Henley Wl

Bio: Henley Wl is an academic researcher. The author has contributed to research in topics: Lupus erythematosus. The author has an hindex of 1, co-authored 1 publications receiving 98 citations.

Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults

Abstract: Objective—To define the pattern of disease expression in patients with childhood onset systemic lupus erythematosus (SLE). Methods—Prospective analysis of clinical manifestations and immunological features of 34 patients in whom the first manifestations appeared in childhood from a series of 430 unselected patients with SLE. Results—Thirty one (91%) patients from the childhood onset group were female and three male (9%) (ratio female/male, 10/1, with no diVerence compared with the adult onset group).Mean age of this group at disease onset was 11 years (range 5‐14) compared with 32 years (15‐48) for the remaining patients. The childhood onset patients more often had nephropathy (20% v 9% in adult onset SLE, p=0.04; OR:2.7; 95%CI:1.1, 7), fever (41% v 21%, p=0.006; OR:2.6, 95%CI:1.2, 5.7), and lymphadenopathy (6% v 0.5%, p=0.03, OR: 12.3, 95%CI: 1.2, 127.6), as presenting clinical manifestations. During the evolution of the disease, the childhood onset patients had an increased prevalence of malar rash (79% v 51%, p=0.002; OR:3.7; 95%CI:1.5, 9.5) and chorea (9% v 0%, p<0.0001). This group exhibited a higher prevalence of anticardiolipin antibodies (aCL) of the IgG isotype when compared with the remaining patients (29% v 13%, p=0.017; OR:2.9, 95%CI:1.2, 6.8). No significant diVerences were found among the other antibodies between the two groups. Childhood onset patients more often received azathioprine (15%v6%, p=0.00004; OR:11.2; 95%CI:2.8, 44.9) but no diVerences were detected between the groups concerning side eVects or drug toxicity. Conclusions—The presentation and the clinical course of SLE varied in this series of 430 patients depending on their age at disease onset. Nephropathy, fever, and lymphadenopathy were more common in childhood onset patients as presenting clinical manifestations, while malar rash, chorea, and detection of IgG aCL were more common during the evolution of the disease.

Lupus nephritis in childhood and adolescence.

Abstract: Lupus nephritis in childhood usually presents after the age of 10 years, and presentation under 5 years is very rare. More males (F:M ratio 4.5:1) are affected than in adult-onset cases, but the ratio is the same in prepubertal and pubertal children. The incidence of clinically evident renal disease is greater at onset than in adults (82%), the usual presentation being with proteinuria, 50% having a nephrotic syndrome. Half the children show World Health Organisation class IV nephritis in renal biopsies. Neuropsychiatric lupus is present at onset in 30%, may complicate 50% at some point and remains a major problem. Prognosis has improved greatly over the past 30 years, at least in part the result of immunosuppressive treatment. Treatment of the initial phase may be guided by the severity of the renal biopsy appearances, more aggressive treatment including cytotoxic agents, i.v. methylprednisolone and perhaps plasma exchange, although the value of exchange is not established. Controversy persists as to the most effective cytotoxic treatment in the acute phase, both oral and i.v. cyclophosphamide and azathioprine being used in different units. In the chronic maintenance phase it seems established both clinically and histologically that addition of a cytotoxic agent improves outcome, but again the drug and route of administration are contentious. Azathioprine has the advantage of being safe for pregnancy and not gonadotoxic, whilst i.v. cyclophosphamide has been demonstrated to improve results over prednisolone alone in controlled trials and has advantages in non-compliant patients. No trial comparing the two regimes has been carried out, and one is needed. Today children much less commonly go into renal failure, and the main causes of actual death (15% of patients over 10 years) are now infections and extra-renal manifestations of lupus, principally neurological. Morbidity of the disease and the treatment remain a major problem, especially when treatment exacerbates complications of the disease itself, such as infections, osteonecrosis, thrombosis, vascular disease and possibly neoplasia.

Age at Onset and Causes of Disease

Abstract: We know a good deal about the origins and pathogenesis of monogenic diseases, but we still know too little of how and what the genes contribute to multifactorial diseases We seldom know which loci are involved, or which alleles inhabit them Nor can we say how the effects of each gene modify those of others in the deranged homeostasis that constitutes the phenotype And we can seldom specify the elements of the environment that interact with the effects of the genes

Differences in clinical manifestations between childhood-onset lupus and adult-onset lupus: a meta-analysis.

Abstract: Objective: It is known that age at disease onset has an impact on the clinical course andoutcome of systemic lupus erythematosus (SLE); however, the precise differences in theprevalence of SLE manifestations are debated. Our objective was to conduct a systematicliterature review and meta-analysis of all studies that directly compare childhood-onset lupus with adult-onsetlupus to determine which clinical manifestations vary with age at disease onset. Methods: A comprehensive literature search of the MEDLINE/PubMed,EMBASE, CINAHL, and SCOPUS databases was conducted to identify relevant articles. Study quality was assessed using the STROBE checklist. Study sample characteristics and clinical manifestation event rates were extracted from each study. Pooled odds ratios (ORs) were calculated using the random effects method, and between-study heterogeneity was quantified using the I2 statistic. Results: Of the 484studies identified by the search strategy, 16 were included in this review. The total number of pati...