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.

scholarly journals Successful Treatment of Hemophagocytic Lymphohistiocytosis Associated with Lupus Nephritis by Using Mycophenolate Mofetil

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Takashi Nawata ◽  
Makoto Kubo ◽  
Kosaku Shiragami ◽  
Yukinori Nakamura ◽  
Masafumi Yano
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Corticosteroid Treatment ◽  
Immunosuppressive Drugs ◽  
Japanese Woman ◽  
Systemic Lupus ◽  
Old Japanese

An estimated 0.9% to 2.4% of patients with systemic lupus erythematosus (SLE) also have hemophagocytic lymphohistiocytosis (HLH). HLH associated with autoimmune diseases is often refractory to corticosteroid treatment; thus, additional immunosuppressive drugs, such as cyclosporine, cyclophosphamide, or tacrolimus, are required. Here, we describe the case of a 44-year-old Japanese woman who developed HLH associated with lupus nephritis. Initially, her HLH was refractory to treatment with a corticosteroid, tacrolimus, and mizoribine. However, alternative treatment with a corticosteroid, mycophenolate mofetil, and tacrolimus improved both her HLH and lupus nephritis. This case suggests the possibility of mycophenolate mofetil as a key drug for treating HLH associated with SLE.

scholarly journals The Truth Behind Hematuria

2020 ◽  
Author(s):  
Hai-bo Yan ◽  
Yu-mei Li
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Lupus Nephritis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Right Atrium ◽  
Urinary Protein ◽  
Systemic Lupus ◽  
Multiple Systems ◽  
The Right

Abstract Background: Systemic lupus erythematosus(SLE) is an autoimmune disease involving multiple systems with various clinical manifestations,renal involvement is common, but intracardiac thrombus is rarely reported as a complication of antiphospholipid syndrome. Drug anticoagulation is the first choice, and surgical treatment is performed in severe cases, we report the case to improve clinicians' understanding of disease diagnosis.Case presentation: A 8-year-old girl was admitted to our hospital because of left costal pain, hematuria and fever.She had obvious edema, urinary protein 3+and occult blood 3+,urinary protein 3.2g/24h, albumin 17.6g/L, total cholesterol 7.21mmol/L, consistent with diagnosis of nephrotic syndrome. Continued to track the etiology of nephrotic syndrome and performed a renal biopsy, dsDNA 1:10 positive,low C3, low platelets and hemoglobin, anti-cardiolipin IgM 12U/ml, anti-β2-glycoprotein I 223R/ml, renal pathology suggested lupus nephritis, finally diagnosed as systemic lupus erythematosus, secondary anticardiolipin syndrome, lupus nephritis. The patient was treated with hormone and immunosuppressant. Sixteen weeks later, urinary protein 1+,the quantity of urine protein was less than 0.5g/d. Echocardiography showed that the mass in the right atrium was thrombosis. Heparin anticoagulant therapy is effective.Conclusion: Systemic lupus erythematosus can involve multiple systems,various complications, it is rare to have thrombus in the right atrium as a complication of antiphospholipid syndrome, early diagnosis and treatment is the key to improve the prognosis of children.

scholarly journals Novel Therapeutic Interventions in Systemic Lupus Erythematosus

2021 ◽  
Author(s):  
Panagiotis Athanassiou ◽  
Lambros Athanassiou ◽  
Ifigenia Kostoglou-Athanassiou
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Induction Treatment ◽  
Systemic Lupus ◽  
Beneficial Effects ◽  
Anti Cd20 ◽  
Steroid Sparing

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease. It is characterized by a variable clinical course ranging from mild to fatal disease. It can affect the kidneys. The aim of treatment in SLE is the prevention of flares and the prevention of accumulation of damage to the main organs affected as well as the prevention of drug side effects. The cornerstone of SLE treatment is hydroxychloroquine. Corticosteroids are used both as induction treatment in disease flares as well as in small doses as maintenance treatment. Immunosuppressants, such as azathioprine, methotrexate and mycophenolate mofetil are used as steroid sparing agents. Calcineurin inhibitors, namely tacrolimus and cyclosporin A may also be used as immunosuppressants and steroid sparing agents. Pulse methylprednisolone, along with mycophenolate mofetil and cyclophosphamide are used as induction treatment in lupus nephritis. Rituximab, an anti-CD20 biologic agent may be used in non-renal SLE. In patients insufficiently controlled with hydroxychloroquine, low dose prednisone and/or immunosuppressive agents, belimumab may be used with beneficial effects in non-renal disease and lupus nephritis.

scholarly journals Secondary angle-closure glaucoma as an ocular presentation of systemic lupus erythematosus: a case report

2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095949
Author(s):  
Xincen Hou ◽  
Wenping Pan ◽  
Anli Wang ◽  
Tao Yu ◽  
Aiping Song
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Immunosuppressive Drugs ◽  
Acute Angle ◽  
Angle Closure Glaucoma ◽  
Angle Closure ◽  
High Dose ◽  
Systemic Lupus

Systemic lupus erythematosus (SLE) is a chronic idiopathic autoimmune disease. SLE can involve almost any part of the eyes. However, bilateral angle-closure glaucoma due to lupus choroidopathy that is accompanied by polyserositis and nephropathy is rare. We report a 21-year-old woman whose clinical manifestations were diagnosed as bilateral angle-closure glaucoma caused by ciliochoroidal effusion. Subsequently, SLE and lupus nephritis were diagnosed on the basis of malar rash, photosensitivity, proteinuria, positive anti-Smith and anti-DNA antibodies, and a renal histopathological biopsy. After 1 month of treatment with steroids and immunosuppressive drugs, the patient’s intraocular pressure returned to normal, visual acuity improved, and lupus nephritis was effectively controlled. Bilateral secondary acute angle closure caused by SLE choroidal disease can be an ocular manifestation of SLE, and is usually accompanied by polyserositis and nephropathy. High-dose steroids and immunosuppressive therapy should be immediately and actively provided for this condition.

scholarly journals Bullous Systemic Lupus Erythematosus and Membranous Lupus Nephritis in a Filipino Child

2019 ◽  
Vol 53 (1) ◽  
Author(s):  
Marc Andrew O. Perez ◽  
Candice B. Brillante ◽  
Lourdes Paula R. Resontoc ◽  
Dolores D. Bonzon ◽  
Francisco E. Anacleto ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Basement Membrane ◽  
Lupus Erythematosus ◽  
Plasma Cells ◽  
Kidney Biopsy ◽  
Direct Immunofluorescence ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Membranous Lupus Nephritis

Bullous eruptions are rare cutaneous manifestations of systemic lupus erythematosus. We report a case of an 8-year old Filipino girl with vesiculobullous systemic lupus erythematosus (SLE) and membranous lupus nephritis on kidney biopsy who presented with clinical nephrotic features of generalized edema, proteinuria, hypoalbuminemia and hyperlipidemia. The 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE were met. Immunohistopathologic examination of the skin lesion revealed a sub-epidermal split with neutrophilic infiltrates along the dermo-epidermal junction, moderate perivascular, periadnexal and interstitial infiltrates composed of predominantly neutrophils with neutrophilic dusts, lymphocytes, plasma cells, rare eosinophils and increased dermal mucin. Direct immunofluorescence showed strong continuous linear IgG deposits along the basement membrane and weak linear IgM and IgA deposition along the basement membrane zone (BMZ). To our knowledge, this is the first report of vesiculobullous SLE in a Filipino child. This case is a rare form of cutaneous lupus in children. Bullous SLE (BSLE) should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.

scholarly journals Characteristics of systemic lupus erythematosus and lupus nephritis in children in the Republic of Belarus and the Republic of Tatarstan

2019 ◽  
Vol 64 (5) ◽  
pp. 199-203
Author(s):  
T. P. Makarova ◽  
A. V. Sukalo ◽  
I. A. Kazyro ◽  
Yu. S. Melnikova ◽  
N. N. Firsova ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Immunosuppressive Drugs ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Severe Manifestation ◽  
Extrarenal Manifestations ◽  
The Republic

Systemic lupus erythematosus is an autoimmune disease characterized by a pronounced polymorphism of clinical manifestations. Lupus nephritis is the most severe manifestation of the disease. The article presents a retrospective analysis of the cases of systemic lupus erythematosus and assessment of the clinical manifestations of the disease and variants of lupus nephritis in children in the Republics of Belarus and Tatarstan. The authors analyzed 60 cases of systemic lupus erythematosus, lupus nephritis. All patients had at least 4 of the 11 diagnostic criteria proposed by the American College of rheumatology (ACR, 1997), and 35 patients had a morphologically verified nephritis. It was found that the disease in children developed very actively with fast multi-organ involvement and it required aggressive therapy with several immunosuppressive drugs. During follow-up, the percentage of patients with renal damage increased, so renal function should be controlled in all patients with systemic lupus erythematosus, especially with early onset. Lupus nephritis is combined with extrarenal manifestations and it is difficult to diagnose when it begins with kidney damage. The overall survival rate of children with systemic lupus erythematosus is closely related to the severity of renal manifestations. Lupus nephritis is a serious problem that requires early aggressive intervention and continuous maintenance therapy.

The patient with systemic lupus erythematosus

2015 ◽  
Author(s):  
Marie Condon ◽  
Philippa Dodd ◽  
Liz Lightstone
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Diagnostic Criteria ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Severe Disease ◽  
Clinical Manifestations ◽  
Central Nervous System Disease ◽  
Systemic Lupus ◽  
Urine Sediment

AbstractSystemic lupus erythematosus (SLE) is a chronic, relapsing, inflammatory, often febrile multisystemic disorder, characterized by involvement of the skin, joints, visceral organs, and serosal membranes. Symptoms and manifestations vary widely over an unpredictable relapsing and remitting course.The presentation of SLE can range from mild forms to severe disease requiring hospitalization. Most commonly it manifests as a combination of constitutional symptoms, particularly fatigue and fever, with cutaneous, musculoskeletal, mild haematological, and serological involvement; however, when renal, haematological or central nervous system disease predominate it can be more severe, even life-threatening. There is a tendency for the disease pattern present at the time of onset to prevail during subsequent exacerbations.Investigating SLE depends to an extent on the presentation of the individual. However a number of haematological, biochemical and immunological investigations provide useful diagnostic information, either for the disease itself or in context of organ system involvement, and should be performed routinely.The presence of lupus nephritis should be considered in any lupus patient with impaired kidney function, proteinuria, hypertension, or an active urine sediment; the gold standard investigation in this context is a kidney biopsy. Glomerular immune complex deposition is the hallmark of lupus nephritis and underpins the International society of Nephrology/Renal Pathology Society classification of lupus nephritis.The diagnosis of SLE is based upon the presence of clinical and/or laboratory features and immunological markers that meet the various published diagnostic criteria. In 2012, lupus nephritis identified on kidney biopsy became an independent diagnostic criterion.This chapter goes through the clinical manifestations, investigations (including a detailed look at the kidney biopsy) and a review of the latest published diagnostic criteria.

Mycophenolate Mofetil and Systemic Lupus Erythematosus: An Overview

Lupus ◽  
2005 ◽  
Vol 14 (3_suppl) ◽  
pp. 9-11 ◽  
Author(s):  
CN Pisoni ◽  
Y Karim ◽  
MJ Cuadrado
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Transplant Rejection ◽  
Immunosuppressive Agent ◽  
Systemic Lupus ◽  
Literature Report ◽  
Proliferative Lupus Nephritis ◽  
Autoimmune Conditions

Mycophenolate mofetil (MMF) is an immunosuppressive agent used in transplantation, with evidence of superior protection against acute transplant rejection compared to azathioprine-containing regimens. Subsequently MMF has been used in a variety of autoimmune conditions. The major experience in systemic lupus erythematosus (SLE) has focused on proliferative lupus nephritis. Following its success in the treatment of lupus nephritis, MMF is now being used to control other SLE manifestations such as, lupus disease activity, haematological manifestations and resistant skin lupus. In this review, we discuss our own experience and the literature report about the use of MMF in SLE.

scholarly journals Primary central nervous system lymphoma in a patient with neuropsychiatric systemic lupus erythematosus receiving mycophenolate mofetil: A case report and literature review

2021 ◽  
Author(s):  
Toru Sakairi ◽  
Masao Nakasatomi ◽  
Mitsuharu Watanabe ◽  
Hiroko Hamatani ◽  
Hidekazu Ikeuchi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Central Nervous System ◽  
Nervous System ◽  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Remission Induction ◽  
High Dose ◽  
Systemic Lupus ◽  
The Brain

ABSTRACT A 41-year-old woman with a 14-month history of systemic lupus erythematosus (SLE) presented with headache, aphasia, and agraphia. A laboratory examination revealed mild proteinuria, hypocomplementemia, and elevated anti-double-stranded DNA antibody levels. A cerebrospinal fluid analysis demonstrated elevated protein and interleukin-6 levels. Magnetic resonance imaging (MRI) of the brain identified multiple lesions suggestive of brain edemas and small haemorrhages. She was diagnosed as having neuropsychiatric lupus and lupus nephritis and received remission induction therapy with high-dose corticosteroid and intravenous cyclophosphamide. She achieved a complete remission, and treatment with mycophenolate mofetil (MMF) was initiated 3 months thereafter for remission maintenance. At 13 months after the exacerbation of SLE, she complained of headache and nausea. A gadolinium-enhanced MRI of the brain revealed a low-signal-intensity tumour with marginal ring enhancement of 50 mm in the left frontal lobe. The tumour was excised, and the histological diagnosis was diffuse large B-cell lymphoma with positive Epstein–Barr virus (EBV). MMF was discontinued. Remission induction therapy with rituximab, high-dose methotrexate, procarbazine, and vincristine was administered, and she achieved remission. Previous reports suggest that use of MMF is associated with primary central nervous system (CNS) lymphoma (PCNSL) in patients with lupus nephritis or other autoimmune diseases or in post-transplant patients. Our observation that PCNSL occurred after CNS involvement of SLE suggests that EBV and CNS inflammation arising from SLE might have contributed to the development of PCNSL.

scholarly journals THU0515 SYSTEMIC LUPUS ERYTHEMATOSUS IN CHILDHOOD AND ADOLESCENCE - UPDATE FROM THE NATIONAL PEDIATRIC RHEUMATOLOGY DATABASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 496.2-496
Author(s):  
C. Sengler ◽  
M. Niewerth ◽  
N. Geisemeyer ◽  
H. Girschick ◽  
A. Klein ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Pediatric Rheumatology ◽  
Kidney Biopsy ◽  
Organ Involvement ◽  
Childhood And Adolescence ◽  
Systemic Lupus ◽  
Kidney Involvement

Background:Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease, which begins in childhood and adolescence in 15 - 20% of cases. Since 2004, data on SLE have been collected by means of a disease-specific questionnaire as part of the National pediatric rheumatology database (NPRD) in Germany. Since 2014, kidney biopsy results have been recorded to further specify kidney involvement.Objectives:Evaluation of clinical signs and symptoms, outcome and laboratory data of patients with juvenile systemic lupus erythematosus from a large database in Germany.Methods:Data from patients with SLE recorded in the NPRD in 2017 were considered for the analysis. In addition to age, sex, onset of disease, the criteria that led to the diagnosis, various laboratory parameters, organ involvement (current, ever) and therapy (current, last 12 months), current disease activity (numerical rating scale 0-10, NRS) and ECLAM (score 0-10) were recorded. Patient-reported outcomes included global assessments of overall-wellbeing and fatigue (NRS 0-10) and functional ability (CHAQ).Results:196 patients (86% female) with a median age of 16 years were documented. Criteria most frequently met at diagnosis included “antinuclear antibodies” (88%), followed by “anti-ds-DNA-Ab” (66%), “butterfly erythema” (42%) and “arthritis” (41%). A positive family history was found in 10% of patients.At documentation, 85% of patients received disease-modifying anti-rheumatic drugs, most frequently hydroxychloroquine (73%), followed by mycophenolate mofetil (32%) and azathioprine (17%). Systemic glucocorticoids obtained 52% of patients, 12% ≥ 0.2 mg/kg/day. Biologics (rituximab 2%) and cyclophosphamide i.v. (3%) were rarely administered during the last 12 months. Disease activity was reported as 1.0 (NRS, median, IQR 0 - 9), ECLAM as 1.0 (median, range 0 - 10). In the laboratory, leukopenia < 3500/µl was found in 9% of patients, lymphopenia < 1500/µl in 47% and erythrocyte sedimentation rate (ESR) > 25 mm in 15% of patients. Mean CHAQ was 0.24, and 86% of patients had a CHAQ score < 0.5. Mean patient`s global assessment of overall-wellbeing was 1.5, while the mean fatigue score was 2.86 (18% NRS score 7-10).The following organ involvement was ever present: general symptoms 84%, skin/mucosa 72%, joints 73%, thyroid 15%, muscle 25%, lungs 17% and CNS 30%. In 45/190 (24%) patients, a kidney involvement was stated. In 34 patients (75%) a kidney biopsy was performed and histology yielded the following results: Class 1: 6.7%, Class 2: 16.7%, Class 3: 40.0%, Class 4: 23.3%, Class 5: 13.3%.Conclusion:The most common clinical symptoms documented in juvenile SLE patients were skin and joint involvement. In the course of the disease, a quarter of the patients developed kidney involvement, mostly proliferative nephritis. Apparently, azathioprine is increasingly being replaced by mycophenolate mofetil, biologicals have hardly been used so far. Although functional outcome and overall-wellbeing of jSLE patients was good, fatigue was a concern for some patients.Disclosure of Interests:Claudia Sengler: None declared, Martina Niewerth: None declared, Nils Geisemeyer: None declared, Hermann Girschick: None declared, Ariane Klein Consultant of: Celgene, Annette Friederike Jansson: None declared, Markus Hufnagel: None declared, Kirsten Minden Consultant of: GlaxoSmithKline, Sanofi, Speakers bureau: Roche

Sign in / Sign up

Export Citation Format

Share Document