scholarly journals Fibrothorax and Severe Lung Restriction Secondary to Lupus Pleuritis and Its Successful Treatment by Pleurectomy

2002 ◽  
Vol 9 (5) ◽  
pp. 335-337 ◽  
Author(s):  
Sat Sharma ◽  
Robert Smith ◽  
Fahad Al-Hameed
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Medical Therapy ◽  
Lupus Erythematosus ◽  
Present Report ◽  
Pulmonary Function Tests ◽  
Immunosuppressive Agents ◽  
Systemic Lupus ◽  
Pleural Thickening ◽  
Restrictive Defect ◽  
The Right

Pleural disease is a common pulmonary manifestation of systemic lupus erythematosus (SLE) that usually responds to corticosteroids and other immunosuppressive agents. In the present report, a new approach, pleural decortication, was used in a patient with medically refractory chronic pleuritis secondary to severe SLE. A 26-year-old woman with known SLE developed progressive dyspnea and pleuritic chest pain over several months. The other systemic manifestations of her lupus were controlled with cyclophosphamide and prednisone. A computed tomography scan revealed a persistent, small, loculated right pleural effusion; pleural thickening; and atelectasis of the right middle and lower lobes. Pulmonary function tests showed a severe restrictive defect. The patient was disabled by her severe dyspnea despite maximal medical therapy, and, therefore, surgery was considered. A right thoracotomy revealed entrapment of the right lung by dense visceral pleura. Decortication was performed. On pathology, pleuritis with vascular pleural adhesions was found. No lupus pneumonitis was noted. Postoperatively, a significant clinical improvement in dyspnea was evident within several weeks. On a 6 min walk test, the patient achieved 384 m with a Borg dyspnea scale rating of 2 compared with 220 m and a Borg dyspnea scale rating of 4 preoperatively. Her forced vital capacity improved from 24% predicted to 47% predicted, and her total lung capacity improved from 35% predicted to 54% predicted. Medical therapy of systemic lupus erythematosus has been proven to be effective in controlling pleuritis in most cases. However, in the event of refractory pleuritis or pleural thickening, decortication may be a viable alternative.

scholarly journals SYSTEMIC LUPUS ERYTHEMATOSUS;

2017 ◽  
Vol 24 (01) ◽  
pp. 14-20
Author(s):  
Muhammad Afzal Hamdani ◽  
Khalid Parvez ◽  
Faisal Naseeb ◽  
Umair Afzal ◽  
Bashiruddin - ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Pulmonary Function Tests ◽  
University Hospital ◽  
Systemic Lupus ◽  
High Resolution Computed Tomography ◽  
Cross Sectional ◽  
Pleural Thickening ◽  
Organ Systems ◽  
Pulmonary Manifestations

Systemic lupus erythematosus (SLE), is a multifactorial, complex etiologicaldisorder, characterized by inflammation and involvement of multiple organ systems includinglungs. Objective: 1-To evaluate whether high resolution computed tomography (HRCT) helpsin the diagnosis of pulmonary manifestations of SLE. 2-To study the pattern and extent of lunginvolvement using HRCT. Design: A Prospective cross - sectional clinical study. Period: Fouryears, July 2012 to June 2016. Setting: King Khalid University Hospital (KKUH) King SaudUniversity (KSU), Rheumatology division Department of Medicine. Methods: This study included113 patients attending outpatients or admitted as inpatients having respiratory symptomsand diagnosed as SLE according to American College of Rheumatology (ACR) classificationcriteria. Chest X- ray, pulmonary function tests, and HRCT chest were done. Investigationsto detect other organ involvement were done. Pregnant females and patients having otherconnective tissue or occupational diseases were excluded. Results: Of the total 113 patients102 were female and 11 males. Age of patients was 37.1 ±13.0 years. The HRCT abnormalitieswere pleural effusion, pleural thickening, atelectasis, ground glass opacities including nonspecificinterstitial pneumonitis (NSIP) and usual interstitial pneumonitis (UIP), pulmonaryarterial hypertension, pulmonary embolism and hilar lymphadenopathy. Conclusion: Variouspulmonary manifestations are present in a significant number of symptomatic SLE patients anda variety of HRCT patterns can be seen to diagnose and treat them.

Shrinking lung syndrome in pediatric systemic lupus erythematosus

Lupus ◽  
2021 ◽  
pp. 096120332110103
Author(s):  
Alfonso Ragnar Torres Jimenez ◽  
Nayma Ruiz Vela ◽  
Adriana Ivonne Cespedes Cruz ◽  
Alejandra Velazquez Cruz ◽  
Alma Karina Bernardino Gonzalez
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pleural Effusion ◽  
Pulmonary Function ◽  
Lupus Erythematosus ◽  
Pulmonary Function Tests ◽  
Age At Diagnosis ◽  
X Rays ◽  
Systemic Lupus ◽  
Function Tests ◽  
Restrictive Pattern

Shrinking Lung Syndrome (SLS) is a rare and little known complication associated with Systemic Lupus Erythematosus (SLE), characterized by progressive and unexplainable dyspnea, pleuritic pain, small pulmonary volumes and elevation of the diaphragm on chest X-rays as well as restrictive pattern on pulmonary function tests. Objective To describe clinical, radiological and treatment characteristics in pediatric patients with SLS. Material and methods This is a descriptive and retrospective study in patients under 16 years old with the diagnosis of SLE complicated by SLS at the General Hospital. National Medical Center La Raza. Clinical, radiological and treatment variables were analyzed. Results are shown in frequencies and percentages. Results Data from 11 patients, 9 females and 2 males were collected. Mean age at diagnosis of SLS was 12.2 years. Age at diagnosis of SLE was 11.1 years. SLEDAI 17.3. Renal desease 72%, hematological 91%, lymphopenia 63%, mucocutaneous 72%, neurological 9%, arthritis 54%, serositis 91%, fever 81%, secondary antiphospholipid syndrome, low C3 72%, low C4 81%, positive ANA 91%, positive anti-DNA 91%. Regarding clinical manifestations of SLE: cough 81%, dyspnea 91%, hipoxemia 81%, pleuritic pain 71%, average oxygen saturation 83%. Chest X-rays findings: right hemidiaphragm affection 18%, left 63%, bilateral 18%. Elevated hemidiaphragm 91%, atelectasis 18%, pleural effusion 91%, over one third of the cardiac silhouette under the diphragm 36%, bulging diaphragm 45%, 5th. anterior rib that crosses over the diaphragm 91%. M-mode ultrasound: diaphragmatic hypomotility 100%, pleural effusion 63%. Pulmonary function tests: restrictive pattern in 45% of the cases. Treatment was with supplementary oxygen 100%, intubation 18%, antibiotics 100%, steroids 100%, intravenous immunoglobulin 54%, plasmapheresis 18%, cyclophosphamide 54% and rituximab 18%. The clinical course was favorable in 81%. Conclusions SLS should be suspected in patients with SLE and active disease who present hipoxemia, pleuritic pain, cough, dyspnea, pleural effusion and signs of restriction on chest X-rays. Therefore, a diaphragmatic M-mode ultrasound should be performed in order to establish the diagnosis.

Different cerebral functional segregation in Sjogren’s syndrome with or without systemic lupus erythematosus revealed by amplitude of low-frequency fluctuation

2021 ◽  
pp. 028418512110324
Author(s):  
Xiao-Dong Zhang ◽  
Jun Ke ◽  
Jing-Li Li ◽  
Yun-Yan Su ◽  
Jia-Min Zhou ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Low Frequency ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Frequency Fluctuation ◽  
Parahippocampal Gyrus ◽  
Systemic Lupus ◽  
The Right ◽  
Left Insula

Background Sjögren’s syndrome (SjS) associated with systemic lupus erythematosus (SjS-SLE) was considered a standalone but often-overlooked entity. Purpose To assess altered spontaneous brain activity in SjS-SLE and SjS using amplitude of low-frequency fluctuation (ALFF). Material and Methods Sixteen patients with SjS-SLE, 17 patients with SjS, and 17 matched controls underwent neuropsychological tests and subsequent resting-state functional magnetic resonance imaging (fMRI) examinations. The ALFF value was calculated based on blood oxygen level dependent (BOLD) fMRI. Statistical parametric mapping was utilized to analyze between-group differences and multiple comparison was corrected with Analysis of Functional NeuroImages 3dClustSim. Then, the ALFFs of brain regions with significant differences among the three groups were correlated to corresponding clinical and neuropsychological variables by Pearson correlation. Results ALFF differences in the bilateral precuneus/posterior cingulate cortex (PCC), right parahippocampal gyrus/caudate/insula, and left insula were found among the three groups. Both SjS-SLE and SjS displayed decreased ALFF in the right parahippocampal gyrus, right insula, and left insula than HC. Moreover, SjS-SLE showed wider decreased ALFF in the bilateral precuneus and right caudate, while the SjS group exhibited increased ALFF in the bilateral PCC. Additionally, patients with SjS-SLE exhibited lower ALFF values in the bilateral PCC and precuneus than SjS. Moreover, ALFF values in the right parahippocampal gyrus and PCC were negatively correlated to fatigue score and disease duration, respectively, in SjS-SLE. Conclusion SjS-SLE and SjS exhibited common and different alteration of cerebral functional segregation revealed by AlFF analysis. This result appeared to indicate that SjS-SLE might be different from SjS with a neuroimaging standpoint.

scholarly journals A patient with systemic lupus erythematosus and lupus nephritis: A 12-year follow-up

2011 ◽  
Vol 68 (8) ◽  
pp. 705-708
Author(s):  
Natasa Jovanovic ◽  
Jasmina Markovic-Lipkovski ◽  
Stevan Pavlovic ◽  
Biljana Stojimirovic
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Immunosuppressive Drugs ◽  
Systemic Lupus ◽  
Younger Women ◽  
The Right

Introduction. Systemic lupus erythematosus (SLE) is a chronic immunological disease causing a significant morbidity and mortality in younger women and involving several organs and systems, most often the kidneys, being consequently the incidence of lupus nephritis (LN) about 60%. Case report. We reported a 57 year-old patient with the diagnosed SLE in 1995. Pathohistological analysis of kidney biopsy revealed LN type V. The patient was treated with corticosteroid pulses and azathioprine during one year. A remission was achieved and maintained with prednisone, 15 mg daily. Nephrotic relapse was diagnosed in 2006 and the second kidney biopsy revealed recent kidney infarction due to extensive vasculitis. Soon, a cerebrovascul insult developed and CT-scan revealed endocranial infarctus. The patient was treated with corticosteroids and cyclophosphamide pulses (totally VI monthly pulses), and also with low-molecular heparine, anticoagulants and salicylates because of the right leg phlebothrombosis. After the pulses, the patient was adviced to take prednisone 20 mg daily and azothioprine 100 mg daily, and 6 months later mycophenolate mofetil because of persistent active serological immunological findings (ANA 1 : 320) and nephrotic syndrome. Mycophenolate mofetil was efficient in inducing and maintaining remission of nephrotic syndrome. Conclusion. The aim of LN treatment is to achieve and maintain remission, improve patients? outcome, reduce the toxicity of immunosuppressive drugs and the incidence of relapses. Mycophenolate mofetil was shown to be efficient in inducing and maintaining remission of nephrotic syndrome in the frame of LN.

Non-central serous chorioretinopathy in a patient with systemic lupus erythematosus and hydroxychloroquine retinopathy

2021 ◽  
Vol 14 (1) ◽  
pp. e237243
Author(s):  
Diogo Hipolito-Fernandes ◽  
Maria Elisa Luís ◽  
Rita Flores ◽  
Rita Anjos
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Central Serous Chorioretinopathy ◽  
Vision Loss ◽  
Subretinal Fluid ◽  
Ophthalmological Examination ◽  
Systemic Lupus ◽  
Diagnostic Challenge ◽  
Hydroxychloroquine Retinopathy ◽  
The Right

Subretinal fluid accumulation in a patient with systemic lupus erythematosus (SLE) may represent a diagnostic challenge. We present a case of a 43-year-old man with baseline diagnosis of SLE and hydroxychloroquine-associated maculopathy who reported progressive vision loss on the right eye, associated with corticosteroids use for an arthritic crisis. Ophthalmological examination did not reveal any acute finding. On optical coherence tomography, subretinal fluid in the perifoveal area was visible on the right eye, with corresponding enlargement of the visual field defect. An increased choroidal thickness was also visible. Fluorescein angiography revealed, on the right eye, two pinpoint areas of leakage and indocyanine green angiography signs of choroidal vascular hyperpermeability. Considering a diagnosis of a non-central central serous chorioretinopathy, corticosteroids use was interrupted, with resolution of the subretinal fluid. This case illustrates the relevance of a multimodal imaging approach to guide the diagnosis of patient with an SLE with subretinal fluid.

Systemic Lupus Erythematosus and Related Disorders

2014 ◽  
pp. 257-270
Author(s):  
Bonnie L. Bermas
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Central Nervous System ◽  
Nervous System ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Future Research ◽  
High Dose ◽  
Childbearing Age ◽  
Systemic Lupus ◽  
Multisystem Disease

Systemic lupus erythematosus (SLE) is a multisystem disease that preferentially affects women of childbearing age. This disorder is both more common and more severe in individuals of African and Asian ancestry. The etiology of SLE is not well understood, although genetics and environmental stimuli clearly are involved. Whether this disease is caused by a T-cell, B-cell, or other immunologic malfunction is debated, but all would agree that clearly autoantibodies such as antinuclear antibodies and anti–double-stranded DNA contribute to the pathophysiology of this disorder. This multisystem disease can affect the skin, joints, lungs, heart, kidneys, and central nervous system. Most of the morbidity and mortality is from renal and central nervous system (CNS) involvement, although accelerated atherosclerosis has recently been appreciated as a major contributor to disease burden. The treatment of SLE has improved over the past decade with less reliance on high-dose corticosteroids and more emphasis on immunosuppressive agents. It is our hope that future research into the pathophysiology of this disorder and the development of more specific therapy, such as biologics, will improve the outcome of this disease.

Immunosuppressive therapy of systemic lupus erythematosus associated peripheral neuropathy: A systematic review

Lupus ◽  
2020 ◽  
Vol 29 (12) ◽  
pp. 1509-1519
Author(s):  
Alexandru Constantin ◽  
Daniela Năstase ◽  
Delia Tulbă ◽  
Paul Bălănescu ◽  
Cristian Băicuș
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Peripheral Neuropathy ◽  
Lupus Erythematosus ◽  
Case Reports ◽  
Immunosuppressive Agents ◽  
Case Series ◽  
Systemic Lupus ◽  
Retrospective Case ◽  
Randomized Controlled ◽  
Neuropsychiatric Manifestations

Introduction Peripheral neurologic manifestations may be associated with most of the collagen vascular diseases including systemic lupus erythematosus (SLE), yet most of the times it is not clear what therapy should be prescribed. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations suggest the use of glucocorticoids and immunosuppressive agents for the treatment of SLE associated peripheral neuropathy (PN) (strength of statement A, category of evidence 1), however these recommendations are based on studies that did not focus specifically on PN but rather on neuropsychiatric manifestations of SLE out of which only one was a randomized controlled clinical trial that included 7 patients with peripheral neuropathy. The objective of this systematic review is to determine whether the pathogenic treatments (corticosteroids, immunosuppressive agents, intravenous immunoglobulins, plasmapheresis) are effective for SLE associated PN. Methods We searched MEDLINE for all the studies that included the pathogenic treatment of SLE associated PN. The purpose was to identify randomized clinical trials, and in the absence of these, we included observational studies and case reports or case series. Results The search returned only retrospective case reports or case series. Only one prospective study, a randomized controlled study, was focused on neuropsychiatric SLE and included few patients with PN (7). Some studies reported cases of PN responsive to glucocorticoids (GC), cyclophosphamide (CYC), rituximab (RTX), azathioprine (AZA), plasmapheresis (PPH), intravenous immunoglobulin (IVIG), mycophenolate mofetil (MMF) or different combinations of these immunosuppressive agents, whereas others noticed effectiveness of sequential treatments (i.e. administration of a therapeutic agent after another single agent or a combination of agents had previously failed). Many studies did not mention how the outcomes were objectively measured. Conclusions There are no interventional studies dedicated to the SLE associated PN, only retrospective case reports or case series which not only did they show contradictory results, but they also represent the lowest level of evidence. There is a strong need for new analytical studies dedicated to SLE associated PN. Protocol registered with PROSPERO (number CRD42019121748).

scholarly journals Retinal vasculitis in systemic lupus erythematosus: an indication of active disease

2012 ◽  
Vol 2 (2) ◽  
pp. 54 ◽  
Author(s):  
Umi Kalthum Md Noh ◽  
Aida Zairani A. Zahidin ◽  
Then Kong Yong
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Visual Acuity ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Retinal Vasculitis ◽  
Panretinal Photocoagulation ◽  
Systemic Lupus ◽  
Retinal Hemorrhages ◽  
Fluorescein Angiogram ◽  
Active Disease

A 26-year-old woman with a recent flare-up of systemic lupus erythematosus presented with peripheral retinal hemorrhages at a routine check-up. She is on a tapering dose of immunosuppressive agents. Her visual acuity was good. Fluorescein angiogram revealed vasculitic changes with capillary non-perfusion areas. A few weeks later, she developed cerebral lupus with advanced lupus nephritis. Immunosuppressive therapy was restarted and panretinal photocoagulation was delivered. Her visual acuity remained stable, despite development of a cataract from prednisolone therapy.

scholarly journals Epileptic seizures and EEG features in juvenile systemic lupus erythematosus

2008 ◽  
Vol 66 (3a) ◽  
pp. 468-470 ◽  
Author(s):  
Simone Carreiro Vieira-Karuta ◽  
Izabella Celidônio Bertoldo Silva ◽  
Paulo Breno Noronha Liberalesso ◽  
Márcia Bandeira ◽  
Loris Janz Jr ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Age Of Onset ◽  
Epileptic Seizures ◽  
Temporal Region ◽  
Systemic Lupus ◽  
Juvenile Systemic Lupus Erythematosus ◽  
Epileptiform Discharges ◽  
The Central Nervous System ◽  
The Right

INTRODUCTION: Juvenile systemic lupus erythematosus is more incident in female affecting different systems including the central nervous system. The aim of this study was to check the incidence of seizures and electroencephalographic features in these patients. METHOD: It was analyzed all patients with juvenile systemic lupus erythematosus referred to the Pequeno Príncipe Hospital in Curitiba, PR, Brazil, in the year of 2007. The patients were submitted to EEG and subdivided into two groups according to the presence or absence of epileptic seizures. Mann-Whitney statistical test was used. RESULTS: Forty-nine cases were included, there were 73.45% female, with an age between 3 and 28 years (µ=17.00 years; s=5.01 years). Seizures (13/26.50%) were the most frequent manifestation followed by headache (13/26.50%) and ischemic stroke (6/12.25%). Cerebral vasculites were the most frequent alteration in neuroimage. The abnormalities of EEG were characterized by asymmetry of the electric cerebral activity, diffuse disorganized background activity, focal epileptiform discharges in the right central-temporal region, generalized paroxysmal of 3 Hz spike-waves, and bursts of theta-delta slowness activity in the right parietal-occiptal region. The statistic analysis showed no significantly difference between age of onset of symptoms and the risk of seizures (p 0.675) as well as between time of the disease and the risk of seizures (p 0.436). CONCLUSION: Neurologic manifestations, in special epileptic seizures, are frequent in systemic lupus erythematosus. Age of onset of symptoms and the time of disease did not increase the risk of epileptic seizures in this disease.

scholarly journals Bullous Systemic Lupus Erythematosus Associated with Esophagitis Dissecans Superficialis

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Meera Yogarajah ◽  
Bhradeev Sivasambu ◽  
Eric A. Jaffe
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Skin Lesions ◽  
Esophageal Mucosa ◽  
Systemic Lupus ◽  
Neck Lymph Node ◽  
The Face ◽  
History Of ◽  
The Right ◽  
Pruritic Skin

Bullous systemic lupus erythematosus is one of the rare autoantibody mediated skin manifestation of systemic lupus erythematosus (SLE) demonstrating subepidermal blistering with neutrophilic infiltrate histologically. We present a case of a 40-year-old Hispanic female who presented with a several months’ history of multiple blistering pruritic skin lesions involving the face and trunk, a photosensitive rash over the face and neck, swelling of the right neck lymph node, and joint pain involving her elbows and wrist. Her malady was diagnosed as bullous systemic lupus erythematosus based on the immunological workup and biopsy of her skin lesions. The patient also complained of odynophagia and endoscopy revealed esophagitis dissecans superficialis which is a rare endoscopic finding characterized by sloughing of the esophageal mucosa. The bullous disorders typically associated with esophagitis dissecans superficialis are pemphigus and rarely bullous pemphigoid. However, this is the first reported case of bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis.

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