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Intravenous
immunoglobulin (IVIg) therapy in
MPO-ANCA related polyangiitis with
rapidly progressive
glomerulonephritis in Japan.
Muso E, Ito-Ihara T, Ono T, Imai
E, Yamagata K, Akamatsu A, Suzuki K.
Division of Nephrology, Kitano
Hospital, The Tazuke Kofukai Medical
Research Institute, Osaka, Japan.
muso@kitano-hp.or.jp
For 30 myeloperoxidase (MPO)
antineutrophil cytoplasmic antibody
(ANCA) related rapidly progressive
glomerulonephritis patients (male
17, female 13, average age of 68 +/-
11.8 years old), intravenous
immunoglobulin (IVIg) (400
mg/kg/day) was administered for 5
consecutive days before or along
with conventional immunosuppressive
therapy in Japan. Twenty patients
were treated with IVIg before the
start or newly increase of
conventional therapy and evaluated
the independent effect of this
therapy. In these patients, just
after IVIg, significant decrease of
CRP from 8.61 +/- 5.77 to 5.47 +/-
4.50 mg/dl (P < 0.001) was noted
with improvement of elevated serum
creatinine in 12 out of 19 patients
(63%). In the analysis of the
overall outcome of 30 patients, at 3
months after IVIg and following
conventional therapy, no patients
showed renal death except 4 for whom
hemodialysis had been started before
IVIg. At 6 months, renal survival
rate were 92% (newly renal death 2
out of 26) and 2 patients died due
to cerebral bleeding (survival rate
was 93%). No fatal infection was
noted. IVIg might be the potent
inducible therapy which can be
promoted before the beginning of
conventional immunosuppressant
treatment for relatively aged and
lower immunopotent MPO-ANCA patients
in Japan.
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Nippon Jinzo Gakkai Shi.
2004 Feb;46(2):79-83. |
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[Successful treatment
of severe ANCA-associated RPGN with
high-dose IVIg therapy]
Gomada M, Akamatsu A.
Division of Nephrology, Ehime
Prefectural Central Hospital, Ehime,
Japan.
We report a case of anti-neutrophil
cytoplasmic antibody(ANCA)-associated
rapid progressive glomerulonephritis (RPGN)
that was treated with intravenous
immunoglobulin (IVIg) therapy. A
37-year-old woman was admitted to our
hospital because of a low-grade fever,
general malaise, and a poor appetite. At
the time of admission, her renal
function had severely deteriorated
(serum creatinine level 9.5 mg/dl; mean
Ccr 3.3 ml/min) and she had severe
anemia (Hb 6.6 g/dl). An immunological
examination revealed the presence of
ANCA-associated RPGN. A biopsy confirmed
a diagnosis of pauci-immune-type
necrotizing crescentic
glomerulonephritis. After initial
treatment with steroid pulse therapy (methylprednisolone,
1,000 mg/day x 3 days), her general
condition deteriorated and two sessions
of hemodialysis were required. On the
10th hospital day, a high dose of
immunoglobulin was administered
intravenously (IVIg 400 mg/kg/day x 5
days). This therapy immediately improved
her general condition and lowered her
serum titer of MPO-ANCA and her serum
creatinine level. After two IVIg
treatments, her MPO-ANCA titer returned
to a normal level and her serum
creatinine level improved from 9.5 mg/dl
to 3.3 mg/dl. A second biopsy confirmed
clinical improvement. These findings
suggest that IVIg therapy is effective
for cases of ANCA-associated
glomerulonephritis that are difficult to
treat using conventional
immunosuppressive therapy.
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