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

Author(s):  
T. P. Makarova ◽  
A. V. Sukalo ◽  
I. A. Kazyro ◽  
Yu. S. Melnikova ◽  
N. N. Firsova ◽  
...  

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.

2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095949
Author(s):  
Xincen Hou ◽  
Wenping Pan ◽  
Anli Wang ◽  
Tao Yu ◽  
Aiping Song

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.


2010 ◽  
Vol 38 (3) ◽  
pp. 450-453 ◽  
Author(s):  
TARANEH MEHRANI ◽  
MICHELLE PETRI

Objective.Antibodies to ß2glycoprotein I (IgG and IgM isotypes) have recently been added to the laboratory criteria of the revised antiphospholipid syndrome classification criteria. We investigated whether IgM anti-ß2-glycoprotein I (anti-ß2-GPI) is associated with clinical manifestations of systemic lupus erythematosus (SLE).Methods.Anti-ß2-GPI was measured in 796 patients with SLE (93% women, 53% white, 38% African American, mean age 45 yrs). IgM anti-ß2-GPI (> 20 phospholipid units) was found in 16%. Associations were determined with clinical manifestations of SLE and with components of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index.Results.As expected, IgM anti-ß2-GPI was highly associated with both the lupus anticoagulant and with anticardiolipin. It was associated with transient ischemic attack (OR 2.64, p = 0.04), but not significantly with venous or arterial thrombosis. IgM anti-ß2-GPI was protective against lupus nephritis (OR 0.54, p = 0.049), renal damage (p = 0.019), and hypertension (OR 0.58, p = 0.008). This protective effect remained after adjustment for ethnicity.Conclusion.In SLE, IgM anti-ß2-GPI is not associated with thrombosis but is protective against lupus nephritis and renal damage. “Natural” autoantibodies of the IgM isotype may have a protective effect.


2018 ◽  
pp. 52-58
Author(s):  
Le Thuan Nguyen ◽  
Bui Bao Hoang

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


2011 ◽  
Vol 68 (8) ◽  
pp. 705-708
Author(s):  
Natasa Jovanovic ◽  
Jasmina Markovic-Lipkovski ◽  
Stevan Pavlovic ◽  
Biljana Stojimirovic

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.


2018 ◽  
Vol 29 (1) ◽  
pp. 36-40
Author(s):  
Tasnuva Saiful ◽  
Quadrat E Elahi ◽  
Mamun Mostafi ◽  
Abdul Ali Mia

The diagnosis of systemic lupus erythematosus (SLE) depends on clinical evidence of renal, rheumatologic, cutaneous, and neurologic involvement, supported by serological markers. Ocular involvement is not included in diagnostic criteria of SLE. Vaso-occlusive retinopathy, as the primary manifestation of systemic lupus erythematosus (SLE), is relatively rare. We report a 30-year-old female, who suddenly suffered bilateral visual loss. Fundus examination revealed bilateral occlusive retinal vasculitis. She fulfilled the American College of Rheumatology criteria for diagnosing SLE; she had arthralgia, photosensitivity, positive ANA, high titre of anti-dsDNA, and proteinuria. A renal biopsy showed stage IV lupus nephritis. Treatment was initiated with pulse steroid and immunosuppressant. Best corrected visual acuity of both eye improved to finger counting after treatment. This case demonstrates that in patients with severe vaso-occlusive retinopathy, a generalized immunological disorder, like SLE, should be suspected.Bangladesh J Medicine Jan 2018; 29(1) : 36-40


2018 ◽  
Vol 8 (1) ◽  
Author(s):  
M Enrica ◽  
A Tjandrawati ◽  
S Rachmayati ◽  
Laniyati Hamijoyo

Background: Lupus nephritis is defined as renal involvement in systemic lupus erythematosus (SLE) patients and the most important cause of morbidity and mortality. The diagnostic criteria that used to diagnose lupus nephritis are 1997 American Collegeof Rheumatology is 24 hours urine protein ≥500 mg and/or cellular cast, but significant renal damage can occur without proteinuria or cellular cast. Anti-C1q is an autoantibody that is produced by a chronic alteration of C1q collagen domain. Anti-C1q is a new specific marker for renal marker.Objective: To determine the validity of anti-C1q serum by using 1997 American College of Rheumatology criteria as a gold standard. Methods: This is a cross sectional study, conducted in October to December 2014 at Hasan Sadikin Hospital Bandung. The subjects had systemic lupus erythematosus with and without renal involvement, based on 1997 American College of Rheumatology criteria for SLE.Results: There were 65 subjects included in this study, 64 subjects were female and 1 subject was male. The age average was 32 (SD 11.7) years old. As many as 66.2% subjects had been diagnosed with lupus erythematosus systemic at least 3 years. Twenty four hours urine protein was measured using spectrophotometry, urine sediment was examinedfor cellular cast, and anti-C1q serum was measured using micro enzyme linked immunosorbent assay. Based on American College of Rheumatology criteria, 34 subjects were classified as lupus nephritis group while 31 subjects were classified as non-lupus nephritis group. The area under the curve of anti-C1q was 0.610. The cut-off value used in this study was 10.43 U/ml. The sensitivity, specificity, positive predictive value,negative predictive value and accuracy of anti-C1q assay were 41.18%, 77.42%, 66.67%, 54.55% and 58.46% respectively.Conclusion: Anti-C1q assay, based on this study, hasa low sensitivity and medium specificity to detect lupusnephritis


2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Takashi Nawata ◽  
Makoto Kubo ◽  
Kosaku Shiragami ◽  
Yukinori Nakamura ◽  
Masafumi Yano

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.


Author(s):  
Nadya Rinda Eka Rana ◽  
Awalia Awaliah ◽  
Yetti Hernaningsih ◽  
Hanik Badriyah Hidayati

    NEUROPSYCHIATRIC MANIFESTATION AMONG SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS IN GENERAL HOSPITAL SURABAYAABSTRACTIntroduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with unknown causes and extensive clinical manifestations and diverse disease pathways. The clinical manifestations of SLE are very diverse, including the involvement of the nervous system and psychiatric syndrome (neuropsychiatric).Aims: To describe clinical neuropsychiatric manifestations of   patients with SLE in Soetomo General Hospital Surabaya.Methods: This was a cross-sectional study based on medical record data on all SLE patients treated at Dr. Soetomo Hospital Surabaya, from January-December 2017. Neuropsychiatric manifestations assessment were based on the nomenclature of the American College of Rheumatology (ACR) in 1999.Results: There were 49 patients, mostly women (98%) with mean age 30,8±10,2 years old. Neurological manifestation was the 3rd  most common diagnosis (43%) after hematologic disorder (73.5%) arthritis (53.1%). The manifestations of neuropsychiatric manifestation were mainly seizures (40.8%), headache (34.7%), cerebrovascular disease (26.5%), acute confusional state (20.4%), cognitive dysfunction (6.1%), and polyneuropathy (8.2%).Discussion: The most common neuropsychiatric clinical features in SLE patients are seizures, headache, cerebrovascular disease, and acute confusional state.Keywords: Lupus neuropsychiatry, prevalence, systemic lupus erythematosusABSTRAKPendahuluan: Lupus eritematosus sistemik (LES) merupakan penyakit inflamasi autoimun kronik yang belum diketahui penyebabnya dengan perjalanan penyakit yang luas. Manifestasi klinis LES sangat beragam, antara lain keterlibatan sistem saraf dan sindrom psikiatri (neuropsikiatri).Tujuan: Untuk mengetahui manifestasi neuropsikiatrik pasien dengan LES di RSUD Dr. Soetomo, Surabaya.Metodologi: Penelitian potong lintang berdasarkan data rekam medik terhadap semua pasien LES yang dirawat di RSUD Dr. Soetomo, Surabaya, pada bulan Januari hingga Desember 2017. Manifestasi neuropsikiatrik dinilain berdasarkan nomenklatur American College of Rheumatology (ACR) tahun 1999.Hasil: Didapatkan 49 subjek yang hampir semuanya (98%) adalah perempuan dengan rerata usia 30,8±10,2 tahun. Gangguan neurologis merupakan ketiga tersering (43%) setelah gangguan hematologi (73,5%) dan artritis (53,1%). Manifestasi neuropsikiatri terutama kejang (40,8%), nyeri kepala (34,7%), penyakit serebrovaskular (26,5%), keadaan konfusi akut (20,4%), dan polineuropati (8,2%).Diskusi: Manifestasi klinis neuropsikiatri yang paling banyak dialami oleh pasien LES adalah kejang, nyeri kepala, penyakit serebrovaskular, dan keadaan konfusi akut.Kata kunci: Lupus eritematosus sistemik, manifestasi klinis, neuropsikiatri  


2021 ◽  
Vol 8 ◽  
Author(s):  
Yi Liu ◽  
Xuelei Liang ◽  
Haixuan Wu ◽  
Fenglin Zhuo

Introduction: Lichen planus/lupus erythematosus overlap syndrome is rarely seen in the clinic and has the characteristic clinical manifestations, histopathology, and immunopathology of lichen planus (LP) and lupus erythematosus (LE). This is the first reported case of bullous lichen planus (BLP)/systemic lupus erythematosus (SLE) overlap syndrome with hair loss as the first symptom.Case Presentation: A 48-year-old female presented with alopecia for half a year, and skin lesions accompanied by itching on her face, trunk, and limbs for 3 months. She had a history suggestive of photosensitivity. Laboratory tests and histopathology were performed for diagnosis. Histopathologic features of the upper arm and back of the hand were consistent with BLP, whereas the scalp lesion indicated LE. Laboratory examination indicated positive for antinuclear antibody (ANA) (1:160), leukopenia, increased urinary protein, decreased C3/C4, and normal BP180. The patient was given glucocorticoid combined with acitretin and immunosuppressive therapy after a definite diagnosis of BLP/SLE overlap syndrome. The lesions of the patient disappeared and some hair had regrown during the two years of follow-up.Conclusion: This is the first reported case of BLP/SLE overlap syndrome which responded well to glucocorticoids, retinoids, and immunosuppressive drugs. Multiple biopsies from characteristic lesions will guide doctors to avoid misdiagnoses and delayed treatment.


2019 ◽  
Vol 16 (6) ◽  
pp. 29-35
Author(s):  
Alina Dima ◽  
Bianca Dumitrescu ◽  
Daniela Nicoleta Popescu ◽  
Magda Pârvu

AbstractSystemic lupus erythematosus (SLE) is considered the prototype of autoimmune diseases, the most complex autoimmune pathology and it is characterized by a wide range of immune processes, important antibodies production as well as an impressive spectrum of clinical manifestations. The great variety of lupus signs and symptoms caused difficulties in establishing well-defined classification criteria, as well as sustaining the clinical diagnosis.In 2019, a joint initiative of European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) released a new set of classification criteria for SLE, worldwide SLE experts were involved, this being the largest SLE classification effort up to date.


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