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REVIEW ARTICLE Advances in dermatology and venereology ActaDV Acta Dermato-Venereologica ActaDV
Henoch-Schönlein Purpura: A Literature Review
Liv Eline HETLAND 1, Kjærsti Sørensen SUSRUD 1, Kim Hein LINDAHL 2 and Anette BYGUM 3
1
Faculty of Health Sciences, University of Southern Denmark, 2 Department of Pathology, Odense University Hospital, and 3 Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark
Henoch-Schönlein purpura is the most common childhood vasculitis, but may also affect adults. This article reviews the literature since 2011 on advances in diagnosis, clinical disease manifestations, pathophysiology and treatment of Henoch-Schönlein purpura. The clinical manifestations are thought to arise from IgA depositions in blood vessel walls in the affected organs, mostly skin, gastrointestinal tract, joints and kidneys. Corticosteroids may be effective in rapid resolution of renal manifestations and treating joint and abdominal pain, but they are not proven effective for treating organ manifestations and complications, such as glomerulonephritis, bowel infarction or intussusception. Mycophenolate mofetil or cyclosporine A may be better treatment choices in case of renal involvement. Other immunosuppressive and immunomodulating drugs, such as rituximab and dapsone, are promising, but larger studies are needed to confirm these findings. Cancer screening should be considered in older males diagnosed with Henoch-Schönlein purpura.
Key words: Henoch-Schönlein purpura; vasculitis; immunoglobulin A; corticosteroids.
Accepted Jun 22, 2017; Epub ahead of print Jun 27, 2017 Acta Derm Venereol 2017; 97: 1160 – 1166.
Corr: Liv Eline Hetland, Faculty of Health Sciences, University of Southern Denmark, Henriettevej 36, DK-5000 Odense, Denmark. E-mail: livelinehetland @ gmail. com
Henoch-Schönlein purpura( HSP) is the most common childhood vasculitis, affecting 10 – 20 children per 100,000 per year. More than 90 % of patients are under 10 years of age, with a mean age of 6 years( 1, 2). HSP is a leukocytoclastic vasculitis involving small vessels( 3). Its clinical presentation includes cutaneous palpable purpura, joint pain, renal involvement, colicky abdominal pain and gastrointestinal bleeding. Most cases of HSP occur in autumn and winter. Proposed triggers include upper respiratory tract infections, medications, vaccinations, and malignancies( 4, 5). The pathophysiology behind HSP is not yet completely understood. HSP is generally self-limiting and harmless, but concomitant nephritis may cause severe complications. The proportion of patients having renal involvement varies between 20 % and 80 % in the literature( 6). The estimated incidence of nephrotic or nephritic syndrome is ~ 7 % of all HSP cases, and 1 % of patients develop end-stage renal failure( 7, 8). HSP nephritis( HSPN) usually occurs within 1 – 2 months after the onset of HSP.
The diagnosis of HSP is criteria-based. The European League Against Rheumatism( EULAR), the Paediatric Rheumatology International Trials Organization( PRIN- TO) and the Paediatric Rheumatology European Society( PRES) published a revised set of criteria in 2010, with high sensitivity and specificity( 9).
The severity and organ involvement of the disease dictates the treatment. In general, treatment for HSP without renal involvement is symptomatic. HSPN is commonly treated with corticosteroids or other immunosuppressive and modulating drugs. Existing studies are inconclusive regarding drug of choice.
METHODS
A systematic search of the literature was performed in PubMed and Embase databases. The MeSH term in the PubMed database was“ Henoch Schönlein purpura”, limited to the title, for articles published between 2011 and 2016. This search yielded 508 articles. In the Embase database, the keywords were“ henoch”,“ henoch schönlein purpura”,“ purpura” and“ schönlein”. The same limitations applied. This search yielded 1,503 articles. The number of articles chosen for further reading was 300. The references in the articles selected for this review were investigated further.
DIAGNOSIS
HSP diagnosis is based on clinical criteria. The revised criteria developed by EULAR / PRINTO / PRES were published in 2010, and are the gold standard for the diagnosis of HSP( Table I). The sensitivity is 100 % and specificity 87 %, when applied to children( 9). One study reviewed these criteria to assess applicability to adults, and found a diagnostic sensitivity of 99.2 % and specifi-
Table I. Diagnostic criteria for Henoch-Schönlein purpura( HSP), as developed by EULAR / PRINTO / PRES
Criterion Mandatory criterion
Minimum 1 out of 4 criteria
Description
Purpura or petechiae with lower limb predominance
1. Diffuse abdominal pain with acute onset
2. Histopathology showing leukocytoclastic vasculitis or proliferative glomerulonephritis, with predominant immunoglobulin A( IgA) deposits 3. Arthritis or arthralgia of acute onset 4. Renal involvement in the form of proteinuria or haematuria
EULAR / PRINTO / PRES: the European League Against Rheumatism, the Paediatric Rheumatology International Trials Organization and the Paediatric Rheumatology European Society( 8, 9). doi: 10.2340 / 00015555-2733 Acta Derm Venereol 2017; 97: 1160 – 1166
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2017 Acta Dermato-Venereologica.