2010 THE AUTHORS; JOURNAL COMPILATION 2010 BJU INTERNATIONAL

Laparoscopic and Robotic Urology

EMPHYSEMATOUS PYELONEPHRITIS
UBEE

ET AL.

BJUI Emphysematous pyelonephritis

BJU INTERNATIONAL

Sarvpreet Singh Ubee1, Laura McGlynn2 and Mark Fordham2

1
Department of Urology, The Heart of England NHS Foundation Trust, Birmingham, UK, 2Department of Urology, The
Royal Liverpool and Broadgreen University Hospital, Liverpool, UK
Accepted for publication 25 May 2010

Study Type – Therapy (case series)
Level of Evidence 4
Emphysematous pyelonephritis (EPN) is an
acute severe necrotizing infection of the
renal parenchyma and its surrounding
tissues that results in the presence of gas in
the renal parenchyma, collecting system, or
perinephric tissue. The cause for mortality in
EPN is primarily due to septic complications.
Up to 95% of the cases with EPN have
underlying uncontrolled diabetes mellitus.
The risk of developing EPN secondary
to a urinary tract obstruction is about
25–40%. There are three classifications
of EPN based on radiological findings.
Acute renal failure, microscopic or
macroscopic haematuria, severe
proteinuria are other positive findings in
EPN. Escherichia coli is the most common
causative pathogen with the organism
isolated on urine or pus cultures in nearly
70% of the reported cases.
What’s known on the subject? and What does the study add?
Emphysematous pyelonephritis (EPN) is a severe necrotizing infection of the renal
parenchyma. The clinical course of EPN can be severe and life-threatening if not
recognized and treated promptly. Most of the information has been from case reports, a
few large series have also been reported. Using an evidence-based approach, this review
describes the pathogenesis, classification, complications, and management of EPN.
A plain radiograph shows an abnormal gas and functioning renal tissue is present. The
shadow in the renal bed raising the suspicion treatment strategies include MM alone, PCD
whereas an ultrasound scan or computed plus MM, MM plus emergency nephrectomy,
tomography (CT) will confirm the presence and PCD plus MM plus emergency
of intra-renal gas thus supporting the nephrectomy. In small proportion of patients
diagnosis of EPN. Gas may extend beyond managed with MM and PCD, subsequent
the site of inflammation to the sub capsular, nephrectomy will be required and in these
perinephric and pararenal spaces. In some patients the reported mortality is 6.6%
cases, gas was found to be extending into Nephrectomy in patients with EPN can be
the scrotal sac and spermatic cord. simple, radical or laparoscopic.
Subsequent case studies have shown
patients being successfully treated with PCD KEYWORDS
when used in addition to medical
management, with significant reduction in emphysematous pyelonephritis, diabetes
the morality rates. PCD should be performed mellitus, escherichia coli, percutaneous
on patients who have localized areas of gas drainage, nephrectomy

INTRODUCTION
Emphysematous pyelonephritis (EPN) is an
acute severe necrotizing infection of the renal
parenchyma and its surrounding tissues that
results in the presence of gas in the renal
parenchyma, collecting system or perinephric
tissue [1,2]. The first case of gas-forming
renal infection was reported by Kelly and
MacCullum in 1898 [3]. Since then terms such
as ‘renal emphysema’, ‘pneumonephritis’ as
well as ‘emphysematous pyelonephritis’ have
been used to describe the gas-forming
infection. In 1962, Schultz and Klorfein [4]
suggested the use of ‘emphysematous
pyelonephritis’ as the preferred term, as it
emphasizes the relationship between infective
pathology and gas formation. Some authors
have suggested that this term should be
applied to gas within the renal parenchyma or
perinephric space [5–7], but several other
investigators define it as gas within the
collecting system, parenchyma, perirenal
space, or in any of these [8,9]. Gas in the
collecting systems only, ‘emphysematous
pyelitis’, is a separate condition and could be
secondary to instrumentation of the urinary
tract. A patient infected with emphysematous
pyelitis has an excellent prognosis with
medical management (MM), whereas EPN
deserves special attention because of its life-
threatening potential with either MM or
surgical management. Mortality from EPN is
primarily attributable to septic complications.
EPN was associated with a mortality rate of
up to 78% until the late 1970s but, over
the last two decades, improvement in
management techniques has reduced the
mortality rate to 21% [7,10].
We performed a structured comprehensive
literature review within the MEDLINE
database and The Cochrane Library Central
Search database. The initial search terms were
‘emphysematous pyelonephritis’ and
‘pyelonephritis’. Based on these results,
additional searches were performed using the
terms ‘percutaneous nephrostomy’ and
‘nephrectomy’. English-language publications
were selected and included in this review
article.
ASSOCIATED FACTORS
Diabetes mellitus is the single most common
associated factor. Up to 95% of patients with
EPN have underlying uncontrolled diabetes
mellitus [7,11]. Other reported factors
associated with the development of EPN are
drug abuse, neurogenic bladder, alcoholism
and anatomic anomaly [1,12,13]. There is a
preponderance of EPN in females; the
female:male ratio reported in relatively

© 2010 THE AUTHORS

1474 BJU INTERNATIONAL © 2 0 1 0 B J U I N T E R N A T I O N A L | 1 0 7 , 1 4 7 4 – 1 4 7 8 | doi:10.1111/j.1464-410X.2010.09660.x

TABLE 1 EPN classification systems
Classification Radiological basis
Michaeli et al. Plain radiograph and
[1] intravenous
pyelogram
Wan et al. [7] CT
Huang and CT
Tseng [14]
small studies is 6 : 1 [10,14,15]. Increased
susceptibility to UTI seems to be the reason
for the higher incidence in females. The risk of
developing EPN secondary to a urinary tract
obstruction is ≈25–40% [1,12]. Although
cases of EPN have been reported worldwide, it
appears to be geographically more common
in Asia with most case studies being reported
from there.
AETIOLOGY AND PATHOGENESIS
Emphysematous pyelonephritis is a severe,
necrotizing form of acute bacterial
pyelonephritisa and Escherichia coli remains
the most common causative pathogen; the
organism has been isolated on urine or pus
cultures in nearly 70% of the reported cases
[16,17]. There have, however, been reports of
Proteus mirabilis, Klebsiella pneumoniae,
Group D Steptococcus and coagulase-
negative Staphylococcus being the causative
agent for EPN [16,17]. Anaerobic micro-
organisms including Clostridium septicum,
Candida albicans, Cryptococcus neoformans
and Pneumocystis jiroveci have, in rare
cases, been reported as the causative
pathogen for EPN [7,17]. Bacteraemia is
present in more than 50% of the patients
and the organisms are the same as those in
urine or pus culture [14]. The presence of a
less common causative organism on urine or
blood culture does not require that patient
to be treated for EPN unless there are
radiological features to suggest that EPN
might be present.
Various factors involved in the pathogenesis
of this condition have been suggested,
including high levels of glucose within the
tissues, the presence of gas-forming micro-
© 2010 THE AUTHORS
BJU INTERNATIONAL © 2010 BJU INTERNATIONAL
Class
I. Gas in the renal parenchyma or perinephric tissue
II. Gas in the kidney and its surroundings
III. Extension of gas through fascia, or bilateral disease
I. Renal necrosis with presence of gas but no fluid
II. Parenchymal gas associated with fluid in renal
parenchyma, perinephric space or collecting system
1. Gas in collecting system only
2. Parenchymal gas only
3A. Extension of gas into perinephric space
3B. Extension of gas into pararenal space
4. EPN in solitary kidney or bilateral disease
organisms, impaired vascular blood supply,
reduced host immunity and the presence of
obstruction within the urinary tract. A high
level of tissue glucose in association with
impaired blood supply to the kidneys, which
is prevalent in patients with diabetes,
facilitates the process of anaerobic
metabolism [16].
Gram-negative facultative anaerobic micro-
organisms such as E. coli are responsible for
the production of gas via the fermentation of
glucose and lactate. This process results in
the production of high levels of carbon
dioxide and hydrogen which accumulate at
the site of inflammation. Radiologically
guided needle aspiration of the gases
released by the tissues was analysed by
Huang and Tseng [14] who found carbon
dioxide and hydrogen to be the main
constituents. Nitrogen and oxygen have also
been found along with traces of ammonia,
methane and carbon monoxide [14]. Gas may
extend beyond the site of inflammation to
the sub-capsular, perinephric and pararenal
spaces. In some cases, gas was found to be
extending into the scrotal sac and spermatic
cord [18].
Pathological examination of the kidney
reveals features of abscess formation, foci of
micro- and macro-infarctions, vascular
thrombosis, numerous gas-filled spaces and
areas of necrosis surrounded by acute and
chronic inflammatory cells implying septic
infarction [7,14].
CLASSIFICATION
There are three classifications of EPN based
on radiological findings (Table 1) [1,7,14].
EMPHYSEMATOUS PYELONEPHRITIS
Michaeli et al. [1] in 1984 were the first to
classify EPN based on the findings of plain
abdominal film of kidney, ureter and bladder,
and intravenous pyelogram. Wan et al. [7] in
1996 categorized their cohort of 38 patients
into two groups based on CT findings.
They defined Type I EPN as parenchymal
destruction with either total absence of fluid
content on the CT images or presence of a
streaky or mottled gas pattern on radiographs
or CT scans, regardless of the absence or
presence of bubbly or loculated gas. Type II
EPN was defined as either the presence of
renal or perirenal fluid in association with a
bubbly or loculated gas pattern or as gas in
the collecting system with acute bacterial or
renal- or perirenal-fluid-containing
abscesses.
Four years later in 2000, Huang and Tseng
published a different classification, which was
also based on the CT findings but described in
more detail and with more sub-categories
than the previous one [14]. They classified
EPN as follows: (1) Class 1: gas in the
collecting system only; (2) Class 2: gas in the
renal parenchyma without extension to
extrarenal space; (3) Class 3A: extension of
gas or abscess to perinephric space; Class 3B:
extension of gas or abscess to pararenal
space; and (4) Class 4: bilateral EPN or solitary
kidney with EPN.
The more detailed classification by Huang and
Tseng was to show the correlation between
the class of EPN and its management.
However, two published meta-analyses
[10,11] assessing the risk factors and the
management of EPN used the classification
described by Wan et al. [7]. The preference for
a particular classification rests with the
reporting radiologist, but it is important
for the treating physician to note the
classification used as it will help to plan the
patient management, which could be either
conservative or surgical.
PROGNOSTIC FACTORS
Emphysematous pyelonephritis requires
urgent attention because of the life-
threatening potential associated with
the septic complications. Various risk
factors have been identified and reported
in the literature. In a meta-analysis by Falagas
et al. [11], seven study cohorts were identified
and studied for the risk factors affecting
mortality.
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U B E E ET AL.
The presence of diabetes mellitus appeared
to be a common risk factor for EPN but,
surprisingly, it is not associated with
increased mortality [odds ratio (OR) 0.32, 95%
CI 0.05–1.99] [11]. Similarly, no significant
association could be established between
higher mortality in EPN with nephrolithiasis,
E. coli or K. pneumoniae aetiology of EPN, age
>50 years, female sex, history of UTIs, or
alcoholism.
Systolic blood pressure less than 90 mmHg,
disturbance of consciousness as well as
increase in serum creatinine level were found
to be associated with higher mortality. The
presence of thrombocytopenia (OR 22.68,
95% CI 4.4– 16.32) and bilateral EPN (OR 5.36,
95% CI 1.41 – 20.33) are both linked with poor
prognosis. MM with antibiotics alone is
associated with a higher risk of mortality (OR
2.85, 95% CI 1.19– 6.81) when compared with
additional interventions of percutaneous
drainage (PCD) of the abscess or nephrectomy
[11]. EPN Type I based on Wan et al.’s
classification is associated with a worse
prognosis as result of a more fulminanting
clinical course and more extensive
parenchymal damage [7].
PRESENTATION
Most patients present in the fourth or fifth
decade [7,16–19]. The presenting physical
symptoms and signs are those of
pyelonephritis such as dysuria, fever/rigours,
nausea, vomiting, and flank pain [17–19].
Further potential clinical manifestations
include acute renal dysfunction, acid-base
disturbances on blood gases, hyperglycaemia,
thrombocytopenia and impaired
consciousness [14,16,18]. Rapid progression
to septic shock may occur and may even
be the presenting feature in patients with
severe emphysematous pyelonephritis
[14,17]. Loin tenderness is the most common
physical sign and in some cases crepitus
around the renal area and the scrotum may
also be felt [14,17].
Laboratory data in 70–80% of the reported
cases showed leucocytosis while
thrombocytopenia was seen in 15–20% of the
patients [16,18]. As most of these patients
have diabetes, high blood glucose level is a
common finding. Acute renal failure,
microscopic or macroscopic haematuria and
severe proteinuria are other positive findings
in EPN [14].
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DIAGNOSIS
Emphysematous pyelonephritis is a
radiological diagnosis which requires
imaging, since most of the clinical and the
laboratory findings will only indicate sepsis of
renal origin. A plain radiograph shows an
abnormal gas shadow in the renal bed raising
suspicion, whereas an ultrasonography or CT
will confirm the presence of intrarenal gas
which supports the diagnosis of EPN. CT is
preferred as it is more sensitive and it also
defines the extent of EPN by identifying
features of parenchymal destruction
[7,14,16]. Ultrasonography and plain
radiograph of the abdomen are only accurate
in 69 and 65% of cases, respectively, so
abdominal CT is necessary for early diagnosis
and further management of EPN [10].
MANAGEMENT
In patients who are being treated for
pyelonephritis, the radiological diagnosis may
be missed, unless appropriate imaging is
obtained. This group of patients, along with
those who fail to respond to the standard line
of treatment of pyelonephritis, should have
an urgent CT scan to confirm the diagnosis.
Basic resuscitation measures of oxygen,
intravenous fluids, acid base balance
correction and appropriate antibiotics should
be commenced along with good glycaemic
control. It is important to maintain a systolic
blood pressure of more than 100 mmHg, with
fluid resuscitation or inotropic support if
required. Meta-analysis of the risk factors
affecting the mortality rate concluded that a
systolic blood pressure of 90 mmHg adversely
affected the mortality rate when compared
with a pressure of more than 100 mmHg [11].
If the clinical condition and the laboratory
results show deterioration, then the level of
care should be stepped up as these patients
may require multi-organ support.
Gram-negative bacteria remain the most
common causative organisms so the initial
antibiotic regimen should target them.
Aminogycosides, β-lactamase inhibitors,
cephalosporins and quinolones can be used
and this is guided by the local hospital policy.
A combination of aminoglycoside with any of
the other three groups can be used in the
initial treatment stage. Once the culture
report is available, the antibiotics can be
changed according to the type and number of
organisms along with their individual
sensitivities.
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The accepted treatment of EPN until the late
1980s has been emergency nephrectomy
and/or open surgical drainage together with
antibiotic therapy, resulting in a reported
mortality rate of 40–50% [1,20]. Some of the
authors suggested early nephrectomy along
with MM to reduce the mortality rate and
shorten the recovery period [7,15,20].
Significant advances in the percutaneous
catheters used made it possible to have PCD
as a treatment option for EPN, which was first
shown by Hudson et al. [21]. Subsequent
case studies have shown patients being
successfully treated with PCD when used in
addition to MM, with significant reduction in
the mortality rates [17,18]. PCD helps to
preserve the function of the affected kidney in
about 70% of cases. PCD should be performed
on patients who have localized areas of gas
and in whom functioning renal tissue is
present. A pigtail drain of at least 14 Fr in size
should be inserted, ideally with CT guidance
which has a better success rate when
compared with an ultrasonography. An
abscess with loculations or multiple abscesses
is not a contraindication for PCD, as more
than one catheter can be used to drain all
loculations [10]. The abscess, which is
technically easier to access and would
significantly reduce the pressure on the viable
renal tissue, should be targeted first with PCD.
The drainage tubes should stay until follow-
up CT shows resolution of the EPN features
and until then, if need arises, the tube can be
flushed with antibiotic solutions.
During the last decade there has been a
gradual shift toward a nephron-sparing
approach with PCD, with or without elective
nephrectomy at a later stage. The treatment
strategies include MM alone, PCD plus MM,
MM plus emergency nephrectomy, and PCD
plus MM plus emergency nephrectomy [10]
(Fig. 1). Patients on PCD plus MM benefit from
follow-up CT in 4 to 7 weeks as recommended
by Chen et al. [22] to look for non-
communicating air/fluid collections. This will
also be helpful in planning a nephrectomy for
non-responders to PCD plus MM. In a meta-
analysis of the management strategies, the
most successful management was MM with
PCD (30–100%), which was also associated
with the lowest mortality at 13.5% (P < 0.001)
[10]. In the small proportion of patients
managed with MM and PCD, subsequent
nephrectomy will be required and in
these patients the reported mortality is
6.6% [10].
© 2010 THE AUTHORS
© 2010 BJU INTERNATIONAL
 EMPHYSEMATOUS PYELONEPHRITIS

FIG. 1. Management algorithm for EPN based on clinico-radiological classification by Huang & Tseng [14]. In conclusion, EPN is a potentially life-
KUB, plain abdominal film of kidney, ureter and bladder. *Risk factors: diabetes, thrombocytopenia, acute threatening condition which is most
renal failure, altered level of cconsciousness, shock. commonly associated with poorly controlled
diabetes. It requires a high index of suspicion
Fever, renal angle pain, diabetes in patients not responding to the routine
management of pyelonephritis. It is a
radiological diagnosis and CT is the best
investigation. Aggressive resuscitation should
KUB Ultrasonography be given and the condition is currently treated
by MM along with PCD. Some patients may
Gas in renal area CT Gas in renal area not respond and nephrectomy may be
required. Reported mortality figures have
improved since the 1970s but still are at
Renal parenchymal gas (EPN)
13.5% [10].

Fluid, electrolyte, glycaemic control,

Antibiotic therapy (MM), CONFLICT OF INTEREST
Classify EPN
None declared.

Class 1 & 2 Class 3A & 3B Class 4 REFERENCES

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In Class 1 and 2 EPN (based on the Huang and
Tseng classification) MM alone or combined
with PCD can provide a good outcome. In
Class 3 and 4 EPN, MM plus PCD provides a
survival rate of 85% in patients with fewer
than two risk factors, whereas in patients with
more than two risk factors MM plus PCD was
unsuccessful in 92% of cases. The proportion
of non-responders in this group requiring
nephrectomy is higher, compared with the
Class 1 and 2 EPN [14,17].
Of the 118 patients who underwent PCD only,
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These are patients who do not respond well
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Bilateral disease Solitary kidney
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Failed
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availability of renal support seems to reduce
the mortality rate [17]. The long-term
outcome for renal function and the need for
further support will depend on the degree and
amount of parenchymal loss and coexisting
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Correspondence: Sarvpreet Singh Ubee,
Department of Urology, Birmingham
Heartlands Hospital, Bordesley Green East,
Birmingham, B9 5SS, UK.
e-mail: sarv21@hotmail.com
Abbreviations: EPN, emphysematous
pyelonephritis; MM, medical management;
OR, odds ratio; PCD, percutaneous drainage.
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© 2010 BJU INTERNATIONAL