Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and churg‐strauss syndrome patients with factors predicting poor prognosis

Abstract

Objective. To define the most effective treatment for severe polyarteritis nodosa (PAN) and ChurgStrauss syndrome (CSS) and to investigate the indication for plasma exchange treatment.Methods. We conducted a prospective, randomized, multicenter trial in which 62 patients were randomly assigned to receive either prednisone plus cyclophosphamide (intravenous bolus) (group A; n = 28) or prednisone plus cyclophosphamide (intravenous bolus) plus plasma exchanges (group B; n = 34) as first-line treatment for severe PAN or CSS. Factors predicting poor prognosis were renal symptoms, gastrointestinal tract involvement, cardiomyopathy, central nervous system involvement, weight loss >lo% of body weight, and age >SO years oid. Patients with hepatitis B virusrelated PAN were not included in this study. The end point of the study was control of the disease (recovery or remission) or death.

Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: A prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty‐five patients

“…In fact, the most frequent side effects were attributable to CS, e.g., fractures and osteonecrosis necessitating surgery were observed. Compared to previous studies (10,17), even though we reduced the total CS dose in this protocol by maintaining an initial dose of 1 mg/kg/day of prednisolone for only 3 weeks, side effects did not disappear. We now recommend the systematic prescription of bisphosphonates and increased calcium and vitamin D intake from the time of the first CS dose and we hope to reduce the number of osteoporotic complications.…”

“…However, the mean creatininemia was higher in the 12-pulse group. Although that difference was not statistically significant, this finding was a matter of concern because of the known pejorative prognostic value on outcome of renal dysfunction in PAN and MPA (17). Given the very similar median creatininemia values for the two groups, this difference probably reflects the inclusion of 2 anuric patients in the 12-pulse group.…”

“…There is nonetheless some evidence coming from previous trials that both diseases respond to the same treatment, at least to induce the remission (8,10,17). We consequently attempted to evaluate the long-term effects of therapy separately for PAN and MPA.…”

Wegener Granulomatosis: Case Report and Brief Literature Review

“…27,28 There is not much clinical data to address the long-term outcome in patients who undergo plasmapheresis for WG. 29,30 In our case, the patient began therapy with monthly intravenous cyclophosphamide pulse therapy. The use of monthly intravenous pulse therapy is primarily to lower the overall cumulative dose of cyclophosphamide and thereby avoid some of the toxic side effects of the oral regimen.…”

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

“…Bei der EGPA scheint die zur Standardinduktionstherapie zusätzlich durchgeführte Plasmaseparation nicht wirksam zu sein [62][63][64]. Jedoch ist die schwergradige RPGN, die bei GPA und MPA eine mögliche Indikation zur Plasmaseparation darstellt, bei der EGPA nicht gezielt untersucht worden.…”