scholarly journals Eculizumab use in a tertiary care nephrology center: data from the Vienna TMA cohort

2021 ◽  
Author(s):  
Christof Aigner ◽  
Martina Gaggl ◽  
Gunar Stemer ◽  
Michael Eder ◽  
Georg Böhmig ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Hospital Pharmacy ◽  
Practice Patterns ◽  
Tertiary Care ◽  
Duration Of Treatment ◽  
Plasma Therapy ◽  
University Of Vienna ◽  
Complement Gene ◽  
Predominantly Female ◽  
Medical University

Abstract Background Practice patterns of eculizumab use are not well described. We examined indications for, and outcomes of, eculizumab therapy in a tertiary care nephrology center. Methods We used the “Vienna TMA cohort” and the hospital pharmacy database at the Medical University of Vienna to identify patients that received eculizumab treatment between 2012 and 2019. We describe clinical characteristics, details of eculizumab use, and outcomes of patients with complement gene-variant mediated TMA (cTMA), secondary TMA (sTMA) and C3 glomerulopathy (C3G). Results As of December 2019, 23 patients received complement blockade at the Division of Nephrology and Dialysis: 15 patients were diagnosed with cTMA, 6 patients with sTMA and 2 patients with C3G. Causes of sTMA were bone marrow transplantation (n = 2), malignant hypertension, malignant tumor, systemic lupus erythematosus, antiphospholipid syndrome and lung transplantation (each n = 1). Across all indications, patients had a median age of 31 and were predominantly female (78%) and the median duration of treatment was 227 days. Hematological recovery was seen in most patients, while renal response was best in patients with cTMA. Adverse events were recorded in 26%. Conclusions In summary, eculizumab is the treatment of choice for cTMA patients that do not respond to plasma therapy. In patients with sTMA and C3G, the response rates to therapy are much lower and therefore, the decision to start therapy needs to be considered carefully. Graphic abstract

scholarly journals P0179ECULIZUMAB USE IN A TERTIARY CARE NEPHROLOGY CENTER

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Christof Aigner ◽  
Gunar Stemer ◽  
Martina Gaggl ◽  
Natalja Haninger-Vacariu ◽  
Zoltán Prohaszka ◽  
...  
Keyword(s):  
Chronic Kidney Disease Stage ◽  
Tertiary Care ◽  
Disease Stage ◽  
Gene Variant ◽  
University Of Vienna ◽  
Ckd Stage ◽  
Life Threatening ◽  
History Of ◽  
Complement Gene ◽  
Medical University

Abstract Background and Aims Practice patterns of Eculizumab use in patients with thrombotic microangiopathies (TMA) and C3-glomerulopathy (C3G) are not well described. Method We used the “Vienna TMA cohort” and the hospital pharmacy database at the Medical University of Vienna to identify adult patients with a history of eculizumab use between 2012 and 2019. We describe clinical characteristics, details of eculizumab use, and outcomes of patients with complement gene variant mediated TMA (cTMA), secondary TMA (sTMA) and C3G. Results As of December 2019, 212 individuals were enrolled in the Vienna TMA cohort comprising 51 cTMA, 144 sTMA, and 17 TTP patients. We included also our cohort of 14 patients with C3G for this analysis. 47 patients (22 TMA and 2 C3G, 23 other indications, i.e. paroxysmal nocturnal haemoglobinuria) received at least one dose of eculizumab at the Medical University of Vienna (Figure 1). Table 1 indicates demographic and clinical details of 15 cTMA (29.4% of all cTMA), 7 sTMA (4.9% of all sTMA) and 2 C3G (14.3% of all C3G) patients treated with eculizumab. 60% of cTMA patients showed a rare complement gene variant, while sTMA was ruled out in the remaining 40%. Causes of sTMA were bone marrow transplantation (BMT) (n=2), malignant hypertension, malignoma, systemic lupus erythematodes, antiphospholipid syndrome and lung transplantation (each n=1). One sTMA patient, a BMT recipient, had a donor with a thrombomodulin gene variant. Patients with cTMA had a greater delay from first diagnosis to treatment with eculizumab than the other groups and received maintenance therapy for a longer period of time. More female patients received eculizumab as compared to male patients. Chronic kidney disease stage improved in 60% and 43% of cTMA and sTMA patients, respectively. TMA relapses did not occur during administration of eculizumab. The 2 patients with C3G didn’t respond to eculizumab in our center. Eculizumab therapy was stopped in 66% of patients with cTMA and in all patients presenting with sTMA or C3G. In general, eculizumab was well tolerated and we did not observe life threatening infections of our patients. Three adverse drug reactions included exanthema, liver injury, and hypertensive emergency. Two patients died during therapy with eculizumab (1 cTMA, 1 C3G,) and two after cessation of eculizumab therapy (1 cTMA, 1 sTMA) resulting in a mortality of 16.7%. Conclusion Improvement of CKD stage was achieved in 60% of patients with cTMA and in 43% of patients with sTMA. In our patients with C3G, eculizumab did not improve kidney function. In general, therapy with eculizumab was well tolerated.

Prescribing trends of antidiabetic drugs in diabetic and hypertensive diabetic patients in an urban tertiary care hospital of Bangladesh

2017 ◽  
Vol 3 (3) ◽  
pp. 338-343
Author(s):  
Mohammad Didar Khan ◽  
Md. Ibrahim ◽  
Md. Mizanur Rahman Moghal ◽  
Dipti debnath ◽  
Asma Kabir ◽  
...  
Keyword(s):  
Urban Areas ◽  
Tertiary Care ◽  
Diabetic Patients ◽  
Care Hospital ◽  
Age Group ◽  
Microsoft Excel ◽  
Patient Profile ◽  
Prescribing Trends ◽  
Medical University ◽  
Insight Into

Objective: The present epidemiological study was conducted with the objectives of providing an insight into the current use of antidiabetic medications to diabetics and hypertensive diabetics in urban areas and determining how the patient factors influence the prescribing of antidiabetic medications. Methodology: Data of patients of past two years were collected from Bangabandhu Sheikh Mujib Medical University (BSMMU) Hospital, Dhaka, Bangladesh. The details were entered in the structured patient profile form. Data were statistically analyzed using the Microsoft Excel 2007 software. Result: A total of 958 patient’s data were collected and analyzed of which 632 (65.97 %) were males and 326 (34.03 %) were females. These patients were further categorized based on their age. 330 patients (34.45 %) belonged to the age group 20 – 44 years, 504 (52.61 %) to the age group 45 – 65 years and 124 (12.94 %) to the age group 65 – 80 years. 684 (71.4%) patients out of the 958 patients studied were suffering from coexisting hypertension. Co-existing hypertension was found to be more prevalent in the age group 45 – 65 years (67.69%) and was found more in females (84.04%). Conclusion: Metformin was the oral hypoglycemic which was the highest prescribed. In hypertensive diabetics Metformin and Pioglitazone were most frequently prescribed drugs. Biguanides and Insulin were the most commonly prescribed antidiabetics. A combination of two or more drugs of different classes was prescribed to hypertensive diabetics. It is necessary to have an improved understanding of the etiology and pathophysiology of diabetes to focus on research efforts appropriately.

Assessment of Quality of Life (QoL) in Systemic Lupus Erythematosus Patients at a Tertiary Care Hospital in Egypt

2019 ◽  
Vol 15 (4) ◽  
pp. 304-311
Author(s):  
Mervat E. Behiry ◽  
Sahar A. Ahmed ◽  
Eman H. Elsebaie
Keyword(s):  
Quality Of Life ◽  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Tertiary Care ◽  
Damage Index ◽  
Life Questionnaire ◽  
Care Hospital ◽  
Systemic Lupus

: Systemic Lupus Erythematosus (SLE) has a profound impact on quality of life. Objective: The objective of this study was to explore the quality of life among Egyptian SLE patients and to assess its relationships with demographic and clinical features. Methods: One hundred sixty-four SLE patients were recruited for this study. Demographic information; clinical parameters; disease activity, as evaluated by the systemic lupus erythematosus Disease Activity Index; and organ damage, as assessed by the systemic lupus international Collaborative Clinics/American College of Rheumatology Damage Index, were reported. Quality of life was assessed with a quality of life questionnaire specifically designed for patients with systemic lupus erythematosus; the questions are grouped in the following six domains: physical function, sociooccupational activities, symptoms, treatment, mood, and self-image. Higher values indicate poorer quality of life. Conclusion: Poor quality of life among Egyptian SLE patients and disease activity are strongly related to impaired lifestyles in these patients.

scholarly journals Pattern and prevalence of neuropsychiatric lupus: a retrospective study from a tertiary level hospital in Bangladesh

2021 ◽  
Vol 57 (1) ◽  
Author(s):  
Fahima Hossain ◽  
Mohammad Delwer Hossain Hawlader ◽  
Dipak Kumar Mitra ◽  
Mohammad Hayatun Nabi ◽  
Md. Mujibur Rahman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Retrospective Study ◽  
Cognitive Dysfunction ◽  
Lupus Erythematosus ◽  
Neuropsychiatric Symptoms ◽  
Tertiary Care ◽  
Care Hospital ◽  
Systemic Lupus ◽  
Case Definitions ◽  
American College Of Rheumatology

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.

scholarly journals Echocardiographic Prevalence and Risk Predictors of Ventricular Dysfunction in Connective Tissue Disorders: Tertiary Care Hospital-Based Prospective Case-Control Study

2020 ◽  
Vol 1 (3-4) ◽  
pp. 132-141
Author(s):  
Saru Thakur ◽  
Geeta Ram Tegta ◽  
Prakash Chand Negi ◽  
Kunal Mahajan ◽  
Ghanshyam Verma ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Systolic Dysfunction ◽  
Tertiary Care ◽  
Left Ventricular ◽  
Care Hospital ◽  
Connective Tissue Disorders ◽  
Cardiac Symptom ◽  
Recorded Data

Background: There is a paucity of contemporary Indian data about the prevalence of cardiac abnormalities in patients of connective tissue disorders (CTD) and their risk determinants. Methods: We prospectively recorded data from 35 consecutive CTD patients who presented to our out-patient department and had no significant cardiovascular risk factors at baseline. We also recorded data from their age- and sex-matched controls. All cases and controls were subjected to 12 lead electrocardiogram and echocardiography after routine investigations. Results: The CTD group comprised 19 (54.3%) patients of systemic lupus erythematosus, 12 (34.3%) patients of systemic sclerosis, 2 (5.7%) patients of mixed CTD, and 1 (2.9%) patient each of overlap syndrome and dermatomyositis. Cardiovascular involvement on echocardiography was documented in 71.4% of CTD patients despite majority of them having no cardiac symptom. Overt left ventricular (LV) systolic dysfunction was observed in 3 (8.6%) CTD patients, while subclinical LV systolic dysfunction was recorded in 13 (37.1%) patients. LV diastolic dysfunction was observed in 11.4% (n = 4) patients. RV systolic dysfunction was prevalent in 20% (n = 7) patients. Pulmonary hypertension was observed in 40% (n = 14) of CTD patients. Conclusion: The present study evaluated subclinical LV systolic dysfunction and pulmonary hypertension in about one third of CTD patients. It is imperative to screen for these abnormalities in CTD to ensure timely diagnosis and treatment.

Characteristics of lupus and lupus nephritis at a tertiary care center in Lebanon

Lupus ◽  
2019 ◽  
Vol 28 (13) ◽  
pp. 1598-1603 ◽  
Author(s):  
S H Koubar ◽  
J Kort ◽  
S Kawtharani ◽  
M Chaaya ◽  
M Makki ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Kidney Disease ◽  
Complete Remission ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Partial Remission ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Systemic Lupus

Introduction Systemic lupus erythematosus affects 4.8–78.5 people per 100,000 worldwide, 90% of whom are females. Geography and ethnicity have been shown to significantly affect the prevalence and natural history of the disease. Lupus nephritis affects around half of patients with systemic lupus erythematosus. Data about systemic lupus erythematosus and lupus nephritis in the Middle East are still scarce. In this study, we aimed to describe the characteristics of systemic lupus erythematosus and lupus nephritis at a tertiary care center in Lebanon. Methods This is a retrospective chart review of all biopsy-proven lupus nephritis patients admitted to the American University of Beirut medical center between January 2000 and December 2018. Patients above 12 years of age who had any International Society of Nephrology/Renal Pathology Society (ISN/RPS) class of lupus nephritis on their renal biopsy were included in the study. Results The study included 55 patients with lupus nephritis. Upon presentation of systemic lupus erythematosus, the most common clinical feature was arthritis, seen in 83% of patients, followed by anemia (82%) and malar rash (48%). In total 93% had positive ANA, 89% had positive anti-dsDNA and 98% of patients had proteinuria. The most common ISN/RPS class of lupus nephritis in our series was IV (49%). At the time of the biopsy 15% of patients underwent dialysis. At 6 months, 11/27 had complete remission, 6/27 had partial remission and 10/27 had no remission. At 1 year, 8/23 had complete remission, 4/23 had partial remission and 11/23 had no remission. During the study period, 15 out of 35 patients available for analysis had chronic kidney disease (CKD) and six out of 34 patients developed end-stage kidney disease requiring renal replacement therapy. In comparison to other series in the region, our series had more males affected, higher creatinine at the time of biopsy and greater degree of proteinuria. Conclusion Our study provided insight on the demographics, characteristics, and outcomes of lupus nephritis in Lebanon. Interestingly, male gender was present in a quarter of patients. This warrants further investigation and confirmation. We are hoping to expand this experience into a national prospective registry to further characterize this entity in our region.

Tumid lupus erythematosus and systemic lupus erythematosus: a rare coexistence

2021 ◽  
Vol 14 (10) ◽  
pp. e245196
Author(s):  
Ailia Ali ◽  
Alexander Woods ◽  
Caroline Porter ◽  
Steven R Feldman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Tertiary Care ◽  
Medical Centre ◽  
Shortness Of Breath ◽  
Systemic Lupus

A patient diagnosed with tumid lupus erythematosus (TLE) was subsequently found to have systemic lupus erythematosus (SLE) after presenting to a tertiary care medical centre with shortness of breath and oedema. In this commentary, we discuss this patient’s presentation and the association between TLE and SLE.

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