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Article Review/Hyperlink
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Provonost PH, Brady HR, Gunning ME, Espinoza O, Rennke HG
Clinical features, predictors of disease progression and results of
renal transplantation in fibrillary/immunotactoid
glomerulopathy
Nephrol Dial Transplant
(May) 11:837-842 1996

These authors report on 25 previously unpublished patients with
fibrillary glomerulopathy (FG) / immunotactoid glomerulopathy (ITG),
and review the literature covering 161 previously reported patients.
FG/ITG were distinguished either by fibril diameter (FG < or =
30nm, ITG greater than 30 nm), or by arrangement (FG random, ITG
focally organized).
Results revealed that more FG patients were female (1.3:1) while more
males had ITG (1.4:1). FG Was much more common in Caucasians than in
blacks (9:1). In both FG and ITG, proteinuria was universal, and
nephrotic syndrome (70 - 86%), hematuria (64 - 75%), hypertension (45
- 85%) and renal insufficiency (42 - 54%)) were very common; no clear
clinical picture differentiated between the two pathologic groups.
When broken into groups with slow, intermediate, or rapid
deterioration in renal function (by 1/Cr slope), patients who
progressed more rapidly not surprisingly were more likely to be
nephrotic and to have worse hypertension. Five renal transplants were
performed in four patients, with recurrence of fibrillary deposits on
graft biopsy in three. However the rate of transplant loss was much
slower than that of the native kidneys, raising the question of
whether the immunosupression modified the disease course. When
patients with circulating or urinary paraproteins were included,
malignancy was more common in patients with ITG (33%) vs. FG (7%),
whereas if they were excluded the incidence of malignancy dropped to
less than 7% in both groups.
Besides helping to better define the clinical picture of fibrillary
and immunotactoid glomerulopathy, this paper suggests that
transplantation may be a good option for patients with this condition.
It further suggests that immunosupression may have a role in slowing
the rate of the disease progression, particularly in patients who
have been transplanted.
Comment: I agree with the author's suggestion not to
overclassify this condition based purely on the fibrillar structure
because the clinical illness, and degree of renal injury, seem similar
in both groups (FG & ITG). Their suggested classification of
glomerulopathy with extracellular fibrillary deposits seems prudent at
the current time:
1) Amyloid-associated fibrillary glomerulopathy (congo-red
positive),
2) Congo-red negative fibrillary glomerulopathy associated with
cryoglobulinemia,
3)Congo-red negative fibrillary glomerulopathy in association with
monoclonal gammopathy and
4) Congo-red negative idiopathic fibrillary glomerulopathy without
measurable cryoglobulins or monoclonal gammopathy.
Whether or not glomerulopathy with congo-red negative fibrillary
deposits represents a forme fruste of the similar disease in
association with cryoglobulins or monoclonal gammopathy still remains
an open question. (Stephen Pastan, M.D., Emory Clinic)
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