A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children
Abstract Background IgA vasculitis (IgAV) is a small vessel vasculitis that is more common in childhood. Very limited evidence exists on patients who experience an atypical disease course. The aim of this study was to describe a cohort of children diagnosed with recurrent or persisting IgAV to ident...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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BMC
2023-08-01
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| Series: | Pediatric Rheumatology Online Journal |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12969-023-00872-1 |
| _version_ | 1797577519419883520 |
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| author | Julien Marro Chloe Williams Clare E. Pain Louise Oni |
| author_facet | Julien Marro Chloe Williams Clare E. Pain Louise Oni |
| author_sort | Julien Marro |
| collection | DOAJ |
| description | Abstract Background IgA vasculitis (IgAV) is a small vessel vasculitis that is more common in childhood. Very limited evidence exists on patients who experience an atypical disease course. The aim of this study was to describe a cohort of children diagnosed with recurrent or persisting IgAV to identify any themes associated with their disease course and areas of unmet needs. Methods A single centre retrospective study of children diagnosed with recurrent or persisting IgAV at Alder Hey Children’s Hospital (Liverpool, UK). Clinical data, including features at presentation and during follow up, potential triggers, abnormal laboratory and histology results, treatment and outcome at last clinical review were retrospectively collected. Key themes were identified. Results A total of 13 children met the inclusion criteria (recurrent disease, n = 4; persisting disease, n = 9). Median age at first presentation was 10.2 years [2.6–15.5], female:male ratio 1.2:1. Children in the atypical cohort were significantly older than a larger cohort of children who followed a non-complicated disease course (median age 5.5 years (range [0.6–16.7], p = 0.003)). All children re-presented with a purpuric rash (either recurring or persisting), accompanied by joint involvement in 92% of patients (12/13). Disease-modifying anti-rheumatic drugs (DMARDs) were used in 8/13 (62%) children. The median time from first presentation to diagnosis of atypical disease was 18.4 months [5.3-150.8] and the time from first presentation to treatment was 24.1 months [1.8–95.4]. Use of corticosteroids was significantly higher in children with renal involvement (p = 0.026). During follow up, 8/13 (62%) children were admitted at least once, whilst 10/13 (77%) had re-presented at least once to the emergency department. Five (38%) children were referred to psychology services and 7 (54%) children reported feelings of frustration. Conclusions This series describes some characteristics of a small cohort of children with atypical IgAV. It also identifies unmet needs in children with atypical IgAV, which includes delays in diagnosis and lengthy waits for treatment, lack of high-quality evidence regarding treatment choices and a high unrecognised disease burden. Further research is needed to study this subgroup of children as evidence is lacking. |
| first_indexed | 2024-03-10T22:09:28Z |
| format | Article |
| id | doaj.art-88bf52e55cd14212848b5d5210f9c590 |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-03-10T22:09:28Z |
| publishDate | 2023-08-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-88bf52e55cd14212848b5d5210f9c5902023-11-19T12:40:07ZengBMCPediatric Rheumatology Online Journal1546-00962023-08-012111910.1186/s12969-023-00872-1A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in childrenJulien Marro0Chloe Williams1Clare E. Pain2Louise Oni3Department of Women’s and Children’s Health, Institute of Life Course and Medical Sciences, University of LiverpoolRoyal Liverpool and Broadgreeen University Hospital TrustsDepartment of Women’s and Children’s Health, Institute of Life Course and Medical Sciences, University of LiverpoolDepartment of Women’s and Children’s Health, Institute of Life Course and Medical Sciences, University of LiverpoolAbstract Background IgA vasculitis (IgAV) is a small vessel vasculitis that is more common in childhood. Very limited evidence exists on patients who experience an atypical disease course. The aim of this study was to describe a cohort of children diagnosed with recurrent or persisting IgAV to identify any themes associated with their disease course and areas of unmet needs. Methods A single centre retrospective study of children diagnosed with recurrent or persisting IgAV at Alder Hey Children’s Hospital (Liverpool, UK). Clinical data, including features at presentation and during follow up, potential triggers, abnormal laboratory and histology results, treatment and outcome at last clinical review were retrospectively collected. Key themes were identified. Results A total of 13 children met the inclusion criteria (recurrent disease, n = 4; persisting disease, n = 9). Median age at first presentation was 10.2 years [2.6–15.5], female:male ratio 1.2:1. Children in the atypical cohort were significantly older than a larger cohort of children who followed a non-complicated disease course (median age 5.5 years (range [0.6–16.7], p = 0.003)). All children re-presented with a purpuric rash (either recurring or persisting), accompanied by joint involvement in 92% of patients (12/13). Disease-modifying anti-rheumatic drugs (DMARDs) were used in 8/13 (62%) children. The median time from first presentation to diagnosis of atypical disease was 18.4 months [5.3-150.8] and the time from first presentation to treatment was 24.1 months [1.8–95.4]. Use of corticosteroids was significantly higher in children with renal involvement (p = 0.026). During follow up, 8/13 (62%) children were admitted at least once, whilst 10/13 (77%) had re-presented at least once to the emergency department. Five (38%) children were referred to psychology services and 7 (54%) children reported feelings of frustration. Conclusions This series describes some characteristics of a small cohort of children with atypical IgAV. It also identifies unmet needs in children with atypical IgAV, which includes delays in diagnosis and lengthy waits for treatment, lack of high-quality evidence regarding treatment choices and a high unrecognised disease burden. Further research is needed to study this subgroup of children as evidence is lacking.https://doi.org/10.1186/s12969-023-00872-1Henoch schonlein purpuraIgAVChildrenChronicRecurrentPersisting |
| spellingShingle | Julien Marro Chloe Williams Clare E. Pain Louise Oni A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children Pediatric Rheumatology Online Journal Henoch schonlein purpura IgAV Children Chronic Recurrent Persisting |
| title | A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children |
| title_full | A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children |
| title_fullStr | A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children |
| title_full_unstemmed | A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children |
| title_short | A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children |
| title_sort | case series on recurrent and persisting iga vasculitis henoch schonlein purpura in children |
| topic | Henoch schonlein purpura IgAV Children Chronic Recurrent Persisting |
| url | https://doi.org/10.1186/s12969-023-00872-1 |
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