Differences between adult and pediatric onset Henoch‐Schonlein purpura from North India

Abstract

Aim: Henoch-Sch€ onlein purpura (HSP), a primary vasculitis, characterized by purpura, abdominal pain, arthritis and renal involvement, is predominantly a disease of childhood. However, rarely it can occur in adults in whom it is believed to be a more severe form with poor renal outcomes. We aimed to answer if the age of onset affected the clinical spectrum and renal outcomes of the disease in a north Indian population. Hence, we studied the differences in clinical spectrum and renal outcomes between adult-onset HSP and childhood-onset HSP.Methods: Case records of all adult patients diagnosed with HSP (onset ≥ 18 years) over the last 25 years in our department were retrieved. Data on clinical features, lab abnormalities and outcomes were extracted and compared with that in pediatric HSP patients (onset < 18 years) seen during the same period.Results: A total of 87 patients, including 30 adults and 57 children, were seen during this period. Compared to children, most of the adults had purpura as the first clinical manifestation (86.7% vs. 56.1%, P < 0.01) whereas abdominal pain was only rarely the initial symptom in adults (10.0% vs. 36.8%, P < 0.02). During the disease course, adults had a higher frequency of joint involvement as compared to children (90.0% vs. 43.9%, P < 0.001). There was no significant difference in the frequency of renal involvement (60.0% vs. 50.9%, P = NS) and gastrointestinal involvement (66.7% vs. 84.2%, P = NS) between adults and children. Outcomes were good in both groups and most adults and children achieved complete recovery (83.3% and 86.0%, respectively). There was no difference in the frequency of immunosuppressive treatment required by the two groups. None of the patients developed renal insufficiency on follow-up. Conclusion:Compared to children, adults with HSP seldom have abdominal pain as the first clinical manifestation while joint involvement is seen more commonly in them during the disease course.

Clinical Characteristics of Biopsy-Proven IgA Vasculitis in Children and Adults: A Retrospective Cohort Study

“…During the first 5 years of the disease in the current study, patients were in complete response only 40% of the time, and relapses occurred in 26 patients. Although the rates of remission 14,18,20,22,23,25 and frequency of relapses 14,16,18,19,22 are lower in our study population than in other cohorts, this difference is likely due in part to the strict definitions of disease status used in the current study. This variance is further supported by similar frequencies of disease status in our study compared with the report by Audemard-Verger et al, 28 from which our disease status definitions were adapted.…”

“…[10][11][12][13] Differences in both the presentation and outcome have been described in adults compared with children, with most studies reporting a higher frequency of severe renal manifestations and poorer renal outcome among the adult population. [14][15][16][17][18][19][20][21][22][23][24][25] Although multiple European and Asian studies have been reported, cohorts evaluating the differences between patients with adult-and childhood-onset IgAV in North America are limited. The aim of this study was to describe the differences between baseline clinical characteristics and outcomes of childhood-and adult-onset biopsy-proven IgAV from a large, North American, singleinstitution cohort.…”

Childhood IgA Vasculitis (Henoch Schonlein Purpura)—Advances and Knowledge Gaps

“…Older children, mainly teenagers with IgAV, are more likely to have disease that reflects that of adult-onset IgAV (5). Adult onset disease differs from childhood onset disease in terms of its manifestations with a large comparative series describing adults rarely presenting with abdominal pain (10 vs. 37%) and adults have higher frequency of joint involvement when compared to children (90 vs. 44%) (6). Longer-term outcomes are generally good and appear similar between the two age groups (7) although smaller reports suggest that adults are more likely to progress to end stage kidney disease (ESKD) (8).…”

An Unusual Clinical Presentation of Immunoglobulin A Vasculitis in an Elderly Patient

“…There are differences in the expression of the disease across the different age groups. Children were noted to have a preceding upper respiratory tract infection, fever, and abdominal pain, while adults had mostly symptoms of joint pain and increased sedimentation rates, and more frequent and severe renal involvement [4,7].…”

“…Older children, mainly teenagers with IgAV, tend to have a disease similar to adult-onset IgAV [3]. Adult-onset disease rarely presents with abdominal involvement, but joint involvement is more predominant [4].…”