Henoch-Schönlein purpura - IgA vasculitis (< 18 years) - Curbside
Henoch-Schönlein purpura - IgA vasculitis (< 18 years)
Editors: Dan Imler, MD
Inclusion Criteria  (Any one criteria present)
  • Clinical concern for Henoch-Schönlein purpura
Exclusion Criteria
  • History of thrombocytopenia or coagulopathy
  • History of cancer or immunosuppression
  • History of rheumatologic or vasculitis disease
  • Complex medical disorder
  • Ill appearance or mental status changes
  • Pregnancy

Consider rheumatology or nephrology consult

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Evidence
Total Notes: 5
Evidence

1 Clinical manifestations

HSP (Immunoglobulin A vasculitis) is the most common systemic vasculitis of childhood. The cause of HSP is not fully know, however it likely represents a post-infectious inflammatory condition.

 

Skin findings

  • Occurs in 75% of patients
  • Usually starts with a presention of erythematous, macular/urticarial lesions that evolve into ecchymoses, petechiae, and palpable purpura.
  • Sometimes itchy, rarely painful
  • Typically appears in gravity/pressure-dependent areas (buttocks, lower extremities)

Arthritis/arthralgia

  • May be the sole presenting complaint in up to 15% of patients
  • Usually transient or migratory in large joints of lower extremities
  • Does not produce chronic damage or sequelae

GI symptoms

  • Caused by submucosal hemorrhage and edema
  • Intussusception is the most common associated complication (0.4-3.6%), however 60% of the time it is limited to the small bowel
  • Guaiac-positive stool is found in up to 50% of patients

Renal disease

  • Most commonly presents with isolated hematuria
  • Occurs in 20-50% of patients


References:
  1. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.
    Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA,
    Arthritis Rheum. 2013 Jan;65(1):1-11.
  2. Epidemiology of immunoglobulin A vasculitis (Henoch-Schnlein): current state of knowledge.
    Piram M, Mahr A,
    Curr Opin Rheumatol 2013 Mar;25(2):171-8.
  3. Incidence of Henoch-Schnlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins.
    Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR,
    Lancet 2002 Oct;360(9341):1197-202.
  4. A nationwide survey on epidemiological characteristics of childhood Henoch-Schnlein purpura in Taiwan.
    Yang YH, Hung CF, Hsu CR, Wang LC, Chuang YH, Lin YT, Chiang BL,
    Rheumatology (Oxford) 2005 May;44(5):618-22.
  5. Incidence of IgA vasculitis in the adult Slovenian population.
    Hocevar A, Rotar Z, Ostrovrnik J, Jurcic V, Vizjak A, Dolenc Voljc M, Lindic J, Tomic M,
    Br. J. Dermatol. 2014 Sep;171(3):524-7.
  6. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature.
    Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, De Martino M,
    Semin. Arthritis Rheum. 2005 Dec;35(3):143-53.
  7. Henoch-Schnlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study.
    Calvio MC, Llorca J, Garca-Porra C, Fernndez-Iglesias JL, Rodriguez-Ledo P, Gonzlez-Gay MA,
    Medicine (Baltimore) 2001 Sep;80(5):279-90.
  8. Anaphylactoid purpura nephritis in childhood: natural history and immunopathology.
    Levy M, Broyer M, Arsan A, Levy-Bentolila D, Habib R,
    Adv. Nephrol. Necker Hosp. 1976;6:183-228.
  9. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schnlein purpura.
    Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG, Arend WP, Edworthy SM, Fauci AS, Leavitt RY, Lie JT,
    Arthritis Rheum. 1990 Aug;33(8):1114-21.
  10. The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis.
    Calabrese LH, Michel BA, Bloch DA, Arend WP, Edworthy SM, Fauci AS, Fries JF, Hunder GG, Leavitt RY, Lie JT,
    Arthritis Rheum. 1990 Aug;33(8):1108-13.
  11. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides.
    Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, Kawasaki T, Lindsley C, Petty RE, Prieur AM, Ravelli A, Woo P,
    Ann. Rheum. Dis. 2006 Jul;65(7):936-41.
  12. EULAR/PRINTO/PRES criteria for Henoch-Schnlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.
    Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N, Ruperto N,
    Ann. Rheum. Dis. 2010 May;69(5):798-806.
  13. Clinical course of extrarenal symptoms in Henoch-Schonlein purpura: a 6-month prospective study.
    Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hltt T, Jahnukainen T, Rajantie J, Orml T, Nuutinen M,
    Arch. Dis. Child. 2010 Nov;95(11):871-6.
  14. Intra-abdominal manifestations of Henoch-Schnlein purpura.
    Choong CK, Beasley SW,
    J Paediatr Child Health 1998 Oct;34(5):405-9.
  15. Contrast enema in children with Henoch-Schnlein purpura.
    Schwab J, Benya E, Lin R, Majd K,
    J. Pediatr. Surg. 2005 Aug;40(8):1221-3.
  16. Age of onset as a risk factor of renal involvement in Henoch-Schnlein purpura.
    Ghrahani R, Ledika MA, Sapartini G, Setiabudiawan B,
    Asia Pac Allergy 2014 Jan;4(1):42-7.
  17. Henoch-Schnlein purpura and systemic disease in children: retrospective study of clinical findings, histopathology and direct immunofluorescence in 34 paediatric patients.
    Johnson EF, Lehman JS, Wetter DA, Lohse CM, Tollefson MM,
    Br. J. Dermatol. 2015;172(5):1358-63.

2 Laboratory findings

The laboratory findings in HSP are nonspecific. However, some labs may be useful in evaluation of the potential complications of the disease. Serum IgA levels are elevated in 50-70% of patients, but are rarely nessessary in the diagnosis of HSP.

Evaluation of anemia, thrombocytopenia, other causes of purpura (e.g. coagulopathy) and signs of renal disease are potentially useful.



References:
  1. Henoch-Schnlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study.
    Calvio MC, Llorca J, Garca-Porra C, Fernndez-Iglesias JL, Rodriguez-Ledo P, Gonzlez-Gay MA,
    Medicine (Baltimore) 2001 Sep;80(5):279-90.
  2. Elevated serum IgA globulin in anaphylactoid purpura.
    Trygstad CW, Stiehm ER,
    Pediatrics 1971 Jun;47(6):1023-8.
  3. What is the difference between IgA nephropathy and Henoch-Schnlein purpura nephritis?
    Davin JC, Ten Berge IJ, Weening JJ,
    Kidney Int. 2001 Mar;59(3):823-34.
  4. Henoch-Schnlein purpura with hypocomplementemia.
    Lin Q, Min Y, Li Y, Zhu Y, Song X, Xu Q, Wang L, Cheng J, Feng Q, Li X,
    Pediatr. Nephrol. 2012 May;27(5):801-6.

3 Pain in HSP

Although it does not change the clinical course of HSP, glucocorticoid therapy does shortens the duration of severe abdominal pain. It should be noted however that this only affects the inflammatory part of the disease and therefore may obscure abdominal complications of disease. Given the timing of the disease steroids usually need to be tapper over 1-2 months from the start of treatment.

NSAIDs are the drug of choice for symptomatic relief in patients with mild-moderate pain. However, if the patient has active gastrointestinal bleeding or glomerulonephritis they may be contraindicated.



References:
  1. Effects of corticosteroid on Henoch-Schnlein purpura: a systematic review.
    Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C,
    Pediatrics 2007 Nov;120(5):1079-87.
  2. Steroid effects on the course of abdominal pain in children with Henoch-Schonlein purpura.
    Rosenblum ND, Winter HS,
    Pediatrics 1987 Jun;79(6):1018-21.
  3. Use of intravenous hydrocortisone in Henoch-Schonlein purpura.
    Leung SP,
    J Paediatr Child Health 2001 Jun;37(3):309-10.
  4. Anaphylactoid purpura in children (Schonlein-Henoch syndrome): review with a follow-up of the renal complications.
    ALLEN DM, DIAMOND LK, HOWELL DA,
    AMA J Dis Child 1960 Jun;99:833-54.
  5. Gastrointestinal and renal involvement in vasculitis: management strategies in Henoch-Schnlein purpura.
    Szer IS,
    Cleve Clin J Med 1999 May;66(5):312-7.
  6. Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP).
    Dudley J, Smith G, Llewelyn-Edwards A, Bayliss K, Pike K, Tizard J,
    Arch. Dis. Child. 2013 Oct;98(10):756-63.
  7. Pitfalls in recommending evidence-based guidelines for a protean disease like Henoch-Schnlein purpura nephritis.
    Davin JC, Coppo R,
    Pediatr. Nephrol. 2013 Oct;28(10):1897-903.

4 Renal disease in HSP

20-50% of children with HSP will go on to develop early renal involvement. Of those 90% do so within 2 months and 97% within 6 months. Most patients with renal involvment will improve with no intervention. Patients at risk for long term complications (proteinuria of >1 g/day, nephrotic syndrome, etc.) should be evaluated by a nephrologist and considered for pulse dose steroids and a prolonged steroid taper.



References:
  1. Circulating IgA-immune complexes in Henoch-Schnlein purpura. A longitudinal study of their relationship to disease activity and vascular deposition of IgA.
    Kauffmann RH, Herrmann WA, Meer CJ, Daha MR, Van Es LA,
    Am. J. Med. 1980 Dec;69(6):859-66.
  2. Berger disease: Henoch-Schnlein syndrome without the rash.
    Meadow SR, Scott DG,
    J. Pediatr. 1985 Jan;106(1):27-32.
  3. Henoch-Schnlein purpura in adulthood and childhood: two different expressions of the same syndrome.
    Blanco R, Martnez-Taboada VM, Rodrguez-Valverde V, Garca-Fuentes M, Gonzlez-Gay MA,
    Arthritis Rheum. 1997 May;40(5):859-64.
  4. Henoch-Schnlein purpura: clinical presentation.
    Cameron JS,
    Contrib Nephrol 1984;40:246-9.
  5. Henoch-Schnlein purpura in children. Report of 100 patients and review of the literature.
    Saulsbury FT,
    Medicine (Baltimore) 1999 Nov;78(6):395-409.
  6. Renal manifestations of Henoch-Schonlein purpura in a 6-month prospective study of 223 children.
    Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hltt T, Jahnukainen T, Rajantie J, Orml T, Turtinen J, Nuutinen M,
    Arch. Dis. Child. 2010 Nov;95(11):877-82.
  7. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature.
    Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, De Martino M,
    Semin. Arthritis Rheum. 2005 Dec;35(3):143-53.
  8. Renal manifestations in Henoch-Schnlein purpura: a 10-year clinical study.
    Chang WL, Yang YH, Wang LC, Lin YT, Chiang BL,
    Pediatr. Nephrol. 2005 Sep;20(9):1269-72.
  9. Long term renal prognosis of Henoch-Schnlein purpura in an unselected childhood population.
    Stewart M, Savage JM, Bell B, McCord B,
    Eur. J. Pediatr. 1988 Feb;147(2):113-5.
  10. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review.
    Narchi H,
    Arch. Dis. Child. 2005 Sep;90(9):916-20.
  11. Henoch-Schnlein nephritis in children and adults. Morphological features and clinicopathological correlations.
    Rieu P, Nol LH,
    Ann Med Interne (Paris) 1999 Feb;150(2):151-9.
  12. Pitfalls in recommending evidence-based guidelines for a protean disease like Henoch-Schnlein purpura nephritis.
    Davin JC, Coppo R,
    Pediatr. Nephrol. 2013 Oct;28(10):1897-903.

5 CNS manifistations



References:
  1. Neurologic manifestations of Schoenlein-Henoch purpura: report of three cases and review of the literature.
    Belman AL, Leicher CR, Mosh SL, Mezey AP,
    Pediatrics 1985 Apr;75(4):687-92.
  2. Henoch-Schonlein purpura with intracerebral haemorrhage.
    Misra AK, Biswas A, Das SK, Gharai PK, Roy T,
    J Assoc Physicians India 2004 Oct;52:833-4.
  3. Ataxia and peripheral neuropathy: rare manifestations in Henoch-Schnlein purpura.
    Bulun A, Topaloglu R, Duzova A, Saatci I, Besbas N, Bakkaloglu A,
    Pediatr. Nephrol. 2001 Dec;16(12):1139-41.
  4. Henoch-Schnlein Purpura with Posterior Reversible Encephalopathy Syndrome.
    Stefek B, Beck M, Ioffreda M, Gardner L, Stefanski M,
    J. Pediatr. 2015 Nov;167(5):1152-4.