scholarly journals PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY IN AN ADULT PATIENT WITHSYSTEMICLUPUSERYTHEMATOSUS

2020 ◽  
Vol 8 (11) ◽  
pp. 791-798
Author(s):  
Khalid Abdullah Alghamdi ◽  
◽  
Ahmed Saeed Almaqati ◽  
Nuha Adnan Meraiani ◽  
Nawal Bassuni ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Lupus Erythematosus ◽  
Brain Mri ◽  
Immunosuppressive Agents ◽  
Clinical Manifestations ◽  
White Matter Lesions ◽  
Subcortical White Matter ◽  
Systemic Lupus ◽  
Mri Findings

We report a case of 40-year-old female who is known to have systemic lupus erythematosus (SLE) presenting with severe cognitive impairment with lymphopenia, proteinuria, and evidence of SLE serological activity. Initially, she was managed as a case of neuropsychiatric systemic lupus erythematosus (NPSLE) with Cyclophosphamide and pulse steroids. However, she has been deteriorating clinically in forms of right sided hemiparesis, blindness, and aphasia despite normalization of complements and anti-double stranded DNA antibodies levels. Diagnosis of progressive multifocal leukoencephalopathy (PML) was made based on her clinical manifestations with brain MRI findings of subcortical white matter lesions and detection of John Cunningham virus (JCV) in cerebrospinal fluid analysis. Immunosuppressive agents were discontinued aiming for immune system restoration.

scholarly journals Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology

2016 ◽  
Vol 8 (1) ◽  
pp. 59-65 ◽  
Author(s):  
Shala Ghaderi Berntsson ◽  
Evangelos Katsarogiannis ◽  
Filipa Lourenço ◽  
Maria Francisca Moraes-Fontes
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Lupus Erythematosus ◽  
Jc Virus ◽  
Brain Mri ◽  
Fatal Outcome ◽  
Disease Onset ◽  
Unmet Need ◽  
Human Polyomavirus ◽  
Systemic Lupus

Progressive multifocal leukoencephalopathy (PML) caused by reactivation of the JC virus (JCV), a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE). We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine) since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions) and JCV isolation from the cerebrospinal fluid (700 copies/ml). IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need.

FRI0327 Brain mri findings in patients with diffuse psychiatric/neuropsychological syndromes in systemic lupus erythematosus

2013 ◽  
Vol 72 (Suppl 3) ◽  
pp. A485.1-A485
Author(s):  
Y. Arinuma ◽  
H. Kikuchi ◽  
E. Ogawa ◽  
T. Wada ◽  
T. Nagai ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Brain Mri ◽  
Systemic Lupus ◽  
Mri Findings

Systemic Lupus Erythematosus: A Therapeutic Update

2008 ◽  
Vol 22 (1) ◽  
pp. 29-52 ◽  
Author(s):  
Catherine A. Heyneman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Clinical Manifestations ◽  
Disease Process ◽  
Immunomodulatory Drugs ◽  
Systemic Lupus ◽  
Cellular Mechanisms ◽  
Complex Autoimmune Disease ◽  
Active Lupus

Systemic lupus erythematosus (SLE) is a complex autoimmune disease with variable clinical manifestations that is characterized by flares and periods of relative quiescence. The disease occurs approximately 10 times more frequently in women and is more prevalent among certain ethnic groups. The etiology is complex and dependent upon an interaction of genetic, hormonal, and environmental factors. Corticosteroids and immunosuppressive agents have transformed the outlook for patients with lupus. Unfortunately, the increased lifespan unmasked an accelerated process of atherosclerosis and cardiovascular disease. Early mortality is usually attributable to active lupus, but deaths late in the disease process are often secondary to thrombotic events. Advancements in the understanding of molecular and cellular mechanisms involved in the pathogenesis have resulted in development of novel therapies. Immunomodulatory drugs developed for other diseases are being investigated for use in specific manifestations of lupus. Individualization of treatment and lifelong monitoring are required in most patients.

Systemic Lupus Erythematosus

2019 ◽  
pp. 183-184
Author(s):  
Josephina A. Vossen
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Laboratory Testing ◽  
Immunosuppressive Agents ◽  
Clinical Manifestations ◽  
Connective Tissue Disorder ◽  
Systemic Lupus ◽  
Imaging Evaluation ◽  
Wide Range ◽  
Initial Imaging

Chapter 40 discusses systemic lupus erythematosus (SLE), which is an autoimmune connective tissue disorder with a wide range of clinical features. SLE follows a relapsing and remitting course and predominantly affects women. The clinical manifestations are highly variable, ranging from indolent to fulminant. Arthritis is one of the most common manifestations of SLE. Diagnosis is based on clinical assessment supported by laboratory testing. Imaging of the musculoskeletal system is used primarily for the assessment of symptomatic joints. The initial imaging evaluation of SLE arthropathy is with radiographs of symptomatic joints, usually the hands. Treatment ranges from corticosteroids to immunosuppressive agents.

scholarly journals Case of Acute Disseminated Encephalomyelitis Associated with Cytomegalovirus Reactivation in an Immunocompromised Systemic Lupus Erythematosus Patient

Medicina ◽  
2021 ◽  
Vol 57 (9) ◽  
pp. 882
Author(s):  
Mirela Pavicic Ivelja ◽  
Kresimir Dolic ◽  
Daniela Marasovic Krstulovic ◽  
Gordana Glavina ◽  
Ivo Ivic
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Brain Mri ◽  
Acute Disseminated Encephalomyelitis ◽  
Cognitive Disability ◽  
Systemic Lupus ◽  
Mri Findings ◽  
Cns Lupus ◽  
Ischemic Cerebrovascular Disease ◽  
Wide Range

We present a case of an immunocompromised systemic lupus erythematosus female patient admitted to our hospital for general impairment, monoparesis, and temporary cognitive disability. The case represented a significant diagnostic and therapeutic challenge primarily because of a wide range of differential diagnosis options (CNS lupus, ischemic cerebrovascular disease, viral meningoencephalitis, progressive multifocal leukoencephalopathy, limbic encephalitis, and acute disseminated encephalomyelitis—ADEM). Brain MRI findings were compatible with ADEM, and microbiological tests showed a cytomegalovirus infection (CMV) which is rarely associated with ADEM despite the increasing number of immunocompromised patients prone to symptomatic CMV reactivation. Our patient was treated with intravenous methylprednisolone, immunoglobulin (IVIG), along with antiviral therapy resulting in a favorable therapeutic effect. In conclusion, only a few described ADEM cases have been associated with CMV, and none of them, to the best of our knowledge, in an immunocompromised patient. In this case, a multidisciplinary approach and broad diagnostic considerations were decisive for successful treatment and outcome.

Clinical case of lung lesions in patient with newly diagnosed systemic lupus erythematosus

2019 ◽  
Vol 1 (9) ◽  
pp. 53-57
Author(s):  
T. N. Gavva ◽  
L. V. Kuzmenkova ◽  
Yu. N. Fedulaev ◽  
T. V. Pinchuk ◽  
D. D. Kaminer ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Case ◽  
Clinical Manifestations ◽  
Lung Damage ◽  
Newly Diagnosed ◽  
Systemic Lupus ◽  
Lung Lesions ◽  
Laboratory Parameters ◽  
Clinical Interest

A case of lung damage in systemic lupus erythematosus (SLE) in a 33-year-old woman is described. This case is of clinical interest due to the complexity of diagnosis due to the fact that SLE is a disease with diverse clinical manifestations involving many organs and systems, which often makes it difficult to timely recognize the onset of the disease. SLE still remains a challenge and requires special attention to the patient s history, clinical and laboratory parameters of the patient, as well as specific immunological examinations.

THE CLINICAL, LABORATORY MANIFESTATIONS AND HISTOPATHOLOGY IN NEPHROTIC PATIENTS WITH LUPUS NEPHRITIS

2018 ◽  
pp. 52-58
Author(s):  
Le Thuan Nguyen ◽  
Bui Bao Hoang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Clinical Laboratory ◽  
Activity Index ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Cross Sectional ◽  
Organ Systems ◽  
Tubulointerstitial Lesions

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organ systems. The kidney appears to be the most commonly affected organ, especially nephrotic is a serious kidney injury. The clinical, laboratory manifestations and histopathology are very useful for diagnosis, provide the means of predicting prognosis and guiding therapy in nephrotic patients with lupus nephritis. Methods: Descriptive cross-sectional study of nephrotic patients with lupus treated in the Department of Nephrology Trung Vuong Hospital and Cho Ray Hospital between May/2014 and May/2017. Renal histopathological lesions were classified according to International Society of Nephrology/Renal Pathology Society - ISN/RPS ’s 2003. The clinical, laboratory manifestations and histopathological features were described. Results: Of 32 LN with nephritic range proteinuria cases studied, 93.7% were women. The 3 most common clinical manifestations were edema (93.8%), hypertension (96.8%) and pallor (68.9%), musculoskeletal manifestions (46.9%), malar rash (40.6%). There was significant rise in laboratory and immunological manifestions with hematuria (78.1%), Hb < 12g/dL (93.5%), increased Cholesterol (100%), and Triglycerid (87.5%), Creatinine > 1.4 mg/dL (87.5%), increased BUN 71.9%, ANA (+) 93.8%, Anti Ds DNA(+) 96.9%, low C3: 96.9%, low C4: 84.4%. The most various and severe features were noted in class IV with active tubulointerstitial lesions and high activity index. Conclusion: Lupus nephritis with nephrotic range proteinuria has the more severity of histopathological feature and the more severity of the more systemic organ involvements and laboratory disorders were noted. Key words: Systemic lupus, erythematosus (SLE) lupus nepphritis, clinical

Adult systemic lupus erythematosus in Egypt: The nation-wide spectrum of 3661 patients and world-wide standpoint

Lupus ◽  
2021 ◽  
pp. 096120332110142
Author(s):  
Tamer A Gheita ◽  
Rasha Abdel Noor ◽  
Esam Abualfadl ◽  
Osama S Abousehly ◽  
Iman I El-Gazzar ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Age Of Onset ◽  
Wide Spectrum ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Population Based ◽  
Systemic Lupus ◽  
Cross Sectional

Objective The aim of this study was to present the epidemiology, clinical manifestations and treatment pattern of systemic lupus erythematosus (SLE) in Egyptian patients over the country and compare the findings to large cohorts worldwide. Objectives were extended to focus on the age at onset and gender driven influence on the disease characteristics. Patients and method This population-based, multicenter, cross-sectional study included 3661 adult SLE patients from Egyptian rheumatology departments across the nation. Demographic, clinical, and therapeutic data were assessed for all patients. Results The study included 3661 patients; 3296 females and 365 males (9.03:1) and the median age was 30 years (17–79 years), disease duration 4 years (0–75 years) while the median age at disease onset was 25 years (4–75 years). The overall estimated prevalence of adult SLE in Egypt was 6.1/100,000 population (1.2/100,000 males and 11.3/100,000 females).There were 316 (8.6%) juvenile-onset (Jo-SLE) and 3345 adult-onset (Ao-SLE). Age at onset was highest in South and lowest in Cairo (p < 0.0001). Conclusion SLE in Egypt had a wide variety of clinical and immunological manifestations, with some similarities with that in other nations and differences within the same country. The clinical characteristics, autoantibodies and comorbidities are comparable between Ao-SLE and Jo-SLE. The frequency of various clinical and immunological manifestations varied between gender. Additional studies are needed to determine the underlying factors contributing to gender and age of onset differences.

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