Clinical Features of Children With Henoch-Schonlein Purpura Risk Factors Associated With Renal Involvement
This study aimed to evaluate renal involvement and factors affecting the prognosis in patients with Henoch-Schonlein purpura (HSP).
The outcomes of 107 children diagnosed with HSP who had been followed up for at least 6 months were reviewed.
Renal involvement was observed in 26.1% of the patients. The mean age of the patients with renal involvement was 8.8 ± 4.0 years as compared to 7.1 ± 2.9 years in the patients without renal involvement (P = .02). The risk of renal involvement was found to be significantly higher in the patients who were 10 years old and over (P < .001). In the group with renal involvement, the frequency of scrotal involvement was significantly higher than that of the group without renal involvement (P = .02). The mean serum immunoglobulin A level of the patients with renal involvements was significantly higher (P = .04) and the mean serum complement C3 levels was significantly lower (P = .04) than those of the patients without renal involvement. None of the patients with renal involvement reached end-stage kidney failure. No significant relationship was observed between the development of renal involvement and early steroid treatment.
This study proposes that in old children with HSP, elevated serum immunoglobulin A levels, decreased serum complement C3 levels, and scrotal involvement are associated with renal involvement. We failed to find any effect of steroid treatment on development of renal involvement.
Available from: Amer Lardhi
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ABSTRACT: To evaluate the epidemiological and clinical profile of children with Henoch-Schonlein purpura (HSP) in eastern Saudi Arabia during a 15-year period.
The medical records of children discharged with a diagnosis of HSP from King Fahad Hospital of the University, Al-Khobar, Saudi Arabia, between January 1996, and December 2010, were reviewed retrospectively.
Of 78 patients, 46 (59%) were boys, with a male to female ratio of 1.4:1. The patients' ages ranged from 22 months to 12 years, with a mean of 6.3 years. Approximately 60% of cases were presented during autumn and winter. Upper respiratory tract infection preceded HSP in over half of the patients and antistreptolyzin O (ASO) titer was positive in 11 of the 24 (46%) children tested at presentation. The main clinical features included skin purpura (100%), arthritis or arthralgia (66%), gastrointestinal manifestation (47%), orchitis (15%) of boys, and nephritis (24%). One patient with severe nephritis developed pulmonary hemorrhage and acute respiratory distress syndrome. Eleven (14%) patients received corticosteroid therapy. All children made a full recovery, only one patient with nephritis continued to have hypertension at 2 years follow up. Symptoms recurred in 6 (7.7%) patients over a period ranging from one month to 2 years.
Henoch-Schonlein purpura is a mild disease in the eastern province of Saudi Arabia and with no significant differences in the epidemic and clinical profile than that reported elsewhere.
Available from: Maryam Piram
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ABSTRACT: Purpose of review:
To review the current knowledge of epidemiological features of immunoglobulin (Ig) A vasculitis (Henoch-Schönlein) and disease etiology.
The annual incidence of IgA vasculitis in the population is an estimated 3-26.7/100 000 for children and infants and 0.8-1.8/100 000 for adults. These may be conservative approximations of the true frequency because of skewed case-finding strategies. In children, the marked autumn-winter peak in incidence rates, the frequent occurrence after an upper respiratory tract infection and the short interval between disease onset in index cases and in other family members collectively point to a transmissible infectious process. A subset of adult IgA vasculitis could be related to preceding or concurrent malignancies. Despite several lines of evidence supporting the critical role of an exogenous factor in IgA vasculitis, recent progress has been made in understanding the genetic susceptibility to IgA vasculitis. Recent findings also lessened the suggestion that IgA vasculitis might be triggered by vaccination.
IgA vasculitis is two to 33 times more common in children than adults and appears to have a strong environmental component, with possibly different risk factors in childhood and adulthood. Support is strengthening for a role of genetics in IgA vasculitis.
Available from: Miguel Gonzalez-Gay
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ABSTRACT: The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3–20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2–38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).
In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement.
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