Damage index in childhood-onset systemic lupus erythematosus in Egypt

Salah, Samia; Lotfy, Hayam M.; Mokbel, Abir; Kaddah, A. M.; Fahmy, Nouran

doi:10.1186/1546-0096-9-36

Cited by 13 publications

(10 citation statements)

References 18 publications

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“…As in this study, the presence of neuropsychiatric manifestations as a risk factor for damage was observed in two previous studies by Ravelli et al and Salah et al [ 10 , 11 ]. The American College of Rheumatology has developed a standardized nomenclature and case definitions of the 19 neuropsychiatric syndromes of SLE in 1999 to aid clinical research in multi-centre settings [ 22 ].…”

Section: Discussionsupporting

confidence: 82%

“…The percentage of patients with disease damage in this group was 33.9%. It is again lower than most previous reported series where the percentages ranged from 43.9 to 64.5% [ 4 , 6 – 9 , 11 , 12 , 24 ]. This may be consistent with some of the previous observations where Asian adult lupus patients have lower rates of damage accrual when compared to other ethnicities [ 15 , 33 ].…”

Section: Discussioncontrasting

confidence: 74%

“…Cumulative disease activity over time and the use of certain drugs, notably high-dose steroids, greater number of cyclophosphamide pulses or ever use of cyclophosphamide, have been implicated as important risk factors of disease damage [ 7 , 9 , 10 ]. Furthermore, frequent severe disease flares and longer disease duration are also associated with presence of damage [ 6 , 7 , 9 – 11 ].…”

Section: Discussionmentioning

confidence: 99%

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Risk factors for damage in childhood-onset systemic lupus erythematosus in Asians: a case control study

Sit

Chan

2018

Pediatr Rheumatol

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BackgroundAccumulated damage is an important prognostic factor in systemic lupus erythematous. However, the pattern of disease damage and its risk factors have not been well studied in childhood-onset systemic lupus erythematosus (cSLE) in Asia. The objectives are to evaluate the pattern of damage and to identify the risk factors for accumulated damage in an Asian group of cSLE.MethodsA retrospective chart review was conducted on a group of 59 patients with cSLE. Patient demographics and clinical variables were first collected at diagnosis. Over the course of their disease, clinical variables considered as risk factors for damage were also collected. Damage was measured using the Systemic Lupus International Collaborating Clinics/ American College of Rheumatology Damage Index (SDI) for each patient at their last encounter. Based on their SDI scores, patients were then dichotomized to two groups: a group with presence of disease damage (SDI ≥1) and a group with absence of disease damage (SDI score = 0). Clinical variables including age at diagnosis, gender, ethnicity, disease duration, disease manifestations, laboratory values at diagnosis, disease activity at diagnosis and last encounter, major organ involvement, number of lupus flares, major infection, and intensity of immunosuppressive medications were compared between the two groups. Growth failure and estimated glomerular filtration rate (eGFR) were also analysed as secondary outcomes.ResultsAfter a median disease duration and follow up of 7.8 years, 39 patients (66.1%) had no disease damage while 20 patients (33.9%) had acquired disease damage. Disease damage most frequently occurred in the ocular (15.3%), neuropsychiatric (11.9%) and musculoskeletal (11.9%) domains. The most frequent forms of damage were cataracts (11.9%), and avascular necrosis (unilateral and bilateral combined 10.2%). After controlling for other variables, presence of neuropsychiatric manifestations remained the only statistically significant risk factor for damage. The rate of growth failure in our group of patients was 16%. Patients who experienced growth failure were significantly younger at disease diagnosis. The median age of diagnosis was 10 for those who experienced growth failure, whereas the median age of diagnosis was 13 for those who did not experience growth failure. Despite a high rate of renal involvement in the group (79.7%), renal damage was only seen in 3.2% of the patients. 91.5% of the studied group had normal eGFR of ≥90 ml/min/1.73m2 at their last follow up.ConclusionThis group of patients had a low rate of damage accrual, with one of the lowest rates in renal damage when compared to other cohorts reported. The presence of neuropsychiatric manifestations was identified as the most significant risk factor for disease damage, while the most frequent forms of damage were cataracts and avascular necrosis, which were both related to prolonged steroid use. Despite the limitations of this study, it highlights the need for larger prospective studies to understand the relatio...

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Section: Discussionsupporting

confidence: 82%

Section: Discussioncontrasting

confidence: 74%

Section: Discussionmentioning

confidence: 99%

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Risk factors for damage in childhood-onset systemic lupus erythematosus in Asians: a case control study

Sit

Chan

2018

Pediatr Rheumatol

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“…Age 18 years is the threshold age at diagnosis with SLE that is most frequently used to define SLE with onset during childhood or adolescence in population‐based research around the world (2, 4, 22–49). Nevertheless, age cutoffs used in studies of SLE range from 14–21 years (50–67). This is in line with some studies comparing pediatric versus adult populations with other chronic diseases, including asthma (68) and type 2 diabetes mellitus (69), which consider threshold ages of up to 20 years at diagnosis to define the pediatric subgroup.…”

Section: When Does Adulthood Start?mentioning

confidence: 99%

Taxonomy for systemic lupus erythematosus with onset before adulthood

Silva¹,

Avčin²,

Brunner³

2012

Arthritis Care & Research

153

115

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Objective To propose a common nomenclature to refer to individuals who fulfill the American College of Rheumatology Classification Criteria for systemic lupus erythematosus (SLE) during childhood or adolescence. Methods The medical literature was reviewed for studies conducted in the target population between 1960 and December of 2011 to obtain information about the terms used to refer to such children and adolescents. We reviewed the threshold ages used and disease features considered to discriminate these individuals from patients with onset of SLE during adulthood. Furthermore, the nomenclature used in other chronic diseases with onset during both childhood and adulthood was assessed. Results There was an astonishing variability in the age cut-offs used to define SLE-onset prior to adulthood, ranging from 14 to 21 years but most studies used 18 years of age. The principal synonyms in the medical literature were SLE without reference to the age at onset of disease, childhood-onset SLE, juvenile SLE, and pediatric (or paediatric) SLE. Conclusions Based on the definition of childhood, in analogy with other complex chronic disease commencing prior to adulthood, and given the current absence of definite genetic variations that discriminate adults from children, the term childhood-onset SLE is proposed when referring to individuals with onset of SLE prior to age 18 years.

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“…Avascular necrosis is a debilitating disorder that characterized by interruption of the blood supply which leads to death of the bone marrow and trabecular bone, presenting with joint pain, bone destruction, and loss of function 8 . It is a well-known complication of SLE since its first determination in 1960 and has been reported in 1.4-40% of jSLE patients [1][2][3][4][9][10][11][12][13][14] . The most common affected sites are femoral head, femoral condyles, proximal tibia, and the bones of foot and ankle 8 .…”

Section: Introductionmentioning

confidence: 99%

Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

Balcı

Ekinci

Pişkin

et al. 2020

Cukurova Medical Journal

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Öz Purpose: Avascular necrosis (AVN) is a debilitating complication of juvenile systemic lupus erythematosus (jSLE). The aim of this study was to evaluate the frequency and clinical characteristics of patients with AVN in jSLE from a single center. Material and Methods: Fifty-eight jSLE patients diagnosed according to the American College of Rheumatology classification criteria were included in this retrospective study. Disease activity of jSLE patients was measured by SLE Disease Activity Index-2K (SLEDAI-2K), organ damage was determined by the pediatric version of the systemic lupus international collaborating clinics/American College of Rheumatology damage index (pedSDI) at last visit. Results: Among 58 jSLE patients, the female patients accounted for 86.2% (n=50). Mean baseline SLEDAI-2K score was 21.47±8.96. The number of patients with at least one damage item of pedSDI was 24 (41.4%), in which 5 patients (8.6%) had AVN. There were no statistical differences between the groups except baseline complement 3 (C3) level was significantly lower in patients with AVN. Conclusion: Baseline low C3 level might be a predictor for AVN development in jSLE patients.There is unmet need for multicenter studies investigating possible risk factors of AVN in jSLE patients. Amaç: Avasküler nekrozis (AVN), çocukluk çağı başlangıçlı sistemik lupus eritematozusun (jSLE) yıkıcı bir komplikasyonudur. Tek merkezden yapılan bu çalışmanın amacı jSLE hastalarında AVN sıklığını ve bu hastaların klinik özelliklerini belirlemektir. Gereç ve Yöntem: Amerikan Romatoloji Koleji'nin sınıflama kriterlerine göre tanı almış 58 jSLE hastası bu geriye dönük çalışmaya dahil edildi. jSLE hastalarının hastalık aktiviteleri SLE hastalık aktivite indeksi baz alınarak (SLEDAI-2K) ölçüldü. Organ hasarı Amerikan Romatoloji Koleji'nin SLE hastalarında uyguladığı hasar indeksinin çocukluklardaki versiyonu kullanılarak son muayenede belirlendi. Bulgular: Elli sekiz jSLE hastasının %86,2 (50)'si kız hastaydı. Ortalama bazal SLEDAI-2K skoru 21,47±8,96 idi. En az bir organda hasarı olan hasta sayısı 24 (%41,4) idi, bunlardan 5 tanesinde (%8,6) AVN vardı. AVN olan ve olmayan hastalar arasında çeşitli değikenlere göre istatistiksel anlamlı bir farklılık saptanmadı. Sadece bazal kompleman 3 (C3) düzeyi AVN hastalarında, AVN olmayan hastalara göre anlamlı olarak düşüktü. Sonuç: Bazal düşük C3 düzeyi jSLE hastalarında AVN gelişimini tahmin etmede ön görücü olabilir. jSLE hastalarında AVN gelişimi için muhtemel risk faktörlerini inceleyen çok merkezli çalışmalara ihtiyaç vardır.

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Damage index in childhood-onset systemic lupus erythematosus in Egypt

Cited by 13 publications

References 18 publications

Risk factors for damage in childhood-onset systemic lupus erythematosus in Asians: a case control study

Risk factors for damage in childhood-onset systemic lupus erythematosus in Asians: a case control study

Taxonomy for systemic lupus erythematosus with onset before adulthood

Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

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