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Life-threatening complications of Henoch-Schönlein purpura: diffuse alveolar haemorrhage, venous thrombosis and bowel ischaemia

Dhaliwal KK, Lile NA, Tan CL, Lim CH

BMJ Case Rep, 2020 Sep 29;13(9).
PMID: 32994270 DOI: 10.1136/bcr-2020-235905

Henoch-Schönlein purpura (HSP) is a common systemic vasculitis occurring in children. Making a diagnosis of HSP is often straightforward, managing its complications can be difficult. Diffuse alveolar haemorrhage (DAH), bowel ischaemia and venous thrombosis are rare complications of this disorder. We present a case of a 15-year-old teenage girl presenting with typical purpuric rash of HSP, developed DAH, bowel ischaemia and venous thrombosis. She was successfully treated with pulse methylprednisolone, intravenous Ig and intravenous cyclophosphamide.

Matched MeSH terms: Purpura, Schoenlein-Henoch/diagnosis*
Hypertension in the absence of urinary abnormalities - An unusual presentation of anaphylactoid purpura

Thong PL, How KN, Bakrin IH

Med J Malaysia, 2020 07;75(4):436-438.
PMID: 32724011

Henoch-Schonlein Purpura (HSP) or anaphylactoid purpura, currently named IgA vasculitis is the most common form of systemic vasculitis in children. In adults and young infants, HSP tends to have atypical presentations with higher rates of severe gastrointestinal problems and delayed renal complications. While hypertension is a known complication of HSP nephritis, it is rarely seen in individuals with normal renal function and urinary findings. We report a case of a 7-year-old boy with HSP, who presented with abdominal pain and severe hypertension without other features of glomerulonephritis.

Matched MeSH terms: Purpura, Schoenlein-Henoch/diagnosis*
Fulltext Ibuprofen-induced Henoch-Schönlein purpura nephritis: First reported case

Seong CL, Shanmuganathan M

Indian J Pharmacol, 2016 Nov-Dec;48(6):739-740.
PMID: 28066119 DOI: 10.4103/0253-7613.194848

Ibuprofen is a nonsteroidal anti-inflammatory drug that is used widely in treating pain, fever, and inflammation. Its side effects are mainly due to acute renal impairment and gastric discomfort. We hereby report a rare case of Henoch-Schönlein purpura nephritis secondary to ibuprofen consumption which has not been reported in literature before.

Matched MeSH terms: Purpura, Schoenlein-Henoch/diagnosis*
Outcome of immunosuppression in children with IgA vasculitis-related nephritis

Rohner K, Marlais M, Ahn YH, Ali A, Alsharief A, Novak AB, et al.

Nephrol Dial Transplant, 2024 Jul 31;39(8):1299-1309.
PMID: 38211969 DOI: 10.1093/ndt/gfae009

BACKGROUND: Immunoglobulin A vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Due to a lack of evidence, treatment recommendations are based on expert opinion, resulting in variation. The aim of this study was to describe the clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy-proven IgAVN in order to identify prognostic risk factors and signals of treatment efficacy.
METHODS: Retrospective data were collected on 1148 children with biopsy-proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analysed using multivariate analysis. The primary outcome was estimated glomerular filtration rate (eGFR) and persistent proteinuria at last follow-up.
RESULTS: The median follow-up was 3.7 years (interquartile range 2-6.2). At last follow-up, 29% of patients had an eGFR <90 mL/min/1.73 m2, 36% had proteinuria and 3% had chronic kidney disease stage 4-5. Older age, lower eGFR at onset, hypertension and histological features of tubular atrophy and segmental sclerosis were predictors of poor outcome. There was no evidence to support any specific second-line immunosuppressive regimen being superior to others, even when further analysing subgroups of children with reduced kidney function, nephrotic syndrome or hypoalbuminemia at onset. Delayed start of immunosuppressive treatment was associated with a lower eGFR at last follow-up.
CONCLUSION: In this large retrospective cohort, key features associated with disease outcome are highlighted. Importantly, there was no evidence to support that any specific immunosuppressive treatments were superior to others. Further discovery science and well-conducted clinical trials are needed to define accurate treatment and improve outcomes of IgAVN.

Matched MeSH terms: Purpura, Schoenlein-Henoch/diagnosis