CASE STUDY

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Emphysematous pyelonephritis
Mohammed Nayeemuddin*, Oliver J Wiseman and Alan G Turner

S U M M A RY THE CASE
A 54-year-old nondiabetic male was admitted
Background A 54-year-old nondiabetic male presented with high fever,
vague lower abdominal pain and leakage of urine around his long-standing
with high fever, vague lower abdominal pain
suprapubic catheter. Examination revealed pyrexia and tenderness in and leakage of urine around his suprapubic
the right renal angle. White cell count was 22.8 × 109/l. Plain abdominal catheter. He was wheelchair-bound and disabled
X-ray showed calculi in the right kidney, ureter and bladder. Intravenous with multiple sclerosis. A catheter had been in
pyelogram showed gas confined to the right upper renal pelvis and place for several years to manage his urinary
perinephric space. incontinence. He had previously undergone
multiple cystoscopies and bladder wash-outs
Investigations Urine and blood cultures, plain abdominal X-ray,
for frequent catheter blockages and had expe-
intravenous pyelogram, abdominal ultrasound, MAG3 renogram and
rienced recurrent urinary tract infections, two
histopathology.
of which were complicated with septicemia.
Diagnosis Emphysematous pyelonephritis: class 2 or type 1. Escherichia He also had a history of methicillin-resistant
coli was isolated from urine obtained by endoscopic drainage. Staphylococcus aureus (MRSA) infection in the
Management Endoscopic drainage of pus and simple nephrectomy. sacral wound.
KEYWORDS emphysematous pyelonephritis, indwelling urinary catheter, On examination he was pyrexial at 38.2°C,
intravenous pyelogram, nephrolithiasis, simple nephrectomy with tenderness in the right renal angle. He had
a raised white cell count of 22.8 × 109/l. Urea and
creatinine were normal. Blood cultures showed
no growth; urine cultures showed moderate
mixed growth. Sacral wound and catheter-site
swab cultures grew MRSA.
A plain-film X-ray of the abdomen showed
multiple areas of calcification over the right
kidney, two calculi in the line of the right ureter
and multiple bladder stones. A clinical diagnosis
of calculus obstruction with superimposed infec-
tion was made. Intravenous pyelogram showed
gas within the right upper renal pelvis and peri-
renal space, with no evidence of excretion from
it (Figure 1). Ultrasound findings suggested
distension of the right kidney with central renal
necrosis, and large amounts of echogenic mate-
rial within the collecting system (Figure 2). A
right retrograde pyelogram confirmed the pres-
M Nayeemuddin is a Senior House Officer in Worcester–Warwick Basic ence of right ureteric stones, but an attempt at
Surgical Rotation, OJ Wiseman is a Specialist Registrar in Urology and double-J stenting failed. A ureteroscope was
AG Turner a Consultant Urologist and Medical Director at the Edith Cavell passed over a guide wire into the right renal
Hospital, UK. pelvis and 80 ml of pus was drained. Pus culture
Correspondence
grew Escherichia coli.
*Department of Urology, Edith Cavell Hospital, Bretton Gate, Peterborough PE3 9GZ, UK The patient improved after drainage. A MAG3
md_nayim@yahoo.com renogram performed a few days later showed
only 14% relative right renal function, and a
Received 28 October 2004 Accepted 11 January 2005
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simple nephrectomy was performed 2 weeks
doi:10.1038/ncpuro0095 later. Operative findings revealed multiple

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small abscesses, necrotic areas and surrounding

edema. Histologic analysis confirmed chronic
emphysematous pyelonephritis. The patient
made an excellent postoperative recovery and
was discharged 10 days later, with normal renal
function and his urinary catheter in situ. After
1 year he is doing very well, with normal renal
function and normal left renal ultrasound.

DISCUSSION OF EMPHYSEMATOUS
PYELONEPHRITIS
Emphysematous pyelonephritis is an uncommon
and life-threatening necrotizing infection of the
kidney, first described by Kelly and MacCallum
in 1898. It is characterized by the presence of gas
within the renal parenchyma, collecting system
or perinephric space.1 Most patients are diabetic Figure 1 Intravenous pyleogram showing gas confined to the right upper renal
pelvis and perirenal space. Arrows indicate the crescent of gas around the right
(90%), and the male:female ratio is 1:2, with a kidney and within the renal parenchyma.
mean age at presentation of 55 years. Female
predominance is most likely due to the increased
susceptibility of females to urinary tract infec-
tions.2 Ureteric obstruction has been reported
in 25–40% of cases. Other risk factors include
drug abuse, neurogenic bladder, alcoholism and
anatomic anomaly.3

Clinical features and pathogenesis

No symptoms, signs or laboratory data are useful
in distinguishing emphysematous pyelonephritis
from uncomplicated pyelonephritis.4 Patients
are typically very ill, with circulatory or liver
failure caused by sepsis.5 According to Evanoff
et al., abdominal tenderness (83%), fever (80%)
and flank pain (55%) are the most common
clinical manifestations.6 Other symptoms such
as confusion, nausea, vomiting, shock and Figure 2 Ultrasound scan of the right kidney,
showing gross distension and echogenic debris
abdominal pain reflect the severity of the infec-
(arrows) within the collecting system.
tion.2 Most series report an average delay of 18
days between onset of symptoms and diagnosis,
mainly due to a low index of suspicion, the
nonspecificity of symptoms and inappropriate which may impair tissue perfusion and trans-
radiological investigations.7 portation of gas to the blood stream, allowing
The following four mechanisms for gas gas accumulation.8
formation in emphysematous pyelonephritis E. coli (58%), Klebsiella pneumoniae (24%)
have been proposed: the presence of patho- and mixed flora (10%) have been cultured from
genic Gram-negative, gas-forming facultative patients with emphysematous pyelonephritis;3
bacteria such as E. coli; a defective immune Proteus mirabilis, Enterobacter aerogenes and
response, e.g. due to diabetes, alcoholism or Candida sp. have also been implicated.9
cancer, allowing bacterial multiplication; Urinary tract obstruction, whether partial
high tissue glucose levels that help facultative or complete, allows urinary stasis, negating
anaerobes to ferment glucose and produce the flushing effect of urine flow and allowing
carbon dioxide and hydrogen; and ischemia bacterial multiplication in urine and within the
of local tissue, due to severe inflammation, kidney itself. Obstruction causes a change in
microangiopathy or urinary tract obstruction, intrarenal blood flow and decreases neutrophil

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delivery, inhibiting the body’s natural defences
and the effectiveness of antimicrobials.
The long-term complications of indwelling
catheterization include bladder and upper
urinary tract urolithiasis, hemorrhagic cystitis,
high bladder pressures with associated vesico-
ureteral reflux and renal deterioration, urethral
erosion, cancer and urinary tract infections.10
Indwelling catheters can also predispose to
infectious complications of epididymo-orchitis,
bacterial prostatitis, pyelonephritis, periurethral
abscesses and infection calculi.
There is a higher incidence of bacteriuria
and urinary tract infections in patients with
diabetes,11 and infections tend to be more
serious and protracted. Due to their immuno-
compromized state, these patients are at increased
risk of ascending renal infection, pyelonephritis,
papillary necrosis, renal carbuncle, renal cortico-
medullary and perinephric abscesses and emphy-
sematous pyelonephritis. Factors predisposing
patients with diabetes to complicated infections
include autonomic neuropathy leading to poor
bladder emptying and urinary stasis, micro-
angiopathy, leukocyte dysfunction and frequent
urinary tract instrumentation. Emphysematous
pyelonephritis should be suspected when a
patient with diabetes, especially a woman, has
a urinary tract infection and signs of sepsis, loss
of glycemic control and raised serum creatinine
concentrations.6 Patients require immediate
hospital admission with vigorous intravenous
antibiotics, fluid and electrolyte correction,
insulin administration for controlling blood
glucose, and prompt investigations by radio-
graphy and ultrasonography, followed by CT
scanning if gas is detected or the patient does
not rapidly respond to therapy.
Diagnosis
Plain film is the simplest study to screen
for emphysematous pyelonephritis, and is useful in
50% of patients.12 Ultrasonography is useful
for the diagnosis of urinary tract obstruction,
but is operator-dependent and insensitive for
the detection of renal gas. CT allows accurate
delineation of the extent of intrarenal and extra-
renal disease,2 and is superior to ultrasound in
diagnosing and characterizing the lesion, as
well as in follow-up. Furthermore, CT image-
guided drainage offers effective treatment, often
avoiding emergency nephrectomy.13
A number of staging systems for
emphysematous pyelonephritis exist (Table 1).
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Unlike that of Michaeli et al.,9 the system
proposed by Huang and Tseng4 has important
prognostic and therapeutic implications. In a
retrospective study of 38 patients, Wan et al.3
identified two types of emphysematous pyelone-
phritis based on fluid and gas pattern, and corre-
lated findings with clinical course and prognosis.
The mortality rates almost completely matched
the degree of parenchymal destruction. Type 1
disease had a substantially higher mortality rate
(69%), more extensive parenchymal destruction
and a more fulminant clinical course. Type 2 had
a lower mortality rate (18%) and more indo-
lent clinical course. It is unclear whether these
two subtypes represent stages in the evolution
of emphysematous pyelonephritis or different
pathophysiological responses.
Treatment and outcome
The aim of treatment of emphysematous pyelo-
nephritis is renal conservation without prejudice
to the patient’s safety. This can be achieved by
prompt diagnosis, close monitoring of progress,
interspeciality cooperation and repeated clinical
and radiological assessment and intervention.13
Medical treatment, including correction of fluid
and electrolyte balance, strict glucose control
and appropriate intravenous antibiotics, is the
first-line priority. If intravenous or retrograde
pyelography confirm urinary tract obstruction,
double-J stenting or percutaneous nephros-
tomy should be performed as soon as possible.
Removal of the stone should be deferred until
the infection has settled.
The definitive treatment of choice for
emphysematous pyelonephritis has traditionally
been nephrectomy. Early data showed mortality
for patients who underwent nephrectomy to be
20%, compared with 80% for patients treated
solely medically.6 The higher mortality of non-
nephrectomized patients could have been due
to delayed diagnosis, inadequate medical treat-
ment or lack of modern percutaneous drainage
techniques; or perhaps the patients were too ill
to withstand surgery.
Medical and conservative surgical manage-
ment have since been improved, and there is
controversy regarding optimal treatment. Low
(8%) mortality has been reported with a staged
approach combining CT-guided percutaneous
drainage with antibiotic and supportive therapy.7
Best and colleagues14 reported successful treat-
ment of gas-filled renal abscesses in diabetic
patients with medical treatment alone. Medical
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Table 1 Classification of emphysematous pyelonephritis.
Classification system Basis for classification
Michaeli et al.9 Plain film X-ray
Stage 1
Stage 2
Stage 3
Huang and Tseng4 CT
Class 1
Class 2
Class 3A
Class 3B
Class 4
Wan et al.3 Radiography,
ultrasonography and CT
Type 1
Type 2
therapy is now considered a valid alternative to
surgical intervention,15 and management should
be tailored according to disease severity and
radiographic findings.
Risk factors for poor response to conservative
treatment are thrombocytopenia, acute renal
failure, disturbance of consciousness and shock.
Nephrectomy should be performed in patients
with extensive disease and more than two risk
factors, or those who do not substantially improve
after appropriate medical treatment and drainage.4
If renal or perirenal gas persists on ultrasound or
CT scan despite medical therapy, surgical drainage
or nephrectomy is mandatory. Persistence of gas
after antibiotic treatment and surgical drainage is
also an indication for nephrectomy.16
DISCUSSION OF THE CASE
Diagnosis
The diagnosis of emphysematous pyelonephritis
was not initially considered in the patient
described because he did not have diabetes. His
multiple sclerosis affected his ability to localize
pain, so the symptoms of pyrexia and vague
lower abdominal pain were the only clues to
the diagnosis. The patient did, however, present
with a number of risk factors, including a
long-term indwelling catheter predisposing
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Defining features
Gas present in the renal parenchyma or perinephric tissues
Gas present in the kidney and its surroundings
Extension of gas through Gerota’s fascia and/or bilateral
disease
Gas present in the collecting system only
Gas present in the renal parenchyma, without extension
into the extrarenal space
Extension of gas or abscess into the perinephric space
Extension of gas or abscess into the pararenal space
Bilateral emphysematous pyelonephritis, or
emphysematous pyelonephritis in a solitary kidney
Parenchymal destruction with either lack of fluid collection
or presence of streaky or mottled gas
Renal or perirenal fluid collection with bubbly or loculated
gas or gas in the collecting system
him to infection, and obstruction of the right
kidney by ureteric stones. The fact that he had a
history of multiple lower urinary tract infections
suggested an episode of recurrence, but locali-
zation of tenderness to the right loin indicated
pyelonephritis. The diagnosis was picked up by
careful interpretation of the intravenous pyelo-
gram alone, which was performed to check for
urinary tract obstruction by the ureteric stones
seen on plain abdominal X-ray. Abdominal CT
scan would have been useful in staging the disease,
but the priority was to drain the kidney. Pus
culture revealed a coliform infection, consistent
with the most common gas-forming organism
found in emphysematous pyelonephritis.
Treatment and outcome
In cases managed conservatively, any co-existing
urinary tract obstruction must be corrected to
maximize the clinical response to antibiotics. The
priority in the patient described in our case study
was to drain the kidney. Although we were unable
to insert a double-J stent, ureteroscopic drainage
of pus was carried out with a view to salvaging
the kidney. Although there was some clinical
improvement, whole-kidney function remained
poor due to extensive parenchymal destruction
and so we proceeded with nephrectomy.
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Competing interests
The authors declared
they have no competing
interests.
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CONCLUSION
Emphysematous pyelonephritis is a rare but
serious disease, most commonly occurring in adult
females with diabetes. This case demonstrates that
it must also be considered in nondiabetic patients
with long-standing obstructive uropathy, renal
stones and indwelling urinary catheters.
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