OBJECTIVE: To investigate the clinical and immunological characteristics of systemic lupus erythematosus (SLE) with retinopathy. METHODS: Fifty SLE patients with retinopathy without hypertension and diabetes (retinopathy group) who were hospitalized in the Peking University People's Hospital from January 2009 to July 2022 were screened. Fifty SLE patients without blurred vision during the course of the SLE and without retinopathy in the fundus examinations (non-retinopathy group) matched for sex and age were selected. Their clinical manifestations, laboratory tests and lymphocyte subsets were statistically analyzed. RESULTS: The most common fundus ocular change of the SLE patients with retinopathy was cotton-wool spots (33/50, 66.0%), followed by intraretinal hemorrhage (31/50, 62.0%). Retinopathy could occur at any stage of SLE duration, with a median of 1 year (20 days to 30 years). The proportion of lupus nephritis (72.0% vs. 46.0%, P=0.008) and serositis (58.0% vs. 28.0%, P=0.002) in the retinopathy group were significantly higher than those in the non-retinopathy group. The proportion of neuropsychiatric systemic lupus erythematosus (NPSLE) in the retinopathy group was higher, but there was no significant difference between the two groups. Compared with the non-retinopathy group, the proportion of positive anti-cardiolipin antibody (30.0% vs. 12.0%, P=0.027), the SLEDAI score (median 22.0 vs. 10.5, P < 0.001), erythrocyte sedimentation rate (P < 0.001), C-reactive protein (P=0.019) and twenty-four hours urine total protein level (P=0.026) in the retinopathy group were significantly higher, and the hemoglobin level was significantly lower [(91.64±25.18) g/L vs. (113.96±18.57) g/L, P < 0.001]. The proportion of CD19⁺ B cells in peripheral blood of the patients with SLE retinopathy was significantly increased (P=0.010), the proportion of CD4⁺ T cells was significantly decreased (P=0.025) and the proportion of natural killer (NK) cells was lower (P=0.051) when compared with the non-retinopathy group. CONCLUSION Retinopathy in SLE suggests a higher activity of SLE disease with more frequent hematologic and retinal involvement. It is recommended to perform fundus examination as soon as a patient is diagnosed with SLE. SLE patients with retinopathy may have stronger abnormal proliferation of B cells, and aggressive treatment should be applied to prevent other important organs involvement.
Humans ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System ; Lupus Nephritis ; Antibodies, Anticardiolipin ; Serositis

OBJECTIVE: To investigate the prevalence of avascular necrosis(AVN) of bone in SLE patients, and to examine the contributing factors of AVN. METHODS: 230 patients of 322 SLE patients fulfilling the 1982 ARA criteria of SLE were analysed for the steroid treatment and clinical manifestations. Their mean age was 33 years old. AVN was diagnosed by evidence of AVN in plain X-ray, bone scan and/or MRI. Retrospective analysis for medication history and clinical manifestations were performed. RESULTS: The rpevalence of AVN was 9.3%(30 patients of 322). All of the AVN patients were female and their mean age was 28 years old. Most common site of AVN was fernoral head(54.3%), other sites were distal femur(22%o) and proximal tibia(15%), proximal humerus(7%) and tarsal bone(2%), in order. 25 patients had multiple bony involvements. AVN was significantly associated with cutaneous vasculitis, CNS involvement and lupus nephritis. Recent(6 months before arthralgia) daily steroid dose of AVN(+) patients was higher than AVN(-) patients(15.2 mg vs 7.6mg p(0.05). Total mean daily steriod dose was not significantly different (20.3mg/d vs 12.3 mg/d) between AVN(+) and AVN(-). CONCLUSION: AVN is an important cause of musculoskeletal damage and disability in SLE. SLE patients with higher steroid treatment and major organ involvement need to be carefully evaluated for avascular necrosisl
Adult ; Female ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Magnetic Resonance Imaging ; Osteonecrosis* ; Prevalence ; Retrospective Studies ; Vasculitis, Central Nervous System

No abstract available.
Arteritis* ; Lupus Erythematosus, Systemic* ; Nephritis*

Introduction: Systemic lupus erythematosus (SLE) predominantly affects individuals at peak age of productivity, and medical costs negatively impact on personal, family and community resources. This study aimed to identify annual medical costs and cost predictors among Filipino SLE patients. Methods: Direct annual healthcare costs were determined by survey questionnaires conducted among patients aged > 18 years with minimum one-year illness duration, consecutively seen at the lupus clinics of University of Santo Tomas (UST) Hospital from February to July 2016. Excluded were costs related to biologics. Predictors of cost were estimated using multiple regression analysis. Results: Respondents included 300 SLE patients, 270 females, (94%) age range 11-62 years, mean age 32.84±9.89 at SLE diagnosis, mean disease duration 5.87±5.58 (1-36) years. Median and mean annual direct medical cost was Php 90,950 and Php 133,040 respectively (range Php 17,440859,050). Annual cost was higher in those requiring dialysis (n=16) compared to those not requiring dialysis (n=150) vs those without nephritis (n=134), (median Php 595,400 vs 144,700 vs 55,020 respectively), p<0.001. End stage renal disease (ESRD) (p<0.001), mycophenolate use (p<0.001), high frequency of clinic visits (p=0.016) and lupus-related hospitalizations (p=0.018) were independent variables which significantly contributed to annual costs. Conclusion Nephritis especially if requiring dialysis was the most important cost predictor in this cohort, increasing annual costs to seven-fold. Mycophenolate use, lupusrelated hospitalizations and clinic visits increased annual costs by 147.2%, 173.8% and 2.6% respectively. This study reinforces the need for early recognition and aggressive disease control to prevent complications especially in those with renal involvement.
Hospitalization ; Nephritis ; Lupus Erythematosus, Systemic

Lupus nephritis is one of major complications of systemic lupus erythematosus, which determine the outcome of the disease. A case of lupus nephritis was admitted to our pediatric department and renal biopsy was done, which reveraled membranophroliferative type in electron-microscopy. He was treated with prednisone only and responded well in urinary findings. Now, he is maintained with low dose prednisone.
Biopsy ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Prednisone

Introduction: Systemic lupus erythematosus (SLE) is increasingly being diagnosed in our country. This study aims to describe the clinical features, management strategies and outcome of patients with SLE during a ten-year period. Methods: This is a retrospective cohort study of patients first diagnosed with SLE at the National Kidney and Transplant Institute in 2004 who were then followed up in the next ten years. Results: Eighty-five patients were first diagnosed with SLE in 2004. The mean age was 28.1±12.03 years old. Hypertension (34.12%) was the most common co-morbid illness. Renal involvement (74.12%) was seen in a majority but only those with cardiopulmonary manifestations (mean=0.71 years, p=0.030) significantly affected survival. Eleven patients (12.94%) expired during the study period. Active disease and infection were the most common causes of death. Biopsyproven lupus nephritis had a significantly higher survival rate (mean=10.57 years, p=0.006). Those on hemodialysis had a significantly lower survival time (mean=8.82 years, p=0.040). Discussion: The estimated 10-year cumulative survival rate of patients with SLE in our cohort was 75%. This is comparable to the rates reported in some countries. Regular follow-up at six to eight weeks intervals with more frequent follow-up for patients with an SLE flare and/or on intensive immunosuppression was the most likely reason for studies reporting higher survival rates. The disparity in the survival rates may also be attributed to the frequency of exacerbations with better survival among those who never had exacerbations. The most common cause of death was due to septic shock secondary to pneumonia. The authors believe that one factor that was contributory to death was the degree of immunosuppression as observed in studies describing high doses of corticosteroids on those who have died. Conclusion The cumulative survival rate decreased from 90% at the time of diagnosis to 75% on the tenth year which was comparable to several countries. Patients with cardiopulmonary manifestations were found to significantly affect survival in this study. Although renal involvement was the most common initial manifestation, it did not significantly affect survival similar to other studies. However, biopsyproven lupus nephritis cases had better survival since this allowed treatment to be streamlined based on the class of lupus nephritis. Active disease and infection were the most common causes of death.
Lupus Erythematosus, Systemic ; Lupus Nephritis ; Survival

Introduction: Systemic lupus erythematosus (SLE) is a chronic inflammatory systemic disease that can affect any organ including the central nervous system (CNS). Subarachnoid hemorrhage (SAH) is one of its rare CNS manifestations. Posterior reversible encephalopathy syndrome (PRES), with features of headache, seizures, altered mental status, visual loss, and typical imaging findings, has recently been associated with SLE and immunosuppression, including use of high dose steroids. The patient was seen in University of the Philippines-Philippine General Hospital (UP-PGH), a tertiary training hospital in Manila, Philippines Case: A 33-year-old female with lupus had PRES and SAH after methylprednisolone pulse therapy (MPPT) for nephritis. She presented with headache, hypertension and seizure. Initial cranial imaging showed hypodense areas in both parietotemprooccipital regions and small acute infarcts. She was intubated and treated with anti-convulsants for seizure; hydrocortisone and mycophenolate mofetil for SLE. She regained awareness on the seventh hospital day and was extubated. On the eleventh hospital day, she had fever, cough and was noted to be drowsy. She had increasing serum creatinine and decrease in urine output. Repeat cranial CT scan showed subarachnoid hemorrhage at the right sylvian fissure and better delineation of the previously described hypodensities (consistent with PRES). She was treated for hospital acquired pneumonia and underwent hemodialysis. Pneumonia was resolved and patient became conscious with no recurrence of neurologic symptoms. Consecutive outpatient visits showed a conscious and less edematous patient. Hemodialysis was eventually discontinued until she had normal creatinine with adequate urine output. Anti-seizure medications were also discontinued. Cranial CT scan a year later revealed normal brain parenchyma indicating resolution of previously noted hypodensities and subarachnoid hemorrhage. Conclusion There is a need to recognize PRES and differentiate it from irreversible neurologic conditions. With early identification and prompt intervention, permanent neurologic deficits may be prevented.
Lupus Erythematosus, Systemic ; Lupus Nephritis ; Subarachnoid Hemorrhage

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease that is a significant source of morbidity and mortality when it manifests in the central nervous system. The early detection and treatment of neuropsychiatric SLE (NPSLE) is very important, but a confirmative diagnostic tool has yet to be developed. CASE REPORT: We report here a case of neuropsychiatric manifestations in a patient that were associated with SLE, and evidence of reversal of bilateral amygdala and parahippocampal lesions in the brain revealed by 18fluorodeoxy glucose-positron emission tomography. CONCLUSIONS: We are suggestive of 18fluorodeoxy glucose-positron emission tomography appear to be more sensitive in detecting subtle brain changes in NPSLE.
Amygdala* ; Autoimmune Diseases ; Brain* ; Central Nervous System ; Humans ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System* ; Mortality

We report two Filipino women with systemic lupus erythematosus (SLE) who developed diffuse alveolar hemorrhage (DAH), a rare, life-threatening complication associated with a high mortality rate. DAH should be suspected in patients with SLE presenting with new pulmonary infiltrates, a decline in hemoglobin, hemoptysis, dyspnea, and persistent desaturation. The first patient is 23 years old and was diagnosed with SLE 8 years ago; initially presenting with malar rash, oral ulcers, nephritis, and positive antinuclear antibodies (ANA). She had a poorly controlled disease and was admitted for facial and bipedal edema due to lupus nephritis. She was given 1 gram of methylprednisolone intravenously (IV) for three consecutive days. She then became tachypneic producing bloody secretions, with desaturation and sudden decline in hemoglobin. She was given cyclophosphamide 1 gram IV and referred for plasmapheresis but eventually succumbed. The second patient is 56 years old with generalized body weakness. Laboratory workup showed nephritis, anemia, ANA, low C3, and high anti-dsDNA titers. Pulse methylprednisolone 1000 mg was initiated. However, there was new-onset hemoptysis and desaturation and the patient was intubated. Bronchoscopy revealed diffuse bleeding on the right middle lobe and she eventually expired. Both patients with active SLE nephritis presented in this study died within days of DAH diagnosis. Hence, aside from early recognition to improve outcomes we should anticipate its possible occurrence in patients with high disease activity.
Lupus Erythematosus, Systemic ; Cyclophosphamide ; Nephritis ; Methylprednisolone

BACKGROUND: Lupus nephritis is very common complications in SLE, with clinical symptoms of renal involvement occurring in 30%-70% of patients. Outcomes in children with proliferative lupus nephritis (PLN) show 9-15% progress to end-stage renal disease (ESRD) at 5 years.

OBJECTIVES: This study compared the outcome of children and adolescent patients with lupus nephritis treated with 9 month versus 6 month induction of cyclophosphamide therapy. Renal frequency and adverse effects of IV cyclophosphamide during and after induction therapy were described and determined.

DESIGN: Retrospective Cohort Study

SETTING: Tertiary Hospital

METHODS: Retrospective cohort study comparing 6 and 9 month protocol of IV cyclophosphamide for lupus nephritis were conducted in a government tertiary pediatric hospital in the Philippines. A total of 39 patients with lupus nephritis were gathered, 23 patients underwent 6 months and 16 patients underwent 9 months protocol.

RESULTS: The comparison of two protocols in the administration of intravenous cyclophosphamide (IVCY) did not show significant difference between the two in terms of changes in GFR levels, but some evidence of a greater percent increase from baseline with the 6 months protocol post treatment were observed. Among 39 subjects, creatinine, albumin and urinalysis profile did not also differ between the two groups and levels within each group changed insignificantly over time up to 24 months. Proportion of subjects with renal flare ups, adverse effects and who expired during the study period were also essentially similar between the two groups.

CONCLUSION: IV Cyclophosphamide seems efficacious if given at the very beginning of the flare and at the start after patient was diagnosed with lupus nephritis. No statistically difference between the duration of the protocol. Renal flare ups and adverse effects of cyclophosphamide such as nausea, vomiting and headache were observed similarly between two protocols. Diligent follow up is needed for further studies and specificity of the results.