Comparative effectiveness of JAK inhibitors and biologic disease-modifying antirheumatic drugs in patients with rheumatoid arthritis

Background/Aims@#We aimed to compare the effectiveness and safety of Janus kinase inhibitors (JAKi) vs. biologic disease- modifying antirheumatic drugs (bDMARD) in Korean patients with rheumatoid arthritis (RA) who had an inadequate response to conventional synthetic DMARDs. @*Methods@#A quasi-experimental, multi-center, prospective, non-randomized study was conducted to compare response rates between JAKi and bDMARDs in patients with RA naïve to targeted therapy. An interim analysis was performed to estimate the proportion of patients achieving low disease activity (LDA) based on disease activity score (DAS)–28– erythroid sedimentation rate (ESR) (DAS28-ESR) at 24 weeks after treatment initiation and to evaluate the development of adverse events (AEs). @*Results@#Among 506 patients enrolled from 17 institutions between April 2020 and August 2022, 346 (196 JAKi group and 150 bDMARD group) were included in the analysis. After 24 weeks of treatment, 49.0% of JAKi users and 48.7% of bDMARD users achieved LDA (p = 0.954). DAS28-ESR remission rates were also comparable between JAKi and bDMARD users (30.1% and 31.3%, respectively; p = 0.806). The frequency of AEs reported in the JAKi group was numerically higher than that in the bDMARDs group, but the frequencies of serious and severe AEs were comparable between the groups. @*Conclusions@#Our interim findings reveal JAKi have comparable effectiveness and safety to bDMARDs at 24 weeks after treatment initiation.

Quality Indicators for Evaluating the Health Care of Patients with Rheumatoid Arthritis: a Korean Expert Consensus

Background@#There is increasing interest in the quality of health care and considerable efforts are being made to improve it. Rheumatoid arthritis (RA) is a disease that can result in favorable outcomes when appropriate diagnosis and treatment are provided. However, several studies have shown that RA is often managed inappropriately. Therefore, the Korean College of Rheumatology aimed to develop quality indicators (QIs) to evaluate and improve the health care of patients with RA. @*Methods@#Preliminary QIs were derived based on the existing guidelines and QIs for RA. The final QIs were determined through two separate consensus meetings of experts. The consensus was achieved through a panel of experts who voted using the modified Delphi method. @*Results@#Fourteen final QIs were selected among 70 preliminary QIs. These included early referral to and regular follow-up with a rheumatologist, radiographs of the hands and feet, early initiation and maintenance of disease-modifying anti-rheumatic drug (DMARD) therapy, periodic assessment of disease activity, screening for drug safety and comorbidities,including viral hepatitis and tuberculosis before biologic DMARD therapy, periodic laboratory testing, supplementation with folic acid, assessment of the risk for cervical spine instability before general anesthesia, patient education, and specialized nurse. @*Conclusion@#These QIs can be used to assess and improve the quality of health care for patients with RA.

Quality Indicators for Evaluating the Health Care of Patients with Rheumatoid Arthritis: a Korean Expert Consensus

Background@#There is increasing interest in the quality of health care and considerable efforts are being made to improve it. Rheumatoid arthritis (RA) is a disease that can result in favorable outcomes when appropriate diagnosis and treatment are provided. However, several studies have shown that RA is often managed inappropriately. Therefore, the Korean College of Rheumatology aimed to develop quality indicators (QIs) to evaluate and improve the health care of patients with RA. @*Methods@#Preliminary QIs were derived based on the existing guidelines and QIs for RA. The final QIs were determined through two separate consensus meetings of experts. The consensus was achieved through a panel of experts who voted using the modified Delphi method. @*Results@#Fourteen final QIs were selected among 70 preliminary QIs. These included early referral to and regular follow-up with a rheumatologist, radiographs of the hands and feet, early initiation and maintenance of disease-modifying anti-rheumatic drug (DMARD) therapy, periodic assessment of disease activity, screening for drug safety and comorbidities,including viral hepatitis and tuberculosis before biologic DMARD therapy, periodic laboratory testing, supplementation with folic acid, assessment of the risk for cervical spine instability before general anesthesia, patient education, and specialized nurse. @*Conclusion@#These QIs can be used to assess and improve the quality of health care for patients with RA.

A Case of Intestinal Behcet's Disease Complicated Enterocutanous Fistula with a Good Response to Adalimumab

Behcet's disease (BD) is a multisystemic disorder, which is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesion. Although intestinal involvement is an uncommon manifestation of the BD, it leads to a poor prognosis, as a result of a high complication, such as intestinal perforation, fistula formation, and massive hemorrhage. Intestinal BD has the tendency for the resistance to conventional medical treatment, and it often requires a surgical intervention because of severe complication. Although the causes of intestinal BD are unknown, some studies show that tumor necrosis factor Alpha (TNF-alpha) plays a key role in the pathogenesis of BD. Therefore, anti-TNF-alpha monoclonal antibody, such as adalimumab, is one of the useful treatment for refractory and relapsed intestinal BD. We describe a patient who had intestinal BD complicated enterocutanous fistula with a good response to adalimumab.

A Case of Atrial Fibrillation Induced by Infliximab in a Patient with Rheumatoid Arthritis

Infliximab, a chimeric monoclonal immunoglobulin antibody to tumor necrosis factor (TNF)-alpha is widely used in the treatment of rheumatoid arthritis (RA). The commonly reported cardiac side effects of infliximab include exacerbation of congestive heart failure, hypotension, and syncope. Cardiac arrhythmia and conduction disturbances have been reported only rarely in a few case reports and to the best of our knowledge, there are no previous reports on the occurrence of atrial fibrillation secondary to infliximab use in RA patients. Here, we report a case of acute atrial fibrillation with rapid ventricular response that occurred 24 hr after first dose of infliximab in a 55-year-old female patient with RA, who recovered to sinus rhythm after pharmacologic management.

A Case of Primary Influenza B Pneumonia in Lupus Nephritis Patient on Immunosuppressive Treatment

Infectious diseases, particularly pneumonia, remain a major cause of morbidity and mortality in patients with systemic lupus erythematous (SLE), accounting for 30~50% of all death. Because infection appears in various forms in lupus patients, early detection and appropriate treatment is very important. In the case of diffuse infiltration in the whole lung field, we should make a differential diagnosis with drug-induced pneumonitis, interstitial lung disease and pneumonia caused by atypical microorganism infection. In particular, in case of atypical microorganism pneumonia, we usually suspect cytomegalovirus, pneumocystis jiroveci and ebstein-barr virus, but influenza pneumonia is relatively neglected. Here, we report on the case of a 26-year-old female patient with lupus nephritis who presented with dyspnea and diffuse pulmonary infiltration immediately after intravenous cyclophosphamide. She was diagnosed with primary influenza B pneumonia and successfully treated with oseltamivir.

Treatment Persistence with TNF Blocker in Korean Rheumatoid Arthritis Patients

OBJECTIVE: To estimate drug persistency and the safety of TNF blocker in Korean patients with rheumatoid arthritis. METHODS: Data were extracted from medical records of rheumatoid arthritis patients who had treated with TNF blocker or are currently using TNF blocker at Hanyang University Hospital for Rheumatic Diseases from December 2000 to November 2009 (REtrospective study for Safety and Efficacy of Anti-RA treatment with biologiCs, RESEARCh). Comprehensive chart reviews were undertaken on all patients and data on drug usages and response of TNF blocker was collected at initiation, 3 months and the time of data collection. Persistency with treatment was examined using life-table analysis and multivariate Cox proportional hazard models were developed to examine potential predictors of discontinuation of TNF blocker. RESULTS: A total of 268 patients were enrolled in this retrospective study. Among them 180 patients were included in the analysis of drug persistency. The 1-year and 5-year drug persistency of TNF blocker was 74% and 46%, respectively. Concomitant use of methotrexate (hazard ratio 0.46, 95% CI 0.27-0.80) was associated with higher persistence. Comparing to etanercept, adalimumab is an independent risk factor for discontinuation (hazard ratio 2.63, 95% CI 1.43-4.84). CONCLUSION: Five-year drug persistency of TNF blocker was 46% and concomitant use of methotrexate is associated with higher persistence.

Acute Retropharyngeal Calcific Tendinitis in an Unusual Location: a Case Report in a Patient with Rheumatoid Arthritis and Atlantoaxial Subluxation

Retropharyngeal calcific tendinitis is defined as inflammation of the longus colli muscle and is caused by the deposition of calcium hydroxyapatite crystals, which usually involves the superior oblique fibers of the longus colli muscle from C1-3. Diagnosis is usually made by detecting amorphous calcification and prevertebral soft tissue swelling on radiograph, CT or MRI. In this report, we introduce a case of this disease which was misdiagnosed as a retropharyngeal tuberculous abscess, or a muscle strain of the ongus colli muscle. No calcifications were visible along the vertical fibers of the longus colli muscle. The lesion was located anterior to the C4-5 disc, in a rheumatoid arthritis patient with atlantoaxial subluxation. Calcific tendinitis of the longus colli muscle at this location in a rheumatoid arthritis patient has not been reported in the English literature.

Clinicial Significance of Spontaneous Pneumomediastinum in Dermatomyositis/Polymyositis / 대한류마티스학회지

OBJECTIVE: Pneumomediastinum (PnM), a rare complication of dermatomyositis and polymyositis (DM/PM), is sporadic and has an unclear pathogenesis. PnM is almost always associated with interstitial lung disease (ILD), and is a poor prognostic factor in inflammatory myositis patients. We studied the prevalence of PnM in Korean DM/PM and its clinical significance. METHODS: We retrospectively studied the medical records of 161 patients diagnosed with DM/PM meeting Bohan-Peter's criteria at Hanyang University Hospital for Rheumatic Diseases from 1995 to 2010. We collected following findings; demographic data, diagnosis, lung involvement, cause of death, and duration from diagnosis to death. RESULTS: One hundred nineteen patients (73.9%) were DM and 42 patients (26.1%) were PM. Eighty three patients (51.6%) developed ILD at diagnosis or during follow up. Eighteen patients (11.2%) died because of ILD aggravation, infection, or malignancy. The mean duration from diagnosis to death was 11.5 months, with 10 patients (6.2%) dying from from ILD aggravation but none with spontaneous PnM. 6 patients (3.7%) presented with PnM, and it was associated with ILD worsening in all cases. PnM resolved with O2 inhalation, corticosteroids, and/or immunosuppressive agents after 11 weeks (mean) of therapy. CONCLUSION: PnM is rare but associates with DM and aggravation of ILD. PnM does not usually cause fatalities and can be cured by appropriate therapy.

ANCA Associated Vasculitis / 대한류마티스학회지

Primary antineutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV) constitute a group of small vessel vasculitides that includes Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. Recently, many in vitro and in vivo studies have highlighted the role of ANCA as the main pathophysiological factor in the development of AAV. Two remarkable studies on ANCA pathogenesis were recently reported. One study examined anti-lysosomal membrane protein-2, which supports the 'shared epitope' theory. The other examined the neutrophil extracellular trap that is released by neutrophils primed by ANCA. Each disease of AAV shows a broad spectrum of the clinical features and severities, which makes it difficult to diagnose and treat them. Considerable effort has been made in the past decades to improve the treatment outcomes, reduce the incidence of relapse and avoid drug toxicity. This review describes the current understanding of AAV along with a few Korean reports.

Safety and Clinical Responses in Ankylosing Spondylitis after Three Months of Etanercept Therapy

We aimed to evaluate the safety and clinical responses in Korean ankylosing spondylitis (AS) patients after three months of etanercept therapy. AS patients satisfying the Modified New York Criteria were enrolled. They were assessed for safety and clinical responses at enrollment and after three months of etanercept therapy. A total of 124 patients completed the study. After three months, the rate of ASsessment in AS International Working Group 20% improvement (ASAS 20) response was 79.8%. The rates of ASAS 40 and ASAS 5/6 responses were 58.5 and 62.8%, respectively. Significant improvement of Korean version of Bath AS Disease Activity Index (KBASDAI) (p<0.0001), Bath AS Functional Activity Index (BASFI) (p<0.0001), and Bath AS Metrology Index (BASMI) (p=0.0009) were achieved after three months. Quality of life was also significantly improved after three months, as demonstrated by scores for SF-36 (p<0.0001) and EQ-5D (p<0.0001). Erythrocyte sedimentation rate and C-reactive protein were significantly decreased (p<0.0001, p<0.0001, respectively). None of the patients developed tuberculosis and there were no serious adverse event. AS patients with inadequate response to conventional therapy showed significant clinical improvement without serious adverse events after three months of etanercept therapy.

Lobular Panniculitis in a Patient with Dermatomyositis / 대한류마티스학회지

Panniculitis is an inflammation within adipose tissue and most commonly affects the subcutaneous fat. Frequently, the panniculitis is associated with certain drugs and systemic diseases, such as various rheumatologic diseases, idiopathic Weber-Christian disease, infection and malignancy. Panniculitis in dermatomyositis (DM) is most commonly an incidental histopathologic finding that less commonly manifests a clinical component. We report the case of a woman with DM who presented with panniculitis as a clinical finding. A 51-year-old woman was admitted to our hospital with diffuse nodular, indurated, painful erythematous plaques on buttocks, back and chest. 18-months ago, she had been diagnosed DM at our hospital. Her medication on admission was azathioprine. High-dose glucocorticoid was prescribed after the diagnosis of lobular panniculitis confirmed by skin biopsy. After then, the patient was getting better and discharged with the medication tapered.

The Prevalence and Trend of Arthritis in Korea: Results from Korea National Health and Nutrition Examination Surveys / 대한류마티스학회지

OBJECTIVE: To estimate the prevalence of musculoskeletal diseases, especially osteoarthritis, rheumatoid arthritis, osteoporosis and lumbar disc herniation, in Korean adults. For arthritis and total musculoskeletal diseases, trend of the age-standardized prevalence rates were evaluated. METHODS: This study was based on the data obtained from the Korea National Health and Nutrition Examination Surveys (KNHANES) I, II and III, conducted in 1998, 2001 and 2005, respectively. KNHANES is a nationwide cross-sectional study using a stratified, multistage probability sampling design for the selection of household units. Annual self-reported prevalence and its confidence interval were estimated in adults aged over 19, using Health Interview Survey in KNHANES. All analyses were done using SAS 9.1 with "survey procedure" except for age-standardized prevalence rates for comparison prevalence rates of each survey. Age-standardized prevalence rates were calculated using a direct-method. RESULTS: The prevalence of musculoskeletal diseases was 144.6, 140.0 and 197.2 and the annual self-reported prevalence of arthritis was 117.9, 109.2 and 146.4 per 1,000 population in 1998, 2001 and 2005, respectively. In KNHANES III, osteoarthritis was the most prevalent disease of the musculoskeletal diseases for both sex. Fifty-eight percent of the over 65 year-old population had at least one musculoskeletal disease and it was higher in women with 73%. CONCLUSION: The prevalence of musculoskeletal diseases was high in Koreans with arthritis being the most prevalent. The prevalence of musculoskeletal disease correlated with low sociodemographic status.

Clinical Features and Course of Systemic Onset Juvenile Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVE: Juvenile rheumatoid arthritis (JRA) is classified as polyarticular, oligoarticular, and systemic onset type by clinical symptoms presented during first six months. This study was performed to investigate the clinical features and course of systemic onset JRA. METHODS: We performed a retrospective study for patients who were diagnosed as JRA between March 2000 and March 2006 according to the JRA criteria of the International League of Association for Rheumatology (ILAR). RESULTS: Of the 216 JRA patients, 33 patients (11 male/ 22 female) were systemic onset type. Because of insufficient data, 6 patients were excluded. Chief complaints at the time of diagnosis were fever (81.5%) and arthralgia (77.7%). During the disease course, all patients manifested fever and arthritis, rash (59.2%) and splenomegaly (22.2%) also occurred. Most patients had symmetric (81.5%) arthritis, and involved more than five joints (59.3%) including knee and wrist. Anemia, leukocytosis, and thrombocytosis were common laboratory abnormalities. Almost all patients had elevated level of C-reactive protein and erythrocyte sediment rate. Some patients had positive results about immunologic marker such as rheumatoid factor (3.8%), antinuclear antibody (57.7%), and antiperinuclear factor (9.5%). Therapeutic regimens included glucocorticoids (88.9%), nonsteroidal anti-inflammatory drugs (81.5%), methotrexate (81.5%), and hydroxychloroquine (55.6%). Biologic agents were applied in 5 patients, and 3 showed improvement of disease activity. Combination therapy was introduced in 18.5% of patients, and 63% of patients still required medications. CONCLUSION: In Korea, systemic onset JRA patients had variable clinical manifestations and chronic course of disease, which often extended into adulthood.

Recurrent Acute Myocardial Infarctions and Budd-Chiari Syndrome in Young Woman with Behcet's Disease / 대한류마티스학회지

We report here a case of vascular Behcet's disease. 32-year-old young woman who suffered from chest pain was diagnosed with acute myocardial infarction four years before admission. At that time she manifested symtoms of Behcet's disease, such as oral ulcers, genital ulcers, multiple arthralgia, and a peculiar hyperirritability reaction to needle puncture. At this time she was admitted due to acute chest pain and intermittent abdominal pain. The diagnosis of an acute anteroseptal myocardial infarction was made on the basis of the electrocardiography findings. Cardiac catheterization revealed segmental stenosis up to 95% at the mid-left anterior descending artery without aneurysm formation at other coronary trees and there were no atherosclerotic changes. Coronary stent was inserted in the lesion. Abdominal CT showed inferior vena cava and right hepatic vein obstruction, which supports the diagnosis of Budd-Chiari syndrome. Our case demonstrated the followings which are not common to vasculo-Behcet disease; 1) initial clinicopathologic manifestations at the coronary artery, 2) abrupt total occlusion with clear cut lesion at left anterior descending artery, 3) recurrent myocardial infarctions, 4) Budd-Chiari syndrome, 5) combined superior mesenteric artery, inferior mesenteric artery and celiac trunk obstruction. Among the systemic manifestation of Behect's disease, cardiac involvement is very rare but should be considered as one of the most important features that influences the prognosis.

Angiotensin-Converting Enzyme Gene Insertion/Deletion Polymorphism in Korean Patients with Systemic Sclerosis

To determine whether angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism is associated with the development and clinical features of systemic sclerosis (SSc) in Korean, we studied seventy two Korean patients with SSc fulfilling the ACR preliminary classification criteria. The controls were 114 healthy, disease free Koreans. ACE I/D genotypes were determined by PCR method using oligonucleotides. Sixty eight patients (94.4%) were women and age at diagnosis was 43.5+/-12.6 yr old (mean+/-SD). Thirty nine patients (54.2%) had a diffuse type of SSc. There were no statistical differences in the frequencies of all ACE I/D genotypes and D allele between patients and controls, and neither between diffuse and limited types of SSc. ACE I/D gene polymorphism was not associated with the development of SSc in Korea. The investigation for the pathogenesis of SSc requires more studies about the role of other candidate genes such as endothelin, TGF-beta, nitric oxide, or angiotensin II receptor in addition to the ACE genes.

Evaluation of Consistency, Generalizability, Applicability of Recommendations about Pharmacological Treatment of Osterarthritis using RAND Method

BACKGROUND: When research based evidence is not sufficient, clinical practice guidelines can be based on opinions. In such situations, formal consensus development methods, often based on the modified nominal group techniques are widely used. It can be used to evaluate consistency, generalizability, applicability of recommendation when evidence comes from other countries. METHODS: To develop evidence based guidelines for osterarthritis pharmacotherapy, a consensus expert panel consisting of internists, family physicians, methodologists, and orthopedic surgeons were convened. After an extensive structured literature searching and evaluation, evidence statements for key question were developed. Rating methods for consistency, generalizability, applicability of statement were adopted from those jointly developed by Rand and the University of California, Los Angeles. RESULTS: We developed 27 evidence statements in 17 question domains. Among 72 rating items, 62 items reached agreement. Among 15 recommendations, 10 recommendation grading were A, 2 were B, and 3 were C. CONCLUSION: When research based evidence is not sufficient, clinical practice guidelines can be based on formal consensus of experts, especially modified nominal group techniques. It can be used to evaluate consistency, generalizability, applicability of recommendation when evidence comes from other countries.

Multicenter Study in Efficacy and Safety of Celecoxib in Patients with Rheumatoid Arthritis and Osteoarthritis / 대한류마티스학회지

OBJECTIVE: The aim of this study was to define the efficacy and safety of celecoxib in Korean patients with rheumatoid arthritis and osteoarthritis. In addition, the impact of gastrointestinal symptom severity on health related quality of life (HRQoL) was measured before and after the treatment with celecoxib. METHODS: Three hundred seventy nine patients with rheumatoid arthritis (n=175) and osteoarthritis (n=204) were enrolled from 25 centers from May 2004 to December 2004. After treatment of celecoxib for 4 weeks, efficacy was determined by physician's global assessment and EQ-5D. Severity of gastrointestinal (GI) symptom was assessed by visual analogue scale. RESULTS: Treatment made improvement in 263 patients (69.4%), but 108 patients (28.5%) didn't show change in their symptom and 8 patients (2.1%) were aggravated after treatment. Mean change was 0.129+/-0.3 in EQ-5D utility score (p<0.05), 11.8+/-17.8 in VAS in EQ-5D (p<0.05) and -7.9+/-19.6 in GI symptom severity (p<0.05). Blood pressure was not elevated after treatment and there was no cardiovascular adverse event. Gastrointestinal symptom improvement correlates with improvement in VAS (r=0.2, p<0.01). CONCLUSION: Celecoxib is not only effective in patients with rheumatoid arthritis and osteoarthritis but also helpful in reducing GI symptom.

Association of Interleukin-18 Polymorphisms with Adult Onset Still's Disease in Korea / 대한류마티스학회지

OBJECTIVE: It has been suggested that overproduction of interleukin -18 (IL-18) may contribute to the pathogenesis of adult onset Still's disease (AOSD). Recently, positive association between a polymorphism in the IL-18 gene and different diseases like diabetes, sarcoidosis and asthma has been reported. The aim of the present study was to investigate the potential association of two single-nucleotide polymorphisms (SNPs) at position -137 (G/C) and -607 (C/A) in the promoter region of the IL-18 gene with susceptibility and clinical feature of AOSD in the Korean population. METHODS: We examined two SNPs of IL-18 in 70 patients with AOSD and 204 healthy control individuals. The genotyping were performed using sequence specific PCR. Haplotypes were analyzed by the estimated haplotype program. The patients with AOSD were subdivided into groups according to disease course: monocyclic systemic, polycyclic systemic, and chronic articular type. RESULTS: As for the -607 genotypes, 13 of the 69 patients had CC genotype (18.8%), 36 the CA type (52.2%) and 20 the AA type (29.0%). AOSD patients had higher frequency of A allele at -607 when compared to controls (OR 1.48, 95% CI 1.00~2.18, p=0.048). AOSD patients had significantly higher frequency of AA genotypes at -607 when compared to controls (AA vs CA& CC, OR 1.90, 95% CI 1.01~3.58, p=0.044). As for the -137 genotypes, of the 68 patients, 57 had GG genotype (83.8%), 9 the GC type (13.2%) and 2 (2.9%) had the CC type. No differences were found in allele and genotype frequencies between two groups. The haplotype frequencies of the IL-18 polymorphism were not significantly different between patients with AOSD and controls. The frequency of -137 GG genotype was significantly increased in chronic articular type compared to healthy control and systemic type of AOSD. CONCLUSION: In IL-18 gene polymorphisms, the A allele and AA genotye at position -607 might be genetic risk factors for the development of AOSD in Korean population. Further investigation in larger groups is required to provide more conclusive evidence regarding the role of the IL-18 gene polymorphism in AOSD.

Rituximab Treatment for the Patient with Refractory Lupus Nephritis / 대한류마티스학회지

OBJECTIVE: To determine preliminary evidence for the safety and efficacy of B lymphocyte depletion therapy in refractory systemic lupus erythematosus (SLE). METHODS: Four female lupus nephritis patients who had been refractory to steroid and one or more immunosuppressive therapy were treated on an open-label basis. During a 4-week period, each patient received two 500-mg infusions of rituximab and two 750-mg infusions of cyclophosphamide. RESULTS: Patient 1, 2, and 3 were responded with rituximab treatment with improvements in SLEDAI and laboratory parameters such as C3/C4 and 24 hour urine protein. However, patient 4 had not improved with rituximab. The variation in the level of anti-double-stranded DNA antibody was different in individual patients. No significant adverse events were observed during follow-up. CONCLUSION: This study provides an evidence for the safety and possible efficacy of B lymphocyte depletion therapy in refractory lupus nephritis. However a further randomized trial is needed to confirm the efficacy and durability of remission.