scholarly journals Vaso-occlusive retinopathy and stage IV Lupus Nephritis; Primary manifestation of a 30 year old female

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


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.


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.


Lupus ◽  
2019 ◽  
Vol 28 (4) ◽  
pp. 492-500 ◽  
Author(s):  
B Costa Rodrigues ◽  
M Ignacchiti Lacerda ◽  
G R Ramires de Jesús ◽  
Flávia Cunha dos Santos ◽  
N Ramires de Jesús ◽  
...  

Objective To analyze the impact of different classes of lupus nephritis as risk variables for maternal and fetal adverse outcomes in a cohort of pregnant lupus patients. Methods This is a cohort study with retrospective and prospective data collection, conducted at the University Hospital of State University of Rio de Janeiro, Brazil, from 2011 to 2016. A total of 147 pregnancies of 137 systemic lupus erythematosus patients of whom 66 had lupus nephritis were included. Demographic and clinical features, as well as maternal and fetal outcomes were observed for each nephritis histological class among systemic lupus erythematosus patients and compared with those without nephritis. Categorical variables were expressed as absolute and relative frequencies and numerical variables as means and standard deviation. The chi-square test with Fisher's correction and Student's t-test were used for statistical analysis. A pvalue < 0.05 was considered statistically significant. Results Systemic lupus erythematosus patients with proliferative nephritis (classes III/IV, n = 54) presented more frequent disease flares ( p = 0.02), continuous active disease during pregnancy and puerperium ( p = 0.006), hospitalization due to systemic lupus erythematosus ( p < 0.001), hospitalization not directly associated to systemic lupus erythematosus ( p = 0.04), higher frequency of cesarean delivery ( p = 0.03) and preeclampsia ( p = 0.01) than patients without nephritis. Permanent damage measured by Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was more frequent in classes III/IV than among the other patients. The frequency of adverse fetal outcomes such as prematurity and admission to neonatal intensive care unit were not different among systemic lupus erythematosus patients with or without nephritis. However, perinatal deaths were more frequent in patients with all classes of nephritis ( p = 0.003). Conclusion Systemic lupus erythematosus patients with proliferative nephritis (classes III/IV) have a higher frequency of adverse maternal outcomes. This is probably due to the major impact of proliferative forms of nephritis on women's global heath, which is corroborated by the higher Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index findings, although we cannot exclude the negative influence of disease activity for the maternal adverse events. The findings indicate a need for further lupus nephritis classification beyond the nonspecific term nephritis in the context of lupus pregnancy as the impact on maternal and fetal outcomes varies according to histological class.


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


2020 ◽  
Vol 8 (1) ◽  
Author(s):  
Jorge Ivan Gamez-Nava ◽  
Valeria Diaz-Rizo ◽  
Edsaul Emilio Perez-Guerrero ◽  
Jose Francisco Muñoz-Valle ◽  
Ana Miriam Saldaña-Cruz ◽  
...  

Abstract Background To date, the association of serum macrophage migration inhibitory factor (MIF) and serum adipokines with lupus nephritis is controversial. Objective To assess the utility of serum MIF, leptin, adiponectin and resistin levels as markers of proteinuria and renal dysfunction in lupus nephritis. Methods Cross-sectional study including 196 systemic lupus erythematosus (SLE) patients and 52 healthy controls (HCs). Disease activity was assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Renal SLE involvement was investigated by renal-SLEDAI. MIF, adiponectin, leptin and resistin levels were quantified by ELISA. We assessed the correlations of quantitative variables by Spearman correlation (rs). Multivariable linear regression adjusted the variables associated with the severity of proteinuria. Results SLE patients had higher MIF (p = 0.02) and adiponectin (p < 0.001) than HCs. Patients with renal SLE involvement (n = 43) had higher adiponectin (19.0 vs 13.3 μg/mL, p = 0.002) and resistin (10.7 vs 8.9 ng/mL, p = 0.01) than patients with non-renal SLE (n = 153). Proteinuria correlated with high adiponectin (rs = 0.19, p < 0.009) and resistin (rs = 0.26, p < 0.001). MIF (rs = 0.27, p = 0.04). Resistin correlated with increased creatinine (rs = 0.18, p = 0.02). High renal-SLEDAI correlated with adiponectin (rs = 0.21, p = 0.004). Multiple linear regression showed that elevated adiponectin (p = 0.02), younger age (p = 0.04) and low MIF (p = 0.02) were associated with the severity of proteinuria. Low MIF and high adiponectin levels interacted to explain the association with the severity of proteinuria (R2 = 0.41). Conclusions High adiponectin combined with low MIF concentrations int+eract to explain the severity of proteinuria in renal SLE. These findings highlight the relevance of adiponectin, resistin and MIF as markers of LN.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1048.1-1048
Author(s):  
W. Hu

Background:Classical lupus nephritis (LN) is characterized by glomerular immune complex(IC) deposition with glomerular proliferation, basement membrane destruction and cell infiltration. Non-IC mediated renal injury with thrombotic microangiopathy (TMA) was also reported in patients with systemic lupus erythematosus (SLE-renal TMA), but most studies were reported in patients with both LN and renal TMA.Objectives:In this study, clinical features and outcomes of SLE-renal TMA in absence of obvious IC in SLE patients were analyzed.Methods:Patients with glomerular TMA and/or vascular TMA in the absence of obvious subendothelial or epithelial immune deposits were screened out from 2332 biopsied in SLE patients who underwent first renal biopsy from January 2005 to August 2016. Their clinical, histological features and outcomes were retrospectively analyzed.Results:In 2332 renal biopsies obtained from SLE patients, 257 (11.0%) showed renal TMA, of which 237 showed both renal TMA and LN, and 20 biopsies had only renal TMA (SLE-renal TMA). There were 2 males and 18 females with an average age of (25 ± 10) years. The median course of SLE and LN were 3.0(1.0, 6.0) and 0.8(0.5, 1.9) months. All 20 patients deserved acute kidney injury, of which 11 (55%) needed renal replacement therapy (RRT) and 12 (60%) were nephrotic syndrome. Blood system involvement was found in all cases, including 13 cases (65.0%) with TMA triad (microvascular hemolytic anemia, thrombocytopenia and elevated lactate dehydrogenase).Pathological examination showed that 17 cases (85.0%) had both glomerular TMA and vascular TMA. Immunofluorescence and electron microscopy showed that 8 cases (40%) had no IC deposition in glomerulus and 12 cases (60%) had only IC deposition in mesangium. Acute tubulointerstitial lesions in patients requiring RRT were more serious than those no needing for RRT((43.6±24.9) %vs(21.7±20.1) %,P=0.047). The fusion range of foot process was positively correlated with proteinuria (r2= 0.347,P=0.006).All patients received high-dose methylprednisolone pulse therapy. Four patients received plasma exchange and three patients received gamma globulin, respectively. Eleven patients requiring RRT all stop RRT in a median time of 16.0 (9.0, 30.0) days. During a median follow-up of 58.0 (36.0, 92.3) months, complete remission (CR) was obtained in 15 cases, partial remission in 4 cases and no remission in 1 case. Six cases (30%) relapsed. No case died or progressed to end stage renal disease.Conclusion:Renal injury characterized by TMA is not uncommon in SLE renal biopsy cases. The clinical manifestation is special and the renal injury is serious. The renal outcome is good by intensive immunosuppressive therapy. It should be considered as a unique type of renal injury in SLE.References:[1]Moake JL. Thrombotic microangiopathies. N Engl J Med. 2002. 347(8): 589-600.[2]Anders HJ, Weening JJ. Kidney disease in lupus is not always ‘lupus nephritis’. Arthritis Res Ther. 2013. 15(2): 108.[3]Song D, Wu LH, Wang FM, et al. The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Res Ther. 2013. 15(1): R12.[4]Hu WX, Liu ZZ, Chen HP, Zhang HT, Li LS, Liu ZH. Clinical characteristics and prognosis of diffuse proliferative lupus nephritis with thrombotic microangiopathy. Lupus. 2010. 19(14): 1591-8.[5]Tomov S, Lazarchick J, Self SE, Bruner ET, Budisavljevic MN. Kidney-limited thrombotic microangiopathy in patients with SLE treated with romiplostim. Lupus. 2013. 22(5): 504-9.[6]Li C, Yap D, Chan G, et al. Clinical Outcomes and Clinico-pathological Correlations in Lupus Nephritis with Kidney Biopsy Showing Thrombotic Microangiopathy. J Rheumatol. 2019 .[7]Chen MH, Chen MH, Chen WS, et al. Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan. Rheumatology (Oxford). 2011. 50(4): 768-75.[8]Park MH, AUID- Oho, Caselman N, Ulmer S, Weitz IC, AUID- Oho. Complement-mediated thrombotic microangiopathy associated with lupus nephritis. Blood Adv. 2018. 2(16): 2090-2094.Disclosure of Interests:None declared


Author(s):  
Fahima Hossain ◽  
Mohammad Delwer Hossain Hawlader ◽  
Dipak Kumar Mitra ◽  
Mohammad Hayatun Nabi ◽  
Md. Mujibur Rahman

Abstract Background Neuropsychiatric systemic lupus erythematosus (NPSLE) is well known for its varying presentations and poor outcomes, but little is evident about its distribution and characteristics among the Bangladeshi population. This study aimed to assess the pattern and prevalence of neuropsychiatric symptoms in female systemic lupus erythematosus (SLE) patients of Bangladesh. A retrospective study was conducted at a tertiary care hospital in Dhaka, Bangladesh, between January and December 2018. One hundred female SLE patients were included in the study purposively. Data were collected on sociodemographic and clinical characteristics of diagnosed SLE cases visiting the SLE clinic and indoor medicine department. Neuropsychiatric (NP) syndromes were defined according to the widely accepted American College of Rheumatology (ACR) nomenclature and case definitions. Results A total of 244 NP events were identified in fifty-five patients. Headache was the most frequent symptom (55%), followed by cognitive dysfunction (50%), anxiety (49%), psychosis (43%), seizure (23%), depression (17%), and cerebrovascular disease (ischemic type, 7%). The NP manifestations were more prevalent among urban residents (58.2%), younger patients (41.8%), and patients with graduate-level education (34.5%). Besides, young age at diagnosis (p = 0.038), Raynaud’s phenomenon (p = 0.015), other organ involvement (p < 0.001), and time of NPSLE development (p < 0.001) were found to be significantly associated with the development of these manifestations. Conclusion NP damage is prevalent among Bangladeshi female SLE patients (55%) with headache and cognitive dysfunction being the most common symptoms. Routine screening for neuropsychiatric symptoms among suspected SLE cases and further evaluation with a larger population are warranted.


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