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Treatment of polyarteritis nodosa and Churg-Strauss syndrome : indications of plasma exchanges . To define the most effective treatment for polyarteritis nodosa ( PAN ) and Churg-Strauss syndrome ( CSS ) , we undertook 4 consecutive prospective therapeutic trials including 236 patients and tried to answer several important questions : Should cyclophosphamide ( CYC ) be given as the first-line treatment ? What is the place of plasma exchanges ( PE ) in the treatment of systemic vasculitis ? and does hepatitis B virus ( HBV ) related PAN require treatment ? Our first randomized trial in 71 patients ( 1981-1983 ) compared the association of CYC with corticosteroids ( CS ) and PE to CS and PE , in order to evaluate the efficacy of CYC given as the first-line treatment to control disease activity and subsequent survival of PAN and CSS patients . Between December 1983 and December 1988 , we conducted two trials simultaneously : one aimed at patients without HBV markers and the second at patients with HBV markers . In 78 patients without HBV markers , we compared prednisone and PE to prednisone alone as the initial therapeutic regimen . In 33 patients with PAN related to HBV , a new therapeutic strategy was applied as an alternative to long-term steroid and immunosuppressive therapy : short-term steroid therapy and PE were used to control the evolution of PAN and anti-viral therapy was administered to suppress the etiological agent of the vasculitis . In the last protocol including 56 patients and addressed to severe PAN without HBV markers or CSS we have shown that PE did not improve the prognosis and control of the disease . Twelve years after the beginning of the trials on PAN and CSS patients , we think that the therapeutic strategy should be as follows : In PAN without HBV and CSS : prednisone in association with CYC improves the control of the disease despite infectious side effects which may be reduced by better CYC dose adaptation . In PAN related to HBV : The first-line treatment should be the association of anti-viral agents and PE . This treatment was effective and cured a majority of patients within 2 to 3 months ; half of them seroconverted . The length of HBV infection before its diagnosis , delay before initiation of treatment and previous immunosuppressive therapy led to a poor seroconversion rate . The role of PE in the treatment of systemic necrotizing vasculitis : PE are obviously useful in PAN related to HBV where immune complex deposition has been demonstrated . When PAN is not related to HBV and in CSS , even in severe cases , there is presently no argument supporting systematic administration of PE at the time of diagnosis .
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Intervention_Pharmacological
cyclophosphamide
412
Intervention_Pharmacological
plasma exchanges
413
Intervention_Pharmacological
prednisone and PE
414
Intervention_Pharmacological
prednisone
415
Intervention_Pharmacological
steroid and immunosuppressive therapy
416
Intervention_Pharmacological
short-term steroid therapy and PE
417
Intervention_Pharmacological
anti-viral therapy
418
Intervention_Pharmacological
prednisone in association with CYC
419
Intervention_Pharmacological
anti-viral agents and PE
420
Intervention_Pharmacological
PE
421
Intervention_Pharmacological
PE
422
Intervention_Pharmacological
PE
423
Outcome_Other
efficacy
424
Outcome_Mortality
subsequent survival
425
Outcome_Physical
evolution of PAN
426
Outcome_Physical
etiological agent of the vasculitis .
427
Outcome_Physical
prognosis
428
Outcome_Physical
control of the disease
429
Outcome_Physical
control of the disease
430
Outcome_Adverse-effects
infectious side effects
431
Outcome_Other
effective
432
Outcome_Physical
length of HBV infection
433
Outcome_Physical
seroconversion rate .
434
Participant_Condition
polyarteritis nodosa
435
Participant_Condition
Churg-Strauss syndrome
436
Participant_Condition
polyarteritis nodosa
437
Participant_Condition
PAN
438
Participant_Condition
Churg-Strauss syndrome
439
Participant_Condition
CSS
440
Participant_Sample-size
236
441
Participant_Sample-size
71
442
Participant_Condition
without HBV markers
443
Participant_Condition
with HBV markers
444
Participant_Sample-size
78
445
Participant_Condition
without HBV markers
446
Participant_Sample-size
33
447
Participant_Condition
PAN
448
Participant_Condition
HBV
449
Participant_Sample-size
56
450
Participant_Condition
severe PAN without HBV markers
451
Participant_Condition
PAN