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Diagnosis: Henoch-Schönlein purpura

Henoch-Schönlein purpura (HSP) (or Schönlein-Henoch purpura, anaphylactoid purpura, rheumatic purpura, or allergic vasculitis) is an inflammatory disorder considered a form of systemic vasculitis with IgA-dominant immune deposits affecting small vessels of the skin, GI tract, kidneys, joints, and, rarely, other organs. It is the most common vasculitis in children.

Our case ilustrates a common clinical presentation in children although this case developed in an adult-young woman. Renal affectation was mild, with only microscopic hematuria and non-nephrotic proteinuria. The kidney prognosis is excellent in most patients. However some patients will have persistent protein in their urine, high blood pressure, and/or renal insufficiency.

The syndrome takes its name from 2 German physicians: Johan Schönlein (he described a case in 1837) and Edouard Henoch (a former student of Schönlein who described several cases many years later).

HSP is thought to be an immunoglobulin A (IgA)–mediated autoimmune phenomenon. An unknown antigenic stimulant has been postulated to cause a rise in IgA. The antigen-antibody complexes deposit locally throughout the body and activate pathways leading to necrotizing vasculitis. The etiology of HSP is thought to be multifactorial with genetic, environmental, and antigenic components. Many patients report antecedent upper-respiratory or GI infections. Cases of HSP also have been reported after drug ingestions and vaccinations.

Clinical presentation include a characteristic rash, migratory polyarthritis, renal involvement, and GI involvement. After a prodrome, a rash, abdominal pain, peripheral edema, vomiting and/or arthritis develop. The rash appears in 100% of patients and is the presenting feature in 50%. Lesions consist of erythematous macules, urticarial papules, pruritic papules, and plaques. Skin lesions tend to appear in lower extremities, lower abdomen, and buttocks). Eruptions usually last an average of 3 weeks. As many as 85% of patients will have GI symptoms, including abdominal pain, nausea, and vomiting. The most common symptom is colicky abdominal pain. Joint involvement is present in 75% of reported patients with HSP and the presenting sign in approximately 25%. The large joints (more commonly knees and ankles) with pain and edema can be the only symptoms. Renal involvement is present in 30-50% of patients and may persist as long as 6 months after the onset of the rash. Renal involvement manifests in a range from mild hematuria or proteinuria to oliguria and renal failure. Permanent renal impairment is seen in 20% of patients who have nephrotic or nephritic syndrome.

Even though the symptoms of HSP make it easier to diagnose in children, confirmation of the diagnosis of HSP requires evidence of tissue deposition in the skin or kidney of IgA immunoglobulin. Renal biopsy is another method to establish the diagnosis, but is reserved for patients in whom the diagnosis is uncertain or in whom there is evidence of more severe renal involvement.

The overall outcome is good in most patients. All of the manifestations of active HSP usually resolve spontaneously, although recurrent episodes of skin rash and hematuria may be seen. Among those with kidney involvement, only a minority have persistent disease. The kidney prognosis is excellent in most patients. However some patients will have persistent protein in their urine, high blood pressure, and renal insufficiency. It is estimated that HSP accounts for approximately 3% of cases of end-stage kidney disease in children. Poor renal prognosis is more common among those with the nephrotic syndrome, renal insufficiency, and more advanced findings on biopsy. About 50% of patients have at least 1 recurrence. Patients <3 years-old usually have an improved prognosis. The prognosis is best for patients with minimal or no renal involvement at the onset of the illness (Dyne P, DeVore HK. Henoch-Schönlein Purpura. In: eMedicine, consulted August 23rd 2007. [Link: www.emedicine.com/emerg/topic767.htm]). Between 20 and 50 percent of children with HSP develop some kidney problems, but only 1% progress to total kidney failure. Progression to kidney failure may take as long as 10 years.

Recurrences are common, occurring in approximately one-third of patients. Since complete recovery occurs in 94% of children and 89 % of adults, respectively, most patients receive no specific therapy. There is suggestive evidence that corticosteroids enhance the rate of resolution of the arthritis and abdominal pain, although they do not appear to prevent recurrent disease. Specific treatment is recommended in patients with marked proteinuria and/or impaired kidney function during the acute episode. A kidney biopsy can be performed to reveal the severity of the lesions which appears to be the best indicator of prognosis. Advanced disease, usually defined as crescentic nephritis, is treated with a regimen consisting of pulse intravenous methylprednisolone followed by oral prednisone. Other regimens include azathioprine, cyclophosphamide, and plasmapheresis, however, since spontaneous recovery is often observed in these patients, it remains unknown whether these regimens are superior to no or less aggressive therapy (Anonymus. Henoch-Schönlein Purpura. In: Vasculitis Foundation. Consulted August 23rd 2007. [Link: www.vasculitisfoundation.org/HenochSchonleinpurpura]).

Kidney transplantation can be performed in those patients who progress to end-stage kidney disease, although recurrent disease can occur. This appears to be more likely in patients with aggressive initial disease who progressed to end-stage kidney disease in less than three years after the onset of HSP. Therefore it is recommended the transplantation to be delayed for 12-24 months after the disappearance of the rash. Some observations suggest that the risk of recurrent disease also may be higher in living-related donors.

See the chapter IgA nephropathy and Henoch-Schönlein purpura (English version).

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Bibliography

• Shenoy M, Bradbury MG, Lewis MA, Webb NJ. Outcome of Henoch-Schonlein purpura nephritis treated with long-term immunosuppression. Pediatr Nephrol. 2007 Jul 24; [Epub ahead of print]. [PubMed link]
• Butani L, Morgenstern BZ. Long-term outcome in children after Henoch-Schonlein purpura nephritis. Clin Pediatr (Phila). 2007 Jul;46(6):505-11. [PubMed link]
• de Almeida JL, Campos LM, Paim LB, Leone C, Koch VH, Silva CA. Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of initial prognostic factors. J Pediatr (Rio J). 2007 May-Jun;83(3):259-66. [PubMed link] [Free ful text]
• Dillon MJ. Henoch-Schonlein purpura: recent advances. Clin Exp Rheumatol. 2007 Jan-Feb;25(1 Suppl 44):S66-8. [PubMed link]
• Coppo R, Andrulli S, Amore A, Gianoglio B, Conti G, Peruzzi L, Locatelli F, Cagnoli L. Predictors of outcome in Henoch-Schonlein nephritis in children and adults. Am J Kidney Dis. 2006 Jun;47(6):993-1003. [PubMed link]
• Shrestha S, Sumingan N, Tan J, Alhous H, McWilliam L, Ballardie F. Henoch Schonlein purpura with nephritis in adults: adverse prognostic indicators in a UK population. QJM. 2006 Apr;99(4):253-65. [PubMed link]
• Dyne P, DeVore HK. Henoch-Schönlein Purpura. In: eMedicine, consulted August 23rd 2007. [Link: www.emedicine.com/emerg/topic767.htm]
• Anonymus. Henoch-Schönlein Purpura. In: Vasculitis Foundation. Consulted August 23rd 2007. [Link: www.vasculitisfoundation.org/HenochSchonleinpurpura]
  

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