Addition of plasma exchange to glucocorticosteroids for the treatment of severe Henoch-Schönlein purpura in adults: a case series.
BACKGROUND
Adult Henoch-Schönlein purpura (HSP) has been associated with poor outcome and end-stage renal disease in >20% of cases. Although the benefit of adding another immunosuppressant to steroids in severe adult HSP has not been shown, the benefit of plasma exchange (PE) therapy has been poorly evaluated.


METHODS
Case series.


METHODS
11 consecutive patients with severe and newly diagnosed HSP since 1988 who were treated with steroids and PE.


METHODS
Patients' characteristics and outcome were analyzed. Birmingham Vasculitis Activity Score (BVAS), estimated glomerular filtration rate (eGFR), and proteinuria were measured at baseline, at the end of PE treatment, at months 6 and 12, and at the last visit. Side effects of corticoid treatment and PE were recorded.


RESULTS
11 patients were identified in 1988-2010. Patients received intravenous corticoid pulses in 64% of cases, followed by oral prednisone for a median of 6.6 months. They received a median of 12 PE sessions. BVAS, eGFR, and proteinuria improved significantly between baseline and the last PE at a median of 2 months. PE sessions were well tolerated, except in one patient who developed central catheter-associated septicemia. One patient required dialysis therapy 15 days after HSP diagnosis and did not recover kidney function. At the last medical evaluation at a mean follow-up of 6 years, median eGFR and proteinuria were 83 ± 22 mL/min/1.73 m(2) and protein excretion of 140 ± 10 mg/d, respectively. 3 women had pregnancy without complications.


CONCLUSIONS
This case series did not have a control group.


CONCLUSIONS
The combination of PE and corticoid therapy in severe forms of HSP was associated with fast improvement and good long-term outcome.
Department of Nephrology-Dialysis-Transplantation, Angers University Hospital, Angers, France. jfaugusto@chu-angers.fr
Jean-François
JF
Augusto
Johnny
J
Sayegh
Laurence
L
Delapierre
Anne
A
Croue
Fréderic
F
Tollis
Maud
M
Cousin
Jean-François
JF
Subra
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate / Q000502:physiology; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D011695:IgA Vasculitis / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011507:Proteinuria / Q000503:physiopathology; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
D005938:Glucocorticoids