Nephrol Dial Transplant (2003) 18 [Suppl 6]: vi45–vi51
DOI: 10.1093/ndt/gfg1058
Focal segmental glomerulosclerosis in adults
J. Stewart Cameron
Renal Unit, Guy’s Hospital, King’s College, London, UK
Abstract
The lesion of focal segmental glomerulosclerosis
(FSGS) presents even greater problems of definition,
interpretation and treatment in adults than it does in
children, to the point where the specificity and utility of
the appearance can even be questioned. The histological
diagnosis of FSGS in adults can be interpreted only if
the clinical circumstances are known, but a group of
nephrotic patients with FSGS can be separated from a
much larger group of non-specific, probably secondary
but similar lesions arising in many circumstances. This
group appears even so to have a diverse aetiology, the
majority being idiopathic, but a significant minority
showing a family history and/or mutations in genes
relating to several intracellular or surface podocyte
proteins, as discussed in other articles in this sym-
posium. This heterogeneity makes conclusions with
regard to prognosis and treatment difficult to draw.
Today, in contrast to 20 or even 10 years ago, it seems
useful to treat all adult nephrotic patients with FSGS
using an adequate course of corticosteroids lasting at
least 4–6 months to establish whether they fall or not
into the 20–30% who will respond to this treatment
with decrease or loss of proteinuria. The prognosis of
such responders (who may well suffer relapse) is
relatively benign with regard to renal function, but the
majority of the remainder evolve into renal failure.
Their management remains a source of controversy in
the absence of a proper database of randomized trials:
more prolonged (up to 24 months) use of lower-dose
corticosteroids with cyclosporine for 12 months or more
has support from uncontrolled studies, but cyclophos-
phamide appears to be of less obvious benefit.
Keywords: adults; focal sclerosis; focal glomerulo-
sclerosis; FSGS; nephrotic syndrome
Correspondence and offprint requests to: Emeritus Professor
J. S. Cameron, MD, Elm Bank, Melmerby, Cumbria CA10 1HB,
UK. Email: jstewart.cameron2@btopenworld.com
ß 2003 European Renal Association–European Dialysis and Transplant Association
Introduction
The lesion of focal segmental glomerulosclerosis
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(FSGS) presents even greater problems of definition,
interpretation and treatment in adults than it does in
children, to the point where the specificity and utility of
the appearance can even be questioned. The possible
pathogenesis and glomerular evolution through podo-
cyte damage, the details and varieties of the main
histological lesion and its sub-groups, presentation
and management in children and its behaviour in allo-
graft kidneys are dealt with in other papers in this
Supplement, so I will concentrate on a general overview
of the appearance of FSGS and its significance as it is
found in patients over the age of 15 years.
Focal segmental glomerulosclerosis in the past
FSGS is a glomerular appearance, which represents
the histological aspect of a final common pathway of
injury, and not a disease entity [1–4] as it has so often
been considered in the past. The words ‘primary’ or
‘idiopathic’ reflect our ignorance of aetiology and
pathogenesis, and as time passes they will become
obsolete and unnecessary. No glomerular appearance
can ever be said to be ‘primary’: ‘cryptogenic’ is a
clumsy, but better, adjective.
FSGS was first described and illustrated adequately
by Theodor Fahr in 1925 [5] in adult nephritic patients.
Even though a number of nephrotic children and adults
must have died with glomeruli showing this appear-
ance, it was the paper of another pathologist, Arnold
Rich, who in 1957 clearly defined the histological lesion
in nephrotic children [6]. Rich’s paper, based on
sections of whole kidneys, was able to make the
important observation that the lesions were either
confined or most prominent in the juxta-medullary
glomeruli.
However, this paper attracted little attention at the
time and the important CIBA foundation meeting of
1961 [7] did not mention it. Despite occasional mention
in adults [8], it was only after another decade, when the
vi46
pathologists of the International Study of Kidney
Disease in Children (ISKDC), Jack Churg, Renée
Habib and Richard White reported on renal biopsies
taken from 1966 to 1969 and pointed out the presence
of this type of lesion in a significant proportion of
childhood nephrotics [9,10].
The initial impetus, during the 1970s, was to
emphasize the differences between FSGS and minimal
change in nephrotic patients, as it became obvious that
the prognosis of FSGS was in general poorer, and a
complete or partial response to corticosteroids less
frequent. Later in the 1980s, however, discussion swung
back to emphasizing the many similarities between
patients with the two appearances, prompted by the
fact that one appearance could evolve into the other in
serial biopsies. Observations on recurrence in allograft
kidneys (discussed in this symposium by Newstead)
further emphasized the unitary hypothesis, suggesting a
common (but as yet undefined) pathogenetic mecha-
nism at work in both situations.
Although proportionately most common in older
children, it rapidly became obvious that this glomerular
appearance could be found in nephrotic patients at any
age, accounting for 5–15% of cases at almost all age
groups (Table 1). Thus, it is an important underlying
pathology in the adult-onset nephrotic syndrome. An
early observation was that, although the lesions visible
on light microscopy might be focal and segmental,
diffuse simplification of foot processes (usually called
‘fusion’ at that time as seen in cross section of
transmission electron microscopy) was present in all
glomeruli and unaffected portions of affected glomer-
uli, at least in apparently ‘primary’ forms of the disease.
This may not, however, be true in renoprival and
‘secondary’ forms of FSGS.
‘Focal segmental glomerulosclerosis’ in
adults today
Histology in a clinical setting
It remains obvious (but is often neglected) that without
information on the clinical setting of the histological
appearance, the significance of a finding of FSGS
cannot be interpreted in detail. Thus, in adults,
identical focal and segmental scarring with hyalinosis
can be found in patients with proteinuria of minor
dimensions (0.5–1.5 g/24 h), often hypertensive, whose
prognosis was good even in the absence of any
treatment [1–4]. Also, we have known for a long time
Table 1. Percentage of all nephrotic patients with FSGS
Children Adults
Age 0–15 15–19 20–30
% FSGS 7 19 18
0–15 years: data from 521 unselected childhood onset nephrotic syndromes from ISKDC patients, predominantly Caucasian and Japanese.
Adults: data from 506 nephrotic patients seen at Guy’s Hospital 1964–1984, predominantly Caucasian.
J. S. Cameron
that scarring, immunoglobulin-positive proliferative
glomerulonephritis or immunoglobulin-negative vas-
culitis, could later give rise to an identical appearance
[11], although some such patients were distinguishable
on immunohistology by the presence of (for example)
IgA; however, in many others immunohistology was
negative.
The lesion of FSGS can be found superimposed in
glomeruli showing widespread pathology. Alport’s
syndrome and membranous nephropathy are two
particular examples, but almost any type of pathology
could be complicated in its chronic course by lesions
which, although clearly secondary to identifiable
processes, seemed morphologically identical to so-
called ‘primary’ or ‘isolated’ FSGS [1–4,12,13]. Few of
these patients, however, showed a nephrotic syndrome.
These considerations apply much more to adult
patients whose histology is classified as FSGS,
compared with children in whom the majority are
nephrotic and show no associated or prior pathology.
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The wider the range of patients with minor urinary
abnormalities who are submitted to a renal biopsy, the
wider the spectrum of changes and the lower the
proportion of patients with classical, so-called ‘pri-
mary’ disease in a nephrotic setting, and the higher
proportion of others, will be found. These others will
often show one the supposed causes of ‘secondary’
FSGS [1–4,12,13], a topic discussed by Mallick in this
symposium (Table 2). Thus, it is impossible to give a
useful estimate of the percentage of biopsies with FSGS
in adults, because this figure depends too much upon
local biopsy policy, and in particular policy with regard
to patients with minor proteinuria, with or without
haematuria and hypertension.
Finally, in adults particularly, the problem of focal
and segmental lesions in renoprival settings arises. Loss
of one kidney or greater, or advancing destruction of
the kidney in settings seemingly as remote as polycystic
kidney disease, can be accompanied by the appearance
of similar lesions, which have their analogy in the
FSGS lesions found in rats subjected to sub-total
nephrectomy [14,15]. The possible pathogenetic impli-
cations of these observations are discussed elsewhere in
this symposium.
Recently, Howie et al. [16], based on work in animals
by Grond et al. in 1981 [17] as well as their
observations on human biopsies showing FSGS,
suggested that the detailed distribution of this type of
FSGS differs from that of ‘primary’ FSGS. They noted
that the glomeruli have a larger cross-sectional area
and the lesion is almost always perihilar, never at the
30–40 40–50 50–60 >60 years
11 27 16 2
FSGS in adults
Table 2. Nephrotic adults: distribution of different lesions by race
Race n MCD FSGS Memb MCGN
‘White’ 170 20 23 36 6 (%)
‘Black’ 121 14 57 24 2 (%)
Data of Korbet, 1994 [22], Rydel et al., 1995 [23]. The weakness of
any definition of racial origin is well known.
‘tip’ of the glomerulus, and can be distinguished in the
absence of clinical data. They noted this appearance in
11 of 130 patients, five of whom had single kidneys
three were biopsies from the larger of asymmetric
kidneys. These suggestions, however, are not in accord
with the data of Remuzzi et al. [18] discussed below.
These authors noted in a very detailed examination of
four kidneys, exposed previously to subtotal nephrect-
omy that up to eight separate areas of glomerulo-
sclerosis might be present in a single glomerulus. They
also observed that the majority of glomeruli showed
more than one segmental lesion and that there was no
correlation between glomerular size and sclerosis.
Again the jury remains out as to the significance of
these observations.
Thus, the concept of FSGS in adults today, centres
around the clinical identification of so-called ‘primary’
forms, related to minimal changes and dominated by
profuse proteinuria and its consequences, and the
separation of a variety of ‘secondary’ forms, in which
associated conditions can be identified and which
presumably relate to the cause of the change which may
have a variety of clinical presentations.
The histological sub-types of FSGS
The characteristics of the various sub-types of FSGS in
adults have been reviewed by myself and others in the
past, and are discussed by Howie elsewhere in this
symposium, so that here I will mention them only
briefly, although they are of great interest and
importance. The major problem is to know whether
these sub-types—‘cellular’, ‘tip’ or ‘collapsing’—have
useful meaning either in terms of understanding the
pathogenesis, or in predicting outcome or response to
treatment of FSGS [19]. Here, despite evidence for the
contrary view, I will discuss adults with ‘primary’ FSGS
as though they are a homogenous group, regardless of
which sub-type of FSGS they may demonstrate.
Definition of FSGS
We must also consider the broad definition of FSGS.
By implication, this means that in single section
preparations, the lesion is focal in distribution and
segmental in nature, and that the frequency of the
lesions can be assessed by doing glomerular counts. For
vi47
example, Schwartz et al. [19] noted an average of
21 ± 14% glomeruli apparently affected in limited
number of sections from biopsies of 81 adult-onset
patients. That this simple approach is inadequate is
clear from several studies. In ‘primary’ FSGS, Fogo
et al. [20] noted that lesions were more widely
distributed in adults (31.5 ± 6.8%) than children
(11.7 ± 5.7%), but also that on examination of serial
sections the number involved rose to 48 ± 6.6% and
23.2 ± 7.4, respectively. Remuzzi and colleagues [18]
performed an even more extensive study on four sub-
total nephrectomy kidneys involving three-dimensional
reconstructions: in single sections, 51% were of
glomeruli which were globally sclerosed, 12% segmen-
tally sclerosed and 37% normal, whilst these figures
were 51, 42 and 8% normal on serial sectioning.
Finally, Fuiano et al. [21] showed that in 14 biopsies
from adults, although 31.5% of 182 glomeruli had
sclerotic glomeruli, this figure rose to 71.8% on serial
sectioning, and in the 57 glomeruli for which the whole
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glomerulus was available, to 81.7%. Thus, the ‘focal’
nature of the lesion is exaggerated greatly by sampling
errors contingent on their segmental nature. Perhaps
we should drop the ‘focal’ from the name of this
appearance, and call it simply ‘segmental sclerosis’.
FSGS and the minimal change lesion
What can we make of all this today? Clearly the focal
sclerosing lesion seen in circumstances outside the
apparently primary nephrotic syndrome forms a
different group of patients from those with minimal
changes. Nevertheless, they must share some final
common expression of injury to generate a lesion,
which is morphologically the same as that in ‘primary’
FSGS. The continuing question centres on whether, in
a setting of the nephrotic syndrome, minimal change
lesions and FSGS represent two aspects, more or less
severe, of the same pathogenetic mechanism. This
mechanism concerns the presence of a circulating
substance yet to be fully identified, which is discussed in
detail elsewhere in this symposium.
Three important pieces of clinical evidence support
the identity of the two appearances [2]. The first is
the repeated description of occasional patients with
initially and indubitably corticosteroid-sensitive dis-
ease and minimal change on histology, who evolve to
show FSGS on biopsy, develop corticosteroid resis-
tance, end-stage renal failure—and even on occasion
recurrent disease in their transplant, as in one of our
own cases. On the other hand, intense immunosup-
pression can on occasion (see below) push even
patients with renal functional impairment and FSGS
into remission, contrary to what was formerly believed.
Finally, proteinuria can recur in kidneys grafted into
patients with FSGS, with the subsequent appearance
of FSGS lesions only after months of proteinuria.
However, some current data do not support the idea
of identity between FSGS and the minimal change
lesion. In particular, studies of families of patients with
FSGS and with minimal change lesions reveal linkages
vi48
with as yet ill-defined genetic loci (see note added in
proof), which are different in patients with the two
appearances. This is difficult to reconcile with the
unitary hypothesis, and suggests that nephrotic FSGS,
just like other forms, is but one of a group of
appearances, which resemble each other but are not
identical. Only when the pathogenesis can be described
more accurately at various levels of precipitating
factors, genetic background and mediation of injury
is this question likely to be settled.
Clinical features of FSGS in adults
The clinical features of FSGS in adults have been
described in only a few hundred patients in numerous
series over several decades (Table 3) [1,2,22–24] and the
tiny size of this database needs to be remembered.
Proteinuria is almost invariable, of dimensions, which
will induce a nephrotic syndrome in a proportion of
patients, which varies with numbers of non-nephrotic
patients biopsied, but is usually 60% at onset and
may be as high as 80–90% with time. Secondary forms
of FSGS rather rarely present with a full nephrotic
syndrome, even if in the presence of quite heavy
proteinuria they become hypoalbuminaemic. HIV-
positive patients are an exception to these statements,
however. In primary FSGS, macroscopic haematuria is
very rare, but microscopic haematuria usual and
hypertension common. Renal function as judged by
plasma creatinine concentration or, better, glomerular
Table 3. A classification of FSGS
1. ‘Primary’ (idiopathic) FSGS, including:
Collapsing glomerulopathy
Cellular variant
Glomerular tip lesion
2. FSGS with reduced renal mass and glomerulomegaly
Reflux nephropathy
Dysplasia
Oligomeganephronia
Morbid obesity
Failing allografts
Renovascular disease, etc.
3. Secondary FSGS
Alport’s syndrome
Membranous nephropathy
Sickle cell disease
Pre-eclampsia
Diabetes, etc.
HIV-associated FSGS
Heroin-associated FSGS
4. Associated with inherited disorders and anomalies
Alport syndrome
Mitochondrial cytopathies
Charcot-Marie-Tooth disease
Down’s syndrome
Etc.
5. Scarred proliferative disease
IgA nephropathy
Henoch–Schonlein purpura
Lupus nephritis
Post-streptococcal nephritis, etc.
J. S. Cameron
filtration rate (GFR) measurements depends upon the
severity of the nephrotic syndrome and the duration of
disease before investigation, but is frequently reduced
(20–25%).
Only during the past 10 years or so has it been
realized that FSGS shows a remarkable racial
predominance. This is particularly so in adult
nephrotics, with almost two-thirds of Afro-American
adults with a nephrotic syndrome showing the FSGS
lesion [25,26] (Table 4). This is true also for one-third or
more of Afro-American nephrotic children. It has been
suggested in addition that the frequency with which the
lesion is now seen in nephrotic patients has increased,
particularly in adults [27,28] but also in children.
However, all these reports have emerged from the
United States, and it is not clear whether these findings
are true also of European countries. Certainly there has
been no increase in the apparent incidence of FSGS in
our own data, in which the proportion of adult
nephrotics with the appearances of FSGS in their renal
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biopsy remains 20% from the 1970s up to the end of
the century. It is very difficult to be sure about the
current proportion of FSGS in nephrotic children,
because far from all nephrotic children are biopsied and
because today it is almost standard practice between
the ages of 1 and 6 or even 10 years only to biopsy those
who fail to show an initial favourable response to
treatment with corticosteroids, or who suffer a sub-
sequent troublesome course.
Some patients with apparently primary FSGS have
other family members affected [29]. The pattern of
inheritance is probably both autosomal dominant and
autosomal recessive in different families, but this has
not been established with certainty. Linkages with
loci on chromosomes 11 and 19 have been reported,
suggesting heterogeneity of inheritance.
Numerous studies have attempted to correlate
clinical and histological features with outcome so that
prognosis and treatment can be better informed [21–
24,29–31]. Clinically, the over-riding feature is whether
or not proteinuria becomes normal, almost always in
association with treatment. Patients in whom this
happens, even if they relapse repeatedly, almost always
do well. The only other features that have prognostic
value are renal function at the time of investigation
and the amount of proteinuria. Age, sex, race and
hypertension do not seem to affect prognosis. In renal
biopsy specimens—as in almost all forms of so-called
‘glomerular’ diseases—the major prognostic factor is
Table 4. Clinical features of adult-onset patients with FSGS
%
Male sex 62
Nephrotic syndrome 68
Hypertension 43
Haematuria 45
Renal impairment 24
Analysis of 492 patients in the literature from Korbet et al. [22].
FSGS in adults
the presence and extent of tubulo-interstitial damage.
We have not been able to confirm in our own data any
correlation between the site of the glomerular lesions
and prognosis [2].
This issue of whether the site of injury within the
glomerulus is associated with prognosis is controver-
sial, and I have reviewed the evidence as I see it
previously [2]. Since then, no new data have arrived to
allow me to change my position that site of the FSGS
lesion has no influence on outcome.
Treatment of FSGS in adults
In the absence of any real understanding of the
pathogenetic background, treatment of FSGS in adults
was—and remains—empiric. Again, the tiny database
and paucity of controlled trials must be remembered
when making and assessing any statements about the
treatment of FSGS [32–34]. This topic is reviewed in
this Supplement by Filler and Meyrier. However, a few
brief remarks are in order here.
Obviously a first step is to identify patients with
a ‘secondary’ form of FSGS (see Mallick, this
Supplement), as they may need different management.
Here, we are concerned only with so-called ‘primary’
FSGS in adults. Treatment may then be graded
according to the presence or absence of adverse
prognostic features. Thus, it is probably not worthwhile
treating patients aggressively who have only 0.5–2.0
g/24 h proteinuria, as the analyses discussed above
suggest that the great majority of such patients will do
well and will not develop renal failure even on
prolonged follow-up. It is with the remainder suffering
more profuse and persistent proteinuria that we are
concerned. Many of these patients will have reduced
renal function and tubulo-interstitial damage in their
renal biopsy.
Today in my view it seems worthwhile to treat all
adult-onset nephrotic patients showing FSGS in their
biopsies using corticosteroids. In the past, inadequate
courses were often used [35], frequently of only 8
weeks duration, because the majority of children with
minimal change will respond within this time.
Unfortunately it took a long time to realize that this
was not true in adults, even with only minimal changes
present, and that a minimum of 16 weeks treatment
is necessary to establish ‘resistance’ to corticosteroid
therapy. Thus, today a minimum of 4 months cor-
ticosteroids (preferably 6 months) is suggested, not only
because of possible benefit from loss or major
diminution of proteinuria in as many as 30% of
patients, but also because of the prognostic value that
even failure to achieve benefit can bring (see above).
The data (Table 5) include studies with different
dosages and duration of corticosteroid treatment,
which makes evaluation difficult. There is a suggestion
from the data in aggregate, recently emphasized by
Ponticelli and colleagues [36], that the greater the
intensity and duration of immunosuppression, the
vi49
Table 5. Outcome in relation to response to treatment in adult
patients with FSGS
Response to treatment Proportion entering renal failure
n %
Complete response 2 of 278 3
Partial response 8 of 32 25
No response 73 of 132 60
Total 83 of 242 30
From an analysis of the literature by Korbet et al. [24].
greater the rate of response. The response seems likely
to be more frequent in those with normal renal
function, but this point has never been tested
adequately.
If there is no response, what to do next remains
controversial. No controlled clinical trials of cyclophos-
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phamide have ever been conducted in adults with
FSGS, but likely benefit seems low; the only trial, in
children, showed no benefit. However, Ponticelli and
colleagues [36] have made a strong plea for longer and
stronger primary treatment in adult patients, with
corticosteroids alone with or without cytotoxic agents.
Similar and more numerous pleas have been made in
the treatment of children with FSGS also (see Cochat,
this Supplement). Unfortunately it is not clear which
patients might benefit from additional cytotoxic agents,
and in the Italian data the outcome was similar to that
using corticosteroids alone.
Thus, one option after 6 months treatment using
corticosteroids without response is to continue the
treatment for up to 2 years in lower dosages. One
problem is that the Italian database consists only of
patients with a normal or near normal plasma
creatinine. This makes comparison with previous data
difficult, as it is impossible to derive from these
accounts what the relationship between renal function
and apparent response to treatment may have been.
Nevertheless, within this restricted group of those with
normal/near normal renal function, 50% achieved
remission after 3 years treatment, and a further 20%
partial remission [36].
In contrast there is now a considerable quantity of
uncontrolled data [35], and one small controlled trial
reviewed in detail by Meyrier in this Supplement, which
suggests that cyclosporine does confer major benefit
in ‘corticosteroid-resistant’ patients [37]. In 49 such
patients, 70% of those randomized to prednisolone
plus cyclosporine went into complete (9%) or partial
(61%) remission, compared with only 4% of those
continuing to take prednisolone plus placebo. The
effect on progression of renal failure was, however, less
dramatic: a quarter of the treated group and half of the
placebo group suffered a halving of renal function
during 4 years. The weakness of this study is that
‘steroid resistance’ was defined by only a minimum of 8
weeks (mean 14 weeks) treatment, which certainly
means that some ultimately steroid-responsive patients
vi50
will have been included. Also, all patients with a
creatinine clearance <42 ml/min/1.73 m2 were excluded.
Nevertheless, if after 6 months treatment with cortico-
steroids no or partial remission has been achieved, it
seems worthwhile to use a further 6 months of
prednisolone plus cyclosporine.
All the data still leave open the question of what (if
anything) should be done for patients who present
already with considerable reduction in renal function
(GFR <30–40 ml/min). Most physicians today would
use ACE inhibitors with salt restriction in such patients,
as almost all have profuse proteinuria and also
hypertension. Whether it is possible to ‘covert’ these
progressive and resistant patients into responsive state
by heavy immunosuppression remains unknown. It
should hardly be necessary to state that all the regimes
carry a heavy burden of side effects, some evident such
as infections, and some not so evident such as
osteopenia or even frank osteoporosis.
Several other approaches to treatment have been put
forward, including repeated plasma exchange [38], lipid
apheresis and immunoadsorption, but remain experi-
mental. The tendency for the literature to contain only
reports of success when small numbers of patients are
studied must be borne in mind.
Outcome of FSGS in adults
Outcome studies in adult-onset FSGS patients, as in
children, strongly suggest two subpopulations [2]: a
smaller group (20–30%), which maintains renal
function long-term and often loses proteinuria alto-
gether; and the reminder, at least 50%, in whom a
steady fall-off in renal function occurs with persisting
proteinuria and/or nephrotic syndrome, whose half-life
for renal failure is some 4–5 years. Whether these two
groups represent populations with different pathogen-
esis is unclear, and they may represent a statistical
artefact. They do not relate to the intra-glomerular site
of the lesion [2]. The crucial question of whether
treatment can convert one type of outcome into the
other remains unanswered: if not, then identification of
those unlikely to respond becomes paramount. If so,
then intense treatment with a penalty in terms of side
effects becomes more justified.
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vi51
Note added in proof
Since this article was written, some of the mutations in podocyte
molecules described elsewhere in this supplement and since
(Kerjashki, 2001; Devarajan and Spitzer, 2002) in familial cases
of FSGS, have been found also in a proportion of sporadic cases in
children (Caridi et al., 2001; Frischberg et al., 2002; Winn, 2002).
How these findings relate to the heterogeneity, prognosis and
treatment of adult FSGS is not clear yet.
Caridi G, Bertelli R, Carrea A et al. Prevalence, genetics and
clinical features of patients carrying podocin mutations in steroid-
resistant nonfamilial focal segmental glomerulosclerosis. J Am Soc
Nephrol 2001; 12: 2742–2746
Devarajan P, Spitzer A. Towards a biological characterization of
focal segmental glomerulosclerosis. Am J Kidney Dis 2002; 39: 625–
636
Frischberg Y, Rinat C, Megged O et al. Mutations of NPHS2
encoding podocin are a prevalent cause of steroid-resistant
nephrotic syndrome among Israeli-Arab children. J Am Soc
Nephrol 2002; 13: 400–405
Kerjaschki D. Caught flat footed: podocyte damage and the
molecular bases of focal glomerulosclerosis. J Clin Invet 2001; 108:
Downloaded from http://ndt.oxfordjournals.org/ by guest on April 4, 2012
1583–1587
Winn MP. Not all in the family: mutations of podocin in asporadic
steroid-resistant nephrotic syndrome. J Am Soc Nephrol 2002; 13:
577–579
Also, a review of the treatment of FSGS has been published
recently:
Korbet SM. Nephrology forum: Treatment of primary focal
segmental glomerulosclerosis. Kidney Int 2002; 62: 2301–2310
Finally the role of mycophenolate mofetil, now used anecdotally
with success in a few cases of adult FSGS, is not yet clear but may
be important.