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Diagnosis: Schönlein-Henoch purpura.

Schönlein-Henoch purpura (SHP) is now classified as a form of systemic vasculitis with IgA-dominant immune deposits affecting small vessels and typically involving skin, gut, and glomeruli and associated with arthralgias or arthritis, although only the skin is involved in 100% of cases. 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]). Henoch in 1868 reported the first case of a patient with colic, bloody diarrhea, painful joints, and a rash.

The disease is also known as allergic purpura, anaphylactoid purpura, and Schonlein-Henoch syndrome.

SHP may occur at any age, but is primarily a disease of children, with most affected children presenting before the age of 10. In both children and adults, males are affected more often than females, with a male/female ratio near to 1.9:1. As with IgA nephropathy, SHP has been reported to be less common in African-Americans than in Caucasians.

The purpuric lesions usually are concentrated on the lower extremities and buttocks, although such lesions may be seen on arms, face, and other sites. Arthritis, the second most common manifestation of SHP, routinely involves joints of the lower extremities (knees, ankles, feet) and involves the upper extremities in approximately one third of cases. Gastrointestinal (GI) involvement, manifest as colicky abdominal pain, vomiting, and/or occult or overt GI bleeding, occurs in 50% to 75% of cases. Any portion of the bowel may be involved. Potentially serious complications of GI involvement in SHP include bowel infarction, perforation, and intussusception. Arthritis and/or GI symptoms often precede the purpuric rash of SHP by up to 2 weeks. These symptoms in most cases resolve within 8 weeks, although recurrences are not uncommon. Most recurrences present within 4 months of the original illness and tend to be milder and of shorter duration. The manifestation of SHP that may become chronic and ultimately results in the greatest morbidity is nephritis. In different series, the incidence of renal involvement (defined by the presence of hematuria) in children with SHP 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).

The etiology of SHP is unclear. It is thought to be multifactorial with genetic, environmental, and antigenic components. Most patients report antecedent upper-respiratory, pharyngeal, or GI infections. Multiple bacterial and viral infectious agents have been associated with the development of SHP, and cases of SHP also have been reported after drug ingestions and vaccinations.

SHP is an acute self-limited illness and usually resolves without treatment, but may rarely lead to complications. Initial attacks of Henoch-Schonlein purpura can last several months. One third of patients have one or more recurrences. Children younger than 3 years have a shorter, milder course and fewer recurrences. The long-term prognosis of Henoch-Schönlein purpura is directly dependent on the severity of renal involvement. The clinical presentation of SHP is more severe in older adults and the renal prognosis for HSP nephritis is poor compared with what is observed in series in children (Pillebout E, et al. Henoch-Schönlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002 May;13(5):1271-8. [PubMed link] [Free full text]).

Patients with renal disease were more likely to have recurrences. In two unselected series chronic renal disease developed in up to 1.4% of children at about eight years from diagnosis. Mortality is also very unusual occurring in <1% in one series (Stewart M, et al. Long term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population. Eur J Pediatr 1988; 147:113-15 [PubMed link]; Koskimies O, et al. Henoch-Schönlein nephritis: long term prognosis of unselected patients. Arch Dis Child 1981; 56: 482–4. [PubMed link][Free full text]). Although no feature is absolutely predictive of renal outcome many studies confirm that features including nephrotic syndrome, renal impairment, hypertension and decreased Factor XIII at presentation are associated with a poorer prognosis.

In a follow-up study of 78 children who had had SHP the severity of the clinical presentation and the renal biopsy were related to final outcome but nothing reliably predicted individual outcome.

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

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Bibliography

• Sanders JT, Wyatt RJ. IgA nephropathy and Henoch-Schönlein purpura nephritis. Curr Opin Pediatr. 2008;20:163-70. [PubMed link]
• Tizard EJ, Hamilton-Ayres MJ. Henoch Schonlein purpura. Arch Dis Child Educ Pract Ed. 2008;93:1-8. [PubMed link][Free full text]
• Roberts PF, Waller TA, Brinker TM, Riffe IZ, Sayre JW, Bratton RL. Henoch-Schönlein purpura: a review article. South Med J. 2007;100:821-4. [PubMed link]
• Shrestha S, Sumingan N, Tan J, Alhous H, McWilliam L, Ballardie F. Henoch Schönlein purpura with nephritis in adults: adverse prognostic indicators in a UK population. QJM. 2006;99:253-65. [PubMed link][Free full text]
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  Recurrence of IgA nephropathy and Henoch-Schönlein purpura after kidney transplantation: risk factors and graft survival. Transplant Proc. 2005;37:3705-9. [PubMed link]
• Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study. Lancet. 2002;360:666-70. [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;13:1271-8. [PubMed link] [Free full text]
• 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;12:2277-83. [PubMed link][Free full text]
• Goldstein AR, White RHR, Akuse R, et al. Long term follow-up of childhood Henoch-Schönlein nephritis. Lancet 1992; 339: 280–2. [PubMed link]

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