Henoch-Schönlein Purpura in Children
Introduction
Henoch-Schonlein purpura (HSP) is a multisystem disorder affecting mainly the skin, joints, gastro-intestinal tract and kidneys. Involvement of other organs (pulmonary haemorrage and CNS involvement) is rare. The extrarenal manifestations are due to a small vessel leukocytoclastic vasculitis. In the kidneys there is mesangial expansion with deposition of IgA. HSP can occur at any age but is most frequent in children under 10 years. There is a slight male predominance. The incidence is reported as 13.5-21.7/100,000 per year. It is more common in winter and commonly follows an upper respiratory infection. The aetiology and pathogenesis are poorly understood.
Henoch-Schonlein purpura (HSP) is a multisystem disorder affecting mainly the skin, joints, gastro-intestinal tract and kidneys. Involvement of other organs (pulmonary haemorrage and CNS involvement) is rare. The extrarenal manifestations are due to a small vessel leukocytoclastic vasculitis. In the kidneys there is mesangial expansion with deposition of IgA. HSP can occur at any age but is most frequent in children under 10 years. There is a slight male predominance. The incidence is reported as 13.5-21.7/100,000 per year. It is more common in winter and commonly follows an upper respiratory infection. The aetiology and pathogenesis are poorly understood.
Scope
Paediatricians working in paediatric endocrinology network centres across Wessex.
Paediatricians working in paediatric endocrinology network centres across Wessex.
Purpose
The aim of the guideline is to improve patient experience, clinical outcomes, provide an agreed clinical approach to managing these babies and families, enable prompt institution of relevant investigations, optimise communication with parents and facilitate equity of access to specialist services.
The aim of the guideline is to improve patient experience, clinical outcomes, provide an agreed clinical approach to managing these babies and families, enable prompt institution of relevant investigations, optimise communication with parents and facilitate equity of access to specialist services.
Definitions
HSP is a clinical diagnosis.
a. Cutaneous
extensor surfaces of limbs and buttocks
erythematous macules developing into purple, non-blanching, non-pruritic urticarial, palpable purpuric lesions
rarely haemorrhagic bullae and vesicles which desquamate
<3 yr old subcutaneous oedema of the scalp, ears, periorbital area, dorsi of the hands and feet and genitalia
b. Gastrointestinal
occurs in 2/3 of cases (90% of those with renal involvement)
abdominal pain may precede rash in 14 - 36% patients
vomiting, diarrhoea, periumbilical pain, bloody stools, upper GIT haemorrhage
intussusception in 4-5% patients
rarely: haemorrhagic pancreatitis, hydrops of the gallbladder, pseudomembranous colitis
c. Articular
50-80% patients
arthralgia, periarticular oedema
synovial effusions typically absent
knees and ankles most commonly affected, less commonly wrists, fingers and elbows
d. Renal
Present in 40-60%
Renal involvement is often absent at presentation and may only be apparent weeks or months later.
The presentation of renal involvement is variable:
o Microscpoic haematuria, transient or persistent
o Macroscopic haematuria
o Proteinuria
o Nephrotic syndrome
o Mixed nephritic-nephrotic syndrome
e. Other features
genital:orchitis, cord haematoma, testicular pain, testicular necrosis, painful ecchymotic induration of the scrotum
cns: seizures, encephalopathy, coma, Guillain-Barre, cortical blindness, intracerebral haemorrhages, stroke
carditis
parotitis
pulmonary disease with haemorrhage
HSP is a clinical diagnosis.
a. Cutaneous
extensor surfaces of limbs and buttocks
erythematous macules developing into purple, non-blanching, non-pruritic urticarial, palpable purpuric lesions
rarely haemorrhagic bullae and vesicles which desquamate
<3 yr old subcutaneous oedema of the scalp, ears, periorbital area, dorsi of the hands and feet and genitalia
b. Gastrointestinal
occurs in 2/3 of cases (90% of those with renal involvement)
abdominal pain may precede rash in 14 - 36% patients
vomiting, diarrhoea, periumbilical pain, bloody stools, upper GIT haemorrhage
intussusception in 4-5% patients
rarely: haemorrhagic pancreatitis, hydrops of the gallbladder, pseudomembranous colitis
c. Articular
50-80% patients
arthralgia, periarticular oedema
synovial effusions typically absent
knees and ankles most commonly affected, less commonly wrists, fingers and elbows
d. Renal
Present in 40-60%
Renal involvement is often absent at presentation and may only be apparent weeks or months later.
The presentation of renal involvement is variable:
o Microscpoic haematuria, transient or persistent
o Macroscopic haematuria
o Proteinuria
o Nephrotic syndrome
o Mixed nephritic-nephrotic syndrome
e. Other features
genital:orchitis, cord haematoma, testicular pain, testicular necrosis, painful ecchymotic induration of the scrotum
cns: seizures, encephalopathy, coma, Guillain-Barre, cortical blindness, intracerebral haemorrhages, stroke
carditis
parotitis
pulmonary disease with haemorrhage
Differential Diagnosis
- sepsis, especially meningococcal disease. (See guideline for meningococcal disease)
- systemic vasculitides (Wegeners, SLE, hypersensitivity vasculitis, polyarteritis nodosa)
- ITP and other forms of purpura (See ITP guideline)
Investigations
Other investigations are not routine if the patient is well, the clinical diagnosis is confident and the urine dipsticks are clear.
- Blood pressure
- FBC
- Urine dipstick
Other investigations are not routine if the patient is well, the clinical diagnosis is confident and the urine dipsticks are clear.
Initial Management
- Supportive: hydration, nutrition, electrolyte balance
- Simple analgesia
- Use NSAIDs only if renal function is normal
- If severe abdominal pain, arthritis, pulmonary haemorrhage: consider prednisolone 1-2mg/kg [1],[2] after discussion with consultant
- If severe skin manifestations: consider colchicine or dapsone [3], [4] in consultation with a dermatologist
Consider for admission
Any patient with:
Any patient with:
- Severe joint pain
- Significant abdominal pain (may have intussusception)
- Melaena
- Oedema/nephrotic syndrome
- Hypertension
Follow up
as per management plans
as per management plans
Treatment Options- HSP Nephritis
Indications for referral to a paediatric nephrologist:
Renal biopsy
No firm evidence to guide us
Purpose – to confirm diagnosis
Indications for Biopsy
Indications for referral to a paediatric nephrologist:
- Acute nephritic syndrome: haematuria +2 other signs (proteinuria, oedema, hypertensive, oliguria, raised urea and creatinine)
- Macroscopic haematuria 4 weeks, or with 2+ proteinuria
- Microscopic haematuria > 2 years
- Hypertension
- Proteinuria:
- Nephrotic syndrome at any time
- urine protein/creatinine ratio > 200 with normal plasma albumin concentration for 1 month
- Urine protein/creatinine ratio >100 for 6 months
- Urine protein/creatinine ratio > 25 for 1 year
Renal biopsy
No firm evidence to guide us
Purpose – to confirm diagnosis
- to guide treatment
- prognostic information
Indications for Biopsy
- raised ASOT, abnormal C3/C4, ANCA, Auto-antibody profile
- rapidly progressive glomerulonephritis
- Acute nephritic syndrome [8], [9], [10]
- Nephrotic syndrome: 1 month after onset [5]
- Persistent nephrotic range proteinuria: 2 months after onset [11]
Suggested Treatment- HSP Nephritis
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Dialysis dependent RPGN
+ cyclophosphamide (2.5mg/kg/d, 8wks) [18] + prednisolone (60mg/m2 1mth, 40mg/m2 1 mth, then taper over next 4mths: total 6mths) Crescents [18], [19], [20], [21], / Tubulo-interstitial changes [18], [19], [6]
+/- cyclophosphamide [22], [24], [25], [26] +/- +/- plasma exchange |
>50 % Crescents
+ prednisolone (60mg/m2 1mth, 40mg/m2 1 mth, then taper over next 4mths: total 6mths) +/- Plasma exchange Acute nephritic/ nephrotic syndrome with minimal biopsy findings [6], [21]
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Persistent proteinuria (>100mg/mmol creat)
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Nephrotic range proteinuria
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References
[1] Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Henoch-Schönlein syndrome). Am J Dis Child 1960; 99: 147-68
[2] Rosenblum ND, Winter HS. Steroid effects on the course of abdominal pain in children with HSP. Pediatr 1987; 79: 1018-21
[3] Fredenberg MF, Malkinson FD. Sulfone therapy in the treatment of leukocytoclastic vasculitis. J Am Acad Dermatol 1987; 16: 772-78
[4] Hoffbrand BI. Dapsone in Henoch-Schönlein purpura-worth a trial? Postgrad Med 1991; 67: 961-62
[5] Ronkainen J, Ala-Houhala M, Huttunen –P, Jahnukainen T, Koskimies O, Örmälä T, Nuutinen M. Outcome of Henoch-Schönlein nephritis with nephritic range proteinuria. Clinical Nephrology 2003; 60(2): 80-84
[6] Foster BJ, Bernard C, Drummond KN, Sharma AK. Effective therapy for severe Henoch-Schönlein purpura nephritis with prednisolone and azathioprine: A clinical and histopathologic study. Journal of Pediatrics 2000;136(3): 370-375
[7] Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study. Lancet 2002; 360(9334): 666-70
[8] Scharer K, Krmar R, Querfeld U, Ruder H, Waldherr R, Schaefer F. Clinical outcome of Henoch-Schönlein purpura nephritis in children. Pediatr Nephrol 1999; 13(9): 816-23
[9] Coppo R, Mazzucco G, Cagnoli L, Lupo A, Schena FP. Long-term prognosis of Henoch-Schönlein nephritis in adults and children. Italian Group of Renal
Immunopathology Collaborative Study on Henoch-Schönlein purpura. Nephrol Dial Transplant 1997; 12(11): 2277-83
[10] Stewart M, Savage JM, Bell B, McCord B. Long term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population
[11] Andreoli SP. Chronic glomerulonephritis in childhood. Menbranoproliferative glomerulonephritis, Henoch-Schönlein purpura nephritis, and IgA nephropathy. Pediatr Clin North Am 1995; 42(6): 1487-503
[12] Saulsbury FT. Henoch-Schöenlein purpura in children. Report of 100 patients and review of the literature. Medicine 1999; 78: 395-409
[13] Woo KT, Lau YK, Wong KS, Chiang GSC. ACEI/ATRA therapy decreases proteinuria by improving glomerular permselectivity in IgA nephritis. Kidney International 2000; 58: 2485-2491
[14] Russo D, Pisani A, Balletta MM, De Nicola L, Savino FA, Andreucci M, Minutolo R. Additive antiproteinuric effect of converting enzyme inhibitor and Losartan in normotensive patients with IgA nephropathy. Am J Kidney Dis 1999; 33: 851-856
[15] Ruggenenti P, Perua A, Benini R, Bertani T, Zoccali C, Maggiore Q, Salvadori M, Remuzzi G. In chronic nephropathies prolonged ace inhibition can induce remission: Dynamics of time-dependent changes in GFR. J Am Soc Nephrol 1999; 10: 997-1006
[16] Nakamura t, Obata J, Kimura H, Ohno S, Yoshida Y, Kawaihi H, Schimizu F. Blocking angiotensin II ameliorates proteinuria and glomerular lesions in progressive mesangioproliferative glomeerulonephritis. Kidney Int 1999; 55: 877-889
[17] Nakajima M, Hutchinson HG, Fujinaga M, Hayashida W, Morishita R, Zhang L, Horiuchi M, Pratt RE, Dzau VJ. The angiotensin II type 2 (AT2) receptor antagonizes the growth effects of the AT1 receptor: Gain-of-function study using gene transfer. Proc Natl Acad Sci USA 1995; 92: 10663-10667
[18] Öner A, Tinaztepe K, Erdogan Ö. The effect of triple therapy on rapidly progressive type of Henoch-Schönlein nephritis. Pediatr Nephrol 1995; 9: 6-10
[19] Kawasaki Y, Suzuki J, Nozawa R, Suzuki S, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy for severe Henoch-Schönlein nephritis. Pediatrics 2003; 111(4): 785-789
[20] Jardim HM, Leake J, Risdon RA, Barratt TM, Dillon MJ. Crescentic glomerulonephritis in children. Pediatr Nephrol 1992; 6(3): 231-5
[21] Bergstein J, Leiser J, Andreoli P. Response of crescentic Henoch-Schönlein purpura nephritis to corticosteroid and azathioprine therapy. Clinical Nephrology 1998; 49(1): 9-14
[22] Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis. Pediatr Nephrol 1998; 12: 238-243
[23] Hattori M, Ito K, Konomoto T, Kawaguchi H, Yoshioka T, Khono M. Plasmapheresis as the sole therapy for rapidly progressive Henoch-Schönlein purpura nephritis in children. American Journal of Kidney Diseases 1999; 33(3): 427-433
[24] Iijima K, Ito-Kariya S, Nakamura H, Yoshikawa N. Multiple combined therapy for severe Henoch-Schönlein nephritis in children. Pediatr Nephrol 1998; 12: 244-248
[25] Flynn JT, Smoyer WE, Bunchman TE, Kershaw DB, Sedman AB. Treatment of Henoch-Schönlein purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide. Am J Nephrol 2001; 21: 128-133
[1] Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Henoch-Schönlein syndrome). Am J Dis Child 1960; 99: 147-68
[2] Rosenblum ND, Winter HS. Steroid effects on the course of abdominal pain in children with HSP. Pediatr 1987; 79: 1018-21
[3] Fredenberg MF, Malkinson FD. Sulfone therapy in the treatment of leukocytoclastic vasculitis. J Am Acad Dermatol 1987; 16: 772-78
[4] Hoffbrand BI. Dapsone in Henoch-Schönlein purpura-worth a trial? Postgrad Med 1991; 67: 961-62
[5] Ronkainen J, Ala-Houhala M, Huttunen –P, Jahnukainen T, Koskimies O, Örmälä T, Nuutinen M. Outcome of Henoch-Schönlein nephritis with nephritic range proteinuria. Clinical Nephrology 2003; 60(2): 80-84
[6] Foster BJ, Bernard C, Drummond KN, Sharma AK. Effective therapy for severe Henoch-Schönlein purpura nephritis with prednisolone and azathioprine: A clinical and histopathologic study. Journal of Pediatrics 2000;136(3): 370-375
[7] Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study. Lancet 2002; 360(9334): 666-70
[8] Scharer K, Krmar R, Querfeld U, Ruder H, Waldherr R, Schaefer F. Clinical outcome of Henoch-Schönlein purpura nephritis in children. Pediatr Nephrol 1999; 13(9): 816-23
[9] Coppo R, Mazzucco G, Cagnoli L, Lupo A, Schena FP. Long-term prognosis of Henoch-Schönlein nephritis in adults and children. Italian Group of Renal
Immunopathology Collaborative Study on Henoch-Schönlein purpura. Nephrol Dial Transplant 1997; 12(11): 2277-83
[10] Stewart M, Savage JM, Bell B, McCord B. Long term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population
[11] Andreoli SP. Chronic glomerulonephritis in childhood. Menbranoproliferative glomerulonephritis, Henoch-Schönlein purpura nephritis, and IgA nephropathy. Pediatr Clin North Am 1995; 42(6): 1487-503
[12] Saulsbury FT. Henoch-Schöenlein purpura in children. Report of 100 patients and review of the literature. Medicine 1999; 78: 395-409
[13] Woo KT, Lau YK, Wong KS, Chiang GSC. ACEI/ATRA therapy decreases proteinuria by improving glomerular permselectivity in IgA nephritis. Kidney International 2000; 58: 2485-2491
[14] Russo D, Pisani A, Balletta MM, De Nicola L, Savino FA, Andreucci M, Minutolo R. Additive antiproteinuric effect of converting enzyme inhibitor and Losartan in normotensive patients with IgA nephropathy. Am J Kidney Dis 1999; 33: 851-856
[15] Ruggenenti P, Perua A, Benini R, Bertani T, Zoccali C, Maggiore Q, Salvadori M, Remuzzi G. In chronic nephropathies prolonged ace inhibition can induce remission: Dynamics of time-dependent changes in GFR. J Am Soc Nephrol 1999; 10: 997-1006
[16] Nakamura t, Obata J, Kimura H, Ohno S, Yoshida Y, Kawaihi H, Schimizu F. Blocking angiotensin II ameliorates proteinuria and glomerular lesions in progressive mesangioproliferative glomeerulonephritis. Kidney Int 1999; 55: 877-889
[17] Nakajima M, Hutchinson HG, Fujinaga M, Hayashida W, Morishita R, Zhang L, Horiuchi M, Pratt RE, Dzau VJ. The angiotensin II type 2 (AT2) receptor antagonizes the growth effects of the AT1 receptor: Gain-of-function study using gene transfer. Proc Natl Acad Sci USA 1995; 92: 10663-10667
[18] Öner A, Tinaztepe K, Erdogan Ö. The effect of triple therapy on rapidly progressive type of Henoch-Schönlein nephritis. Pediatr Nephrol 1995; 9: 6-10
[19] Kawasaki Y, Suzuki J, Nozawa R, Suzuki S, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy for severe Henoch-Schönlein nephritis. Pediatrics 2003; 111(4): 785-789
[20] Jardim HM, Leake J, Risdon RA, Barratt TM, Dillon MJ. Crescentic glomerulonephritis in children. Pediatr Nephrol 1992; 6(3): 231-5
[21] Bergstein J, Leiser J, Andreoli P. Response of crescentic Henoch-Schönlein purpura nephritis to corticosteroid and azathioprine therapy. Clinical Nephrology 1998; 49(1): 9-14
[22] Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis. Pediatr Nephrol 1998; 12: 238-243
[23] Hattori M, Ito K, Konomoto T, Kawaguchi H, Yoshioka T, Khono M. Plasmapheresis as the sole therapy for rapidly progressive Henoch-Schönlein purpura nephritis in children. American Journal of Kidney Diseases 1999; 33(3): 427-433
[24] Iijima K, Ito-Kariya S, Nakamura H, Yoshikawa N. Multiple combined therapy for severe Henoch-Schönlein nephritis in children. Pediatr Nephrol 1998; 12: 244-248
[25] Flynn JT, Smoyer WE, Bunchman TE, Kershaw DB, Sedman AB. Treatment of Henoch-Schönlein purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide. Am J Nephrol 2001; 21: 128-133
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Document Version:
1.0 Lead Author: Dr Justin Davies, Consultant Paediatric Endocrinologist |
Approving Network:
Wessex Paediatric Endocrine Network Date of Approval: 27/7/19 Review Date: July 2022 |
PIER ContactGary Connett
PIER Clinical Lead, Southampton Children's Hospital Tremona Road, Southampton Hampshire, SO16 6YD Telephone: 023 8079 6972 |
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