Background: Seizures in patients with systemic lupus erythematosus (SLE) are uncommon but life-threatening; mortality rate is 25-29%. Seizure in a person with lupus may be due to lupus itself or other conditions. There are no published studies describing the causes and outcomes of seizures in Filipino patients with lupus. Objective: To describe the causes and outcomes of seizure in a cohort of patients with lupus seen at Philippine General Hospital. Methodology: We reviewed the medical records of patients with SLE) with a documented seizure and admitted between January 2016 and April 2019. History, physical examination and laboratory findings, and clinical course were obtained. Results: We included 29 patients with 31 seizure events. They were all women, mostly single, of low socio-economic status, and had poor functional capacity. Lupus was active in 77.4% (24/31), commonly with mucocutaneous or hematologic manifestations. Seizures were generalized in 87 % (27/31). Prior to seizure, one-third had headache, fever, and vomiting. There were no neurologic localizing signs. Twenty-four seizure events (77%) occurred among patients with active lupus; 16 (67%) was attributed to neuropsychiatric systemic lupus erythematosus (NPSLE) and eight (33%) to other causes: infection (tuberculous meningitis and septic encephalopathy), posterior reversible encephalopathy syndrome (PRES), uremia, arrhythmia, and eclampsia. Seven seizures in inactive lupus were not SLE-related. Mortality rate was 28%; infection was the most common cause. Seizure resolved in 97%. Mean duration of hospitalization was 26.7 days. Patients were discharged improved from 19 seizure events (18 patients); 14 had follow-up consultations, three were readmitted. There was no seizure recurrence within 30 days of discharge. There was improvement in functional capacity. Conclusion The most common cause of seizure was NPSLE, followed by infection. Despite high rates of complete seizure resolution, poor outcomes were noted in almost half of the patients. Prolonged hospitalization was common. A high rate of mortality was observed. Infection was the most common cause of mortality.
Lupus Vasculitis, Central Nervous System

Humans ; Integrative Medicine ; Lupus Vasculitis, Central Nervous System ; therapy

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

The clinical manifestations of nervous system involvement in systemic lupus erythematosus (SLE) are highly diverse and their pathogenic mechanisms are incompletely understood. Neuropsychiatric SLE (NPSLE) poses difficulty in making the proper diagnosis, especially in circumstances where its initial symptoms are diffuse neuropsychiatric symptoms. We describe a 43-year old woman who exhibited a myoclonic jerk of the abdominal wall, followed shortly by acute confusion, which was attributed to SLE. Therapy with high dose corticosteroids completely reversed the symptoms. Myoclonus can be an anticipatory symptom of diffuse neurologic dysfunction in patients with NPSLE.
Abdominal Wall ; Adrenal Cortex Hormones ; Central Nervous System ; Female ; Humans ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System ; Myoclonus ; Nervous System ; Neurologic Manifestations

Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions.
Adult ; Autoimmune Diseases ; Central Nervous System ; Diagnosis ; Erythema ; Female ; Fingers ; Follow-Up Studies ; Humans ; Infarction ; Knee ; Lupus Erythematosus, Systemic* ; Malignant Atrophic Papulosis ; Skin ; Vasculitis ; Vasculitis, Central Nervous System

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 analyze clinical characteristics of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) and to explore the risk factors affecting the occurrence of NPSLE. Methods A total of 63 NPSLE patients and 61 non-NPSLE patients were enrolled. The clinical manifestations and laboratory examination data of the two groups were collected, and the disease characteristics of NPSLE were summarized to analyze the risk factors affecting the occurrence of NPSLE by multivariate Logistic regression. Results The most common clinical manifestations of NPSLE patients were headache (39.7%), affective disorder (33.3%) and cognitive impairment (30.2%), with cranial magnetic resonance abnormalities (63.5%) and a high cerebrospinal fluid protein positive rate (52.4%). Compared with non-NPSLE patients, there were significantly increased levels of Raynaud's phenomenon, renal involvement, anti-RNP antibody, anti-ribosomal P protein, hypocomplementemia, lymphocyte-to-monocyte ratio (LMR) and neutrophil-to-lymphocyte ratio (NLR) in NPSLE patients. Multivariate Logistic regression analysis showed that renal involvement, Raynaud's phenomenon, positive anti-ribosomal P protein antibody, and elevated LMR and NLR were independent risk factors for NPSLE. Conclusion Headache is the most common symptom in patients with NPSLE, and abnormal cranial MRI and cerebrospinal fluid examination are more common. SLE patients who present with renal involvement, Raynaud's phenomenon, positive anti-ribosomal P protein antibodies, and elevated levels of LMR and NLR are more susceptible to developing NPSLE.
Humans ; Lupus Vasculitis, Central Nervous System ; Risk Factors ; Headache ; Antibodies, Antinuclear ; Cognitive Dysfunction

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

OBJECTIVETo investigate the characteristic manifestations of neuropsychiatric systemic lupus erythematosus (NPSLE) in clinical practice.

METHODSA total of 198 NPSLE cases from 1998 to 2003 in Peking Union Medical College Hospital, were included. Their clinical manifestations and the results of accessory examination such as serology, lumbar puncture, head magnetic resonance imaging, and electroencephalogram were analyzed.

RESULTSThe most frequent manifestations of the NPSLE included: headache, psychiatric disorder, seizure, stroke, and acute confusional state. Fifty-two patients took head magnic resonance imaging, and 34 of them showed abnormal, including stroke attack. Among 48 patients who only had a complaint of headache, the increase of intracranial pressure and cerebral spinal fluid protein was significantly higher than other NPSLE syndromes (P < 0.05).

CONCLUSIONDiagnosis of NPSLE was mainly based on its clinical manifestations. Head magnetic resonance imaging and cerebral spinal fluid examination may be helpful. More specific accessory examinations require further development.

Adult ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Lupus Vasculitis, Central Nervous System ; cerebrospinal fluid ; diagnosis ; Magnetic Resonance Imaging ; Male

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown cause, and is characterized by appearance of various autoantibodies and multiple organ involvement. Some of the systems commonly involved in SLE include the muscle and joints, brain and peripheral nervous system, lungs, heart, kidneys, skin, serous membranes, and component of blood. The clinical manifestations of SLE is protean, overlap with other illnesses, and are often subtle. However there are few documented reports on SLE associated with Crohn's disease in other countries and no reporting case in Korea. We experienced a 30-year-old female patient who was admitted for right leg swelling and diagnosed as SLE with Crohn's disease.
Adult ; Autoantibodies ; Brain ; Crohn Disease* ; Female ; Heart ; Humans ; Joints ; Kidney ; Korea ; Leg ; Lung ; Lupus Erythematosus, Systemic* ; Lupus Vasculitis, Central Nervous System ; Peripheral Nervous System ; Serous Membrane ; Skin