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Article Review/Hyperlink
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Jayne DRW, Rasmussen N
Treatment of ANCA-associated systemic vasculitis: initiatives
of the European Community Systemic Vasculitis Clinical Trials Study
Group (ECSYSVASTRIAL)
Mayo Clin Proc
(Aug) 72:737-747 1997

Many in the United States are not aware of the ECSYSVASTRIAL going on in
Europe. This is
a tremendous project, designed to evaluate different forms of therapy in many
types of
vasculitis. The ECSYSVASTRIAL is the Multicenter European Study on Renal
Vasculitis.
The Pathology
Goup is coordinated by Dr. Franco Ferrario from Milan, Italy. The
pathologists involved
in the trial have posted an interesting catalogue of
Renal Morphological Lesions in ANCA-Associated Vasculitis. There
are three
"pages" of annotated figures, 21 in all, taken from actual patients in the
trial. Each
image is accompanied by a very brief commentary. The home page for the
ECSYSVASTRIAL as a whole
is still
being developed.
This paper is a wonderful summary of the various trials, how vasculitis is
defined, the
role of ANCA, etc. The article is useful for those not interested in
clinical trials, as
it specifies the "best standard" treatment regimens for various phases of
vasculitis. For
a definition of the various types of vasculitis, see the
talk (to be posted officially next month) by Dr. Charles Jennette on
HDCN.
In a slightly confusing terminology, they refer to AASV (ANCA-associated
systemic
vasculitis) to include both ANCA + and ANCA - vasculitis, and even localized
lesions. The
primary grouping of syndromes is clinical, and this determines the various
trial designs.
The trials are as follows:
OCS = oral corticosteroids, CYC = oral cyclophosphamide, AZA =
azathioprine
ECSYSVASTRIAL Early Protocol Group |
Subgroup | Name | Regimens |
Localized
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MAYO
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For patients with disease limited to the respiratory tract: The standard arm
is OCS alone,
compared to trimethoprim-sulfamethoxazole for 0-12 months.
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Early systemic
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NORAM
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For patients with non-renal disease: OCS/CYC vs. OCS/MTX. The study compares
the ability
to substitute MTX for CYC, hopefully reducing toxicity.
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Generalized
Scr < 500 µM
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CYCAZAREM
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OCS/CYC for 12-18 mos vs. OCS/CYC for 3 mos, OCS/AZA 3-18 mos.
In patients with mild renal disease, studies ability to substitute AZA for
CYC after 3
months.
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Severe renal
Scr > 500 µM
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MEPEX
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All get OCS/CYC for 6 mos. Study compares adjuvant effect of plasma exchange
(standard)
vs. pulse medrol (alternative).
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Refractory
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WARCRY
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Antithymocyte globulin (standard) vs. anti-CD52 and anti-CD4 humanized
monoclonal
antibodies.
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But there's more! The above are the standard arms. Newer trials are also
being planned:
ECSYSVASTRIAL Newer Treatments |
Subgroup | Name | Regimens |
Early systemic or
generalized, Scr < 150 µM
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IVISTAT
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The standard arm is OCS + CYC for 3 months, then OCS+AZA: Trial compares
adjuvant IVIg or
trimethoprim-sulfamethoxasole, or both.
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Scr >150 µM plus non-renal,
or Scr >500 µM (severe renal)
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CYCLOPS
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Both arms use OCS + CYC for 6 mos, then CYC is switched to AZA; trial
compares oral CYC
with pulse IV CYC during the first 6 mos.
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Remission of early systemic
or generalized disease with
Scr > 50 ml/min
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MUPIBAC
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Compares mupirocin vs placebo nasal ointment; outcome is ability to maintain
remission
from 18-42 mos
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Remission of generalized
or severe renal disease
with Scr < 50 ml/min
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REMAIN
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In disease in remission at 18 mos, the question is whether to continue
OCS/AZA for 4 years
or to stop it by 2 years post onset.
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Comment: Additional useful information in this artice is prednisonse,
cyclophosphamide, azathioprine, and methotrexate regimens for both standard
therapy and to
treat exacerbations. Much of this information SHOULD be on the ECSYSVASTRIAL
website!
(John T. Daugirdas, M.D., University of Illinois at Chicago)
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