renal biopsy also confirmed the coexistence of thrombotic thrombocytopenic purpura and diffuse proliferative lupus nephritis. Although prompt extensive plasmapheresis and high dose steroid therapy were performed, oliguric renal failure and thrombocytopenia persisted. After addition of cyclophosphamide to the treatment with plasmapheresis and steroid, clinical manifestations of thrombotic thrombocytopenic purpura and lupus nephritis were markedly improved.