Article

Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria: histological regression possibly associated with cyclosporin A and steroid treatment.

Department of Paediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine, Severance Hospital, Seoul, Korea.
Scandinavian Journal of Rheumatology (impact factor: 2.47). 34(5):392-5. DOI:10.1080/03009740510026544 pp.392-5
Source: PubMed

ABSTRACT To clarify the therapeutic role of cyclosporin A (CyA) for patients with Henoch-Schönlein purpura nephritis (HSPN) showing nephrotic-range proteinuria.
The clinical and histological findings of eight children (7.7+/-3.8 years), who were treated with CyA and prednisolone, were evaluated retrospectively. All underwent a renal biopsy before therapy, and six of the eight patients received a follow-up biopsy after therapy.
The histological grade of the International Study of Kidney Disease in Children (ISKDC) was improved in all six patients who received a follow-up biopsy (pre-therapy, four grade IIIa and two grade IIIb; post-therapy, one grade I and five grade II) and it was statistically significant (p = 0.031). The activity index was significantly decreased after therapy (8.3+/-1.6 vs. 3.5+/-1.5, p = 0.031), and the chronicity index (0.5+/-0.5 vs. 0.7+/-1.0) and tubulointerstitial (TI) scores (1.5+/-1.3 vs. 0.8+/-1.6) did not change. There was a reduction in proteinuria from 3.2+/-2.3 to 0.1+/-0.1 g/m2/day (p = 0.008) and renal function remained normal in all patients after therapy. However, one patient showed CyA-induced nephrotoxicity at a second biopsy. After an average follow-up period of 3.8 years, six patients showed normal urine and renal function, and two showed minor urinary abnormalities.
This study suggests that CyA therapy is effective in reducing proteinuria, which is a known risk factor for the development of renal insufficiency in HSPN and may regress the renal pathology in patients with nephrotic-range proteinuria.

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    Article: Therapy for children with henoch-schonlein purpura nephritis: a systematic review.
    Marco Zaffanello, Milena Brugnara, Massimo Franchini
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    ABSTRACT: Although severe kidney involvement in children with Henoch-Shonlein purpura (HSP) is rarer than that in adults, morbidity should not be underevaluated and follow-up is mandatory. Some drugs are introduced as well-defined treatment options, others can be promising therapeutic alternatives. Therapy of HSP nephritis in children can range from simply steroids to combined immunosuppressant treatments. The prophylactic treatment for renal complication of patients with HSP has been sometimes suggested, but with conflicting results and ultimately not clearly proven. The treatment of overt HSP nephritis includes steroids and other immunosuppressant drugs. Methylprednisolone pulse therapy and prednisone per os are tested drugs. These steroids could be used in combination with other immunosuppressant drugs, such as cyclosporin A and cyclophosphamide. Unfortunately, of these two drugs, only cyclophosphamide is demonstrated as effective in a recent randomized controlled trial. However, since there are insufficient data and unstructured study designs, ACE-I, azathioprine, mycophenolate mofetil, and urokinase need to be more tested in childhood HSP nephritis. In addition to drugs, other techniques are used to treat the severe form of nephritis. Of these, in a multicenter study, plasmapheresis demonstrated efficacy in delaying the progression of kidney disease. However, no convincing studies have been made to date concerning either intravenous immunoglobulin, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP nephritis.
    TheScientificWorldJOURNAL 02/2007; 7:20-30. · 1.66 Impact Factor
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.

Keywords

activity index
 
average follow-up period
 
chronicity index
 
CyA therapy
 
CyA-induced nephrotoxicity
 
eight patients
 
follow-up biopsy
 
grade IIIa
 
Henoch-Schönlein purpura nephritis
 
International Study
 
Kidney Disease
 
known risk factor
 
minor urinary abnormalities
 
nephrotic-range proteinuria
 
renal biopsy
 
renal function
 
retrospectively
 
second biopsy
 
six patients
 
therapeutic role
 

J I Shin