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Diagnosis: IgA nephropathy (plus acute tubular necrosis, and changes suggesting initial diabetes nephropathy) associated to cutaneous vasculitis: Schönlein-Henoch purpura

Schönlein-Henoch (S-H) purpura is a disease characterized by acute nephritis associated to purpuric skin lesions, mainly in lower extremities and buttocks, migratory arthritis, and gastrointestinal hemorrhage. It is also known as S-H syndrome, S-H nephritis, anaphylactoid purpura, and rheumatoid purpura. Named for the German physicians Johann Lukas Schönlein (1793-1864) and Eduard Heinrich Henoch (1820-1910). Some authors think that Schönlein's name should come first because he was the first person to recognize the condition (in 1837) (although the condition appears to have first been described by Heberden in 1801 [Jennette JC, et al. Heptinstall's Pathology of the Kidney. 6th ed. Wolters Kluwer Lippincott Williams & Wilkins, Philadelphia, 2007]).

The syndrome is considered a form of IgA nephropathy with prominent extrarrenal involvement: systemic vasculitis mediated by IgA rich immune complexes. The etiology is unknown.

S-H purpura may occur at any age, but is primarily a disease of children; there are few reports of case series on S-H purpura in the elderly (Diehl MP, et al. Elderly-onset Henoch-Schonlein purpura: a case series and review of the literature. J Am Geriatr Soc. 2008;56(11):2157-9. [PubMed link] [Free full text])

The reported incidence of renal involvement in patients with S-H purpura varies considerably between different studies. In different series, the incidence of renal involvement (defined by the presence of hematuria) in children with S-H purpura is 20% to 56% (overall 32%), whereas in adults it is 49% to 78% (overall 59%) (Jennette JC, et al. Heptinstall's Pathology of the Kidney. 6th ed. Wolters Kluwer Lippincott Williams & Wilkins, Philadelphia, 2007).

Most though not all studies of adults with S-H nephritis have reported a worse prognosis: a high long-term risk of renal dysfunction (Hung SP, et al. Clinical manifestations and outcomes of Henoch-Schönlein purpura: comparison between adults and children. Pediatr Neonatol. 2009;50(4):162-8. [PubMed link]; Fogazzi GB, et al.. Long-term outcome of Schönlein-Henoch nephritis in the adult. Clin Nephrol. 1989;31(2):60-6. [PubMed link]; Faull RJ, et al. Adult Henoch-Schönlein nephritis. Aust N Z J Med. 1987;17(4):396-401. [PubMed link]).

See the chapter IgA nephropathy and Schönlein-Henoch purpura of our tutorial.

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Bibliography

• Hung SP, Yang YH, Lin YT, Wang LC, Lee JH, Chiang BL. Clinical manifestations and outcomes of Henoch-Schönlein purpura: comparison between adults and children. Pediatr Neonatol. 2009;50(4):162-8. [PubMed link]
• Diehl MP, Harrington T, Olenginski T. Elderly-onset Henoch-Schonlein purpura: a case series and review of the literature. J Am Geriatr Soc. 2008;56(11):2157-9. [PubMed link] [Free full text]
• Kellerman PS. Henoch-Schönlein purpura in adults. Am J Kidney Dis. 2006;48(6):1009-16. [PubMed link]
• Ilan Y, Naparstek Y. Henoch Schonlein purpura in children and adults: is it one entity? Semin Arthritis Rheum. 2002;32(3):139-40. [PubMed link]
• Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schönlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002;13(5):1271-8. [PubMed link] [Free full text]
• Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD, Flageul B, Morel P, Rybojad M. Schönlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol. 1997;133(4):438-42. [PubMed link]
• Fogazzi GB, Pasquali S, Moriggi M, Casanova S, Damilano I, Mihatsch MJ, Zucchelli P, Ponticelli C. Long-term outcome of Schönlein-Henoch nephritis in the adult. Clin Nephrol. 1989;31(2):60-6. [PubMed link]
• Faull RJ, Aarons I, Woodroffe AJ, Clarkson AR. Adult Henoch-Schönlein nephritis. Aust N Z J Med. 1987;17(4):396-401. [PubMed link]

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