Nephrol Dial Transplant (1997) 12: 2737–2739
Nephrology
Case Report
Renal-limited polyarteritis nodosa presenting with loin pain and
haematuria
J. L. Górriz1, A. Sancho1, R. Ferrer2, E. Alcoy1, J. F. Crespo1, J. Palmero3 and L. M. Pallardó1
Departments of 1Nephrology, 2Pathology, and 3Radiology, Hospital Universitario Dr. Peset, Valencia, Spain
Key words: haematuria; hypertension; kidney failure;
loin pain; polyarteritis nodosa; vasculitis
Introduction
Classic polyarteritis nodosa (PAN ) is a medium-sized
vessel vasculitis with multiorgan involvement [1]. Its
clinical polymorphism renders diagnosis difficult,
especially in cases with uncommon presentation [1–3].
We report a case of polyarteritis nodosa with renal-
limited involvement presenting with haematuria, loin
pain, and subacute renal failure.
Case report
A 49-year-old Caucasian man with a previous history
of long term headache and left renal lithiasis treated
with extracorporeal lithotripsy was seen at emergency
room because of severe loin pain as well as macroscopic
haematuria. Hypogastric and left lower quadrant pain
was also noted. Blood pressure was 180/110 mmHg,
and abdominal tenderness was detected. Apart from
macroscopic haematuria there were no relevant labor-
atory findings, with a plasma creatinine level of
97 mmol/l. A diagnosis of renal colic was made, which
improved with analgesic treatment. He was also given
nifedipine as an antihypertensive treatment. One
month later he returned to hospital because abdominal
loin pain and macroscopic haematuria reappeared,
showing renal functional impairment (urea nitrogen
12 mmol/l, creatinine 185 mmol/l ). He looked healthy,
his temperature was 36.4°C and his blood pressure was
180/110. On physical examination, abdominal ten-
derness in the hypogastrium was detected. No abdom-
inal bruits were heard. No other abnormal physical
findings were noted. Progressive renal function impair-
ment was observed in the following 2 days (urea
nitrogen 23 mmol/l, creatinine 354 mmol/l ). In order
Correspondence and offprint requests to: Jose Luis Górriz MD,
Servicio de Nefrologı́a, Hospital Universitario Dr. Peset, Avda.
Gaspar Aguilar, 90, 46017 Valencia, Spain.
© 1997 European Renal Association–European Dialysis and Transplant Association
Dialysis
Transplantation
to control blood pressure atenolol 25 mg daily, nifedip-
ine 30 mg twice a day, and enalapril 20 mg daily were
given. Other laboratory studies showed phosphorus
5.7 mg/dl and haemoglobin of 14.0 g/dl. The remaining
parameters were normal, including bilirubin, glucose,
serum proteins and electrophoresis, aminotransferases,
cholesterol, triglycerides, creatine kinase, amylase,
carbon dioxide, coagulation tests, white-cell and
differential count. Serological evaluation for immmun-
oglobulins, complement, ANA, anti-DNA, anti-
glomerular basement membrane antibodies, ANCA,
anti-ENA, cryoglobulins, circulating immune com-
plexes, anticardiolipin, hepatitis B and C viruses and
HIV antibodies were either normal or negative. Plasma
renin activity was 16 ng/ml/h (normal 0.5–2.8), plasma
aldosterone 477 mg/ml (normal<150). Proteinuria
was 0.6 g/day. Urinary analysis showed nephritic sedi-
ment, with numerous erythrocyte cell and granular
casts, and low-grade microhaematuria/leukocyturia.
Urine culture was negative. Abdomen and chest X-ray
films, fundoscopy and electrocardiogram were normal.
Abdominal and renal ultrasonographic study, includ-
ing renal artery Doppler sonogram, showed no
abnormalities.
Because of the presence of subacute renal failure, a
percutaneous renal biopsy was performed, producing
11 glomeruli that were histologically normal.
Interstitium and tubules were unremarkable except at
one site, where interstitial oedema and an artery with
moderate neutrophilic and eosinophilic infiltration of
the adventitial and medial layers were seen, besides
reduplicated and ruptured elastic fibres. Focal fibrinoid
intramural deposition was found. Inmunofluorescence
microscopy was negative. An angiographic study
showed multiple microaneurysms of the renal vas-
culature in both kidneys ( Figure 1) and a large aneur-
ysm in the left renal artery. Supra-aortic branches and
mesenteric arteries showed no aneurysms.
A diagnosis of PAN was made and three intravenous
methylprednisolone pulses (1 g×3 days) were given,
followed by prednisone at initial dose of 1 mg/kg/day.
Six monthly cyclophosphamide courses (0.5 g/m2)
were also administered. A routine renal Doppler ultra-
sonography 1 week after biopsy revealed an arterio-
2738
Fig. 1. Right renal artery angiography showing multiple
microaneurysms.
venous fistula. After 12 months, the patient remains
asymptomatic, with a serum creatinine of 123 mmol/l,
urea nitrogen 7 mmol/l, and creatinine clearance of
82 ml/min; urinalysis is normal in the absence of pro-
teinuria and haematuria. A renal Doppler ultrasono-
graphic study showed disappearance of arteriovenous
fistulae. The treatment of the patient includes 10 mg
of prednisone on alternate days, and the blood pressure
is normal with 20 mg of enalapril and 30 mg of nife-
dipine daily.
Discussion
PAN is a necrotizing vasculitis of medium-sized vessels.
Its origin is unknown. It may have an acute or subacute
course [4] and cause formation of multiple aneurysms
[1]. The clinical picture is characterized by fever and
malaise, with peripheral neuropathy, arthralgia, myal-
gia, and visceral involvement (gastrointestinal tract,
kidneys, and heart) [1]. Renal involvement occurs in
80–90% of patients, characterized by haematuria and
loin pain due to renal infarction or renal impairment
related to ischaemia [5]. It is usually a systemic disease
and the renal-limited form is rare [6 ]. Hypertension is
common, affecting 25–71% of patients [1], and it is
J. L. Górriz et al.
usually related to increased levels of plasma renin
activity [7]. The diagnosis can be established by histo-
logy, e.g. biopsy of an affected organ, usually striated
muscle [8], or by renal biopsy. The hallmark is involve-
ment of medium-sized arteries. Another typical sign is
the presence of aneurysms on arteriography [1,8].
Our case was remarkable because he presented with
renal manifestations only, i.e. haematuria, loin pain,
hypertension and subacute renal failure. The misdia-
gnosis of renal colic was made because of flank pain
and a history of renal lithiasis. Progressive renal func-
tion impairment at the time of the the second admission
led to reversal of the diagnosis. Renal biopsy strongly
suggested the possibility of PAN, a diagnosis which
was confirmed by arteriography.
In our patient high renin plasma levels were detected,
as has been described by other authors, related to focal
ischaemia, which causes activation of the renin–angiot-
ensin system analogous to the changes caused by renal
artery stenosis [7]. Thus subacute renal failure with
high plasma renin in the absence of malignant hyper-
tension or bilateral renal artery stenosis is consistent
with the possibility of PAN.
Although ANCA were negative in our patient, they
are present in 25–50% of PAN patients [9]. The
prevalence is lowest in cases that are not associated
with hepatitis B virus infection [4]. Nevertheless, the
use of differing diagnostic criteria in PAN studies
reported in the literature makes it difficult to assess
the true prevalence of ANCA in these patients.
PAN treatment includes corticosteroids, but most of
authors advocate adding a cytotoxic agent, usually
cyclophosphamide [10]. The addition of plasmapher-
esis to corticosteroids and cyclophosphamide was not
followed by significant differences in the 5-year patient
survival [8]. The outcome was poor in untreated
patients and in those with impaired renal function on
admission [8]. So early diagnosis and treatment are
crucial. In fact the delay of a month to establish the
correct diagnosis in our patient led to severe renal
functional impairment, which fortunately was revers-
ible. Although an arteriovenous fistula can be a com-
plication of a renal biopsy, an increased risk of bleeding
and arteriovenous fistulae formation has been reported
in PAN patients [11]. In our case an arteriovenous
fistulae was detected after the kidney biopsy, but it
healed spontaneously.
In conclusion, PAN may have an uncommon pre-
sentation as a renal-limited form presenting with loin
pain and haematuria.
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Received for publication: 13.5.97
Accepted in revised form: 11.7.97