AJR 2001; 176:116-118
© American Roentgen Ray Society
Multiple Renal Masses as Initial Manifestation of Wegener's Granulomatosis
Eduardo Ruiz Carazo1,
Antonio Medina Benitez1,
Genaro López Milena1,
Jesús Rabaza Espigares1,
Laura León2 and
Belgica Marquez3
1
Department of Radiology, University Hospital Virgen de las Nieves, Avenida
Coronel Muñoz S/N, E-18012 Granada,
Spain.
2
Department of Internal Medicine, University Hospital Virgen de las Nieves,
E-18012 Granada, Spain.
3
Department of Pathology, University Hospital San Cecilio, Avenida del Doctor
Oloriz, N°16, E-18012 Granada, Spain.
Received April 4, 2000;
accepted after revision June 12, 2000.
Address correspondence to E. R. Carazo.
Introduction
Wegener's granulomatosis is a multisystem disease characterized by
necrotizing vasculitis of the upper respiratory tract and is frequently
associated with glomerulonephritis. Renal involvement is often a late
complication and manifests as a segmental and focal glomerulonephritis,
sometimes accompanied by a granulomatous necrotizing vasculitis. On rare
occasions this renal disease adopts the form of a single renal mass
[1]. We describe a patient in
whom the disease initially manifested as bilateral renal masses. To our
knowledge, no similar case reports have been published.
Case Report
A 29-year-old man came to the emergency department with a 2-week history of
pain in the left flank, fever, and night sweats. Laboratory analysis showed
leukocytosis (WBC, 19.6 x 109/L) and neutrophilia (15 x
109/L); renal function was not affected. He was diagnosed as having
acute pyelonephritis and antibiotics were prescribed. Three days later, he
returned to the emergency department for persistence of the symptoms. Urine
cultures were negative. Excretory urography showed pruning of the upper and
middle caliceal groups of the left kidney. CT revealed multiple bilateral
renal masses, two on the right kidney and two on the left. These masses were
round and of various sizes. The largest mass was at the upper pole of the left
kidney, which was enlarged, with a decreased nephrogram compared with that of
the right kidney. This mass was mildly hypodense (23 H [not shown]) on
unenhanced CT. The normal parenchyma showed attenuation values of 35 H. After
the administration of IV contrast material, the lesion appeared as an
irregularly marginated area of nonperfusion, with a thin perfusing cortical
rim. The three other masses were smaller (1-2.5 cm). On unenhanced CT images,
the largest of the three masses, localized at the upper pole of the right
kidney, was mildly hypodense (25 H) and the other two were isodense with the
renal parenchyma. On IV contrast—enhanced CT, these masses showed
minimal or absent enhancement in their central portion, with a poorly defined
halo of greater perfusion at the periphery (Figs.
1A and
1B). Color and power Doppler
sonography showed that the masses were isoechogenic with the renal cortex and
without vascularization (Fig.
1C).
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Fig. 1A. —29-year-old man with bilateral renal masses. CT scans after
IV administration of contrast material show hypodense bilateral renal masses
with poorly defined borders (arrows). Left kidney is enlarged and its
function diminished.
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Fig. 1B. —29-year-old man with bilateral renal masses. CT scans after
IV administration of contrast material show hypodense bilateral renal masses
with poorly defined borders (arrows). Left kidney is enlarged and its
function diminished.
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Needle biopsy of the largest mass in the left kidney revealed signs of
focal interstitial nephritis with lymphoplasmocyte infiltration and edema
accompanied by tubular atrophy, patchy interstitial fibrosis, and glomerular
sclerosis, which suggested an immune process without providing a diagnosis.
However, biopsy results ruled out a neoplastic or infectious origin of the
lesions. Serologic analysis for cytoplasmic antineutrophil autoantibodies was
positive, which indicated a diagnosis of Wegener's granulomatosis. The patient
was treated with corticoids and cyclophosphamide with a good clinical
response.
Follow-up CT 8 months after the initial emergency consultation showed
resolution of the right renal masses. However, the left kidney involvement was
greater, with extension of the masses to the perirenal space
(Fig. 1D). An arteriogram
obtained immediately after the CT revealed stenosis of the left renal artery,
with irregularities in the caliber and pruning of its branches suggestive of
vasculitis (Fig. 1E). A left
nephrectomy was performed. Histologic examination of the specimen revealed
glomerulonephritis and granulomatous necrotizing vasculitis affecting small-
and medium-caliber vessels and the renal artery, and extension of the vascular
process to the perirenal space.
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Fig. 1D. —29-year-old man with bilateral renal masses. IV
contrast—enhanced CT scan 8 months after A and B shows
disappearance of right kidney lesions and progression of left kidney lesions,
with involvement of perirenal space and absence of function. Size of left
kidney was markedly reduced.
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Fig. 1E. —29-year-old man with bilateral renal masses. Left renal
arteriogram obtained immediately after D shows stenosis of renal artery
(arrow) with focal irregularities and pruning of upper pole branches
(arrowheads).
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Fifteen months after his first emergency department consultation, the
patient's general health status is good and his renal function is normal. He
remains on cytotoxic (cyclophosphamide) and corticoid (prednisone) therapy. He
has experienced only one extrarenal manifestation of vasculitis, an upper
limbic scleritis in the right eye, and his respiratory system is
unaffected.
Discussion
Wegener's granulomatosis is a multisystem disease of unknown etiology
characterized by necrotizing granulomatous vasculitis that affects the upper
and lower respiratory systems and the kidneys; however, other organs also can
be affected. Respiratory manifestations are the most frequent symptoms and
usually mark the onset of the disease. There are very few cases in which renal
symptoms predominate. Until 1994, only 23 cases of severe renal disease
without respiratory manifestations, as occurred in our patient, had been
reported [1].
Wegener's granulomatosis in the kidney is characterized by a focal and
segmental glomerulonephritis, sometimes accompanied by a necrotizing
granulomatous vasculitis. Only rarely is there a mass in the kidney as a
consequence of this disease. Maguire et al.
[2] published the first report
of a patient with a large right renal mass that distorted the upper pole
calix. There have been five subsequent reports of renal masses produced by
Wegener's granulomatosis [1,
3,4,5,6].
A solitary lesion occurred in all six cases and was the initial manifestation
of the disease in only two of the cases
[1,
4]. Scant radiologic
information was provided on these six cases.
To our knowledge, ours is the first report of a patient presenting with
multiple bilateral solid renal masses as a manifestation of Wegener's
granulomatosis. The appearance of the masses varied according to their size.
On unenhanced CT images, the two largest masses were mildly hypodense and the
two smallest were isodense with the renal parenchyma. IV contrast-enhanced CT
showed the largest mass to have an absence of perfusion and to be irregularly
marginated with a thin perfusing cortical rim, similar to that observed in
renal infarction. The other three masses showed a peripheral enhancing halo
poorly demarcated from the adjacent parenchyma. This distinct appearance may
be the result of a greater or lesser predominance of the granulomatous or
vascular processes that coexist in this disease. In fact, after successful
treatment, the patient's right kidney showed no cortical scars associated with
renal infarction. The inflammatory and vascular involvement of the perirenal
space occurred at a late stage of the disease. The patient described by
Schydlowsky et al. [4] also
showed this involvement, but to a lesser degree than that seen in our
patient.
Sonography revealed that the masses were isoechogenic with the renal cortex
and could be distinguished only by the mass effect they produced and by the
absence of vascularization on color and power Doppler images.
The imaging findings reported above are not diagnostic. The differential
diagnosis should include other diseases that produce bilateral renal masses,
especially metastasis and lymphoma. Metastatic disease is often disseminated
by the time renal involvement is identified. Renal lymphoma is most often seen
with multisystemic disseminated lymphoma or as tumor recurrence. Renal
lymphoma may also be seen in patients who are immunocompromised or, rarely, as
primary disease [7]. Bilateral
renal masses can also originate from abscesses, synchronous renal tumors, or
infarctions. Synchronous renal tumors should also be considered, particularly
if there are features suggestive of von Hippel-Lindau disease. Infarctions
show well-defined borders and a triangular morphology, with a characteristic
enhanced external border caused by the presence of cortical collaterals.
In this case report we highlight the favorable response of some of the
masses to steroid and immunosuppressive treatment, which caused the
disappearance of the masses in the right kidney. Nevertheless, the largest
mass continued to enlarge until it infiltrated the entire left kidney and the
perirenal space.
In conclusion, the presentation of Wegener's granulomatosis as multiple
renal masses is rare. However, in the appropriate clinical context, systemic
vasculitis should be considered in the diagnosis of multiple bilateral renal
masses.
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