AJR 2000; 174:1597-1598
© American Roentgen Ray Society
Wegener's Granulomatosis Presenting As a Renal Mass
Kyrsten D. Fairbanks1,
David B. Hellmann2,
Elliot K. Fishman3,
Syed Z. Ali4 and
John H. Stone5
1
Department of Medicine, 600 N. Wolfe St., Nelson Tower 110, Baltimore, MD
21287.
2
Department of Medicine, Division of Rheumatology, Johns Hopkins University,
1830 E. Monument St., Ste. 9030, Baltimore, MD 21205.
3
Department of Radiology, Johns Hopkins University, Johns Hopkins Outpatient
Center, Caroline St., Rm. 3254, Baltimore, MD 21205.
4
Department of Pathology, Division of Cytopathology, Johns Hopkins University,
600 N. Wolfe St., Meyer 434, Baltimore, MD 21287.
5
Department of Medicine, Division of Rheumatology, Johns Hopkins University,
Johns Hopkins Vasculitis Center, 1830 E. Monument St., Ste. 7500, Baltimore,
MD 21205.
Received August 24, 1999;
accepted after revision October 19, 1999.
Address correspondence to J. H. Stone.
Introduction
Wegener's granulomatosis is a form of systemic necrotizing vasculitis that
primarily involves the upper and lower respiratory tracts and the kidneys.
Renal manifestations, the most ominous manifestation of this disease,
typically consist of a segmental necrotizing glomerulonephritis that often
leads to rapidly progressive renal failure
[1]. This type of renal
involvement is usually unapparent on radiography. Recently, an association
between Wegener's granulomatosis and renal cell carcinoma was reported
[2], but renal mass as a
presentation of Wegener's granulomatosis is rare
[3]. We describe a patient with
Wegener's granulomatosis who presented with profound constitutional symptoms
and CT findings that suggested the presence of a renal mass.
Case Report
In November 1998, a 68-year-old man presented with a 6-month history of
fever, a 22-kg weight loss, and ear pain. His symptoms had begun in May 1998
with bilateral ear fullness and decreased hearing. Despite myringotomies and
antibiotic treatment, his otalgia and progressive hearing loss continued.
Chest radiography revealed a right lower lobe infiltrate. Bronchoscopy with
biopsy of the infiltrate revealed nonspecific inflammation but no evidence of
malignancy. The patient was prescribed a brief course of corticosteroids,
which led to resolution of his fever. However, because of persistent otalgia
and concern about his weight loss, he was admitted for further evaluation.
On examination, he was febrile to 38.5°C and had tenderness to
palpation behind the left ear. His hearing had markedly decreased bilaterally.
He had a normochromic, normocytic anemia (hematocrit, 34.5%) and a moderate
leukocytosis (WBC, 16.3 x 103/µl). His serum creatinine
was 61 µmol/l, and a urinalysis showed no proteinuria, hematuria, or RBC
casts. The erythrocyte sedimentation rate was 115 mm/hr (normal, <20
mm/hr), and a serum test for antineutrophil cytoplasmic antibodies was
positive by immunofluorescence in a peri-nuclear (pANCA) pattern (titer 1:20).
CT of the chest showed an irregular 2-cm mass in the left lung apex, moderate
right hilar lymphadenopathy, and a posterior segment infiltrate in the right
lower lung. CT of the temporal bones showed coalescence of the mastoid air
cells with air-fluid levels, thickening and retraction of the tympanic
membranes, and inflammation of the paranasal sinuses. Abdominal CT, performed
as part of a malignancy workup, revealed an infiltrating process in the left
kidney with decreased enhancement on the nephrogram compared with that of the
right kidney. There was a dominant infiltrating mass in the posterior portion
of the left kidney with a second smaller lesion also noted
(Fig. 1A). This was suspicious
for an infiltrating tumor such as lymphoma or transitional cell carcinoma,
with renal cell carcinoma being less likely. Infiltration caused by
inflammation or infection could not be excluded. A percutaneous fine-needle
aspiration of the mass revealed granulomatous inflammation with scattered
multinucleated giant cells, vasculitis, and fibrinoid necrosis, consistent
with Wegener's granulomatosis (Figs.
1B and
1C). The patient was given
prednisone (40 mg/day) and methotrexate (15 mg/week). Three months later,
follow-up abdominal CT showed resolution of the infiltrating process, and the
patient had no clinical evidence of active Wegener's granulomatosis.
View larger version (112K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1A. —68-year-old man who presented with mastoiditis, pulmonary
infiltrates, fever, and weight loss. Axial contrast-enhanced CT scan reveals
left renal mass. Note 4 x 3 cm mass (straight arrow) in
posterior aspect of left kidney's mid pole, and 1.5 x 2 cm mass
(curved arrow) laterally. Also note lymphadenopathy in paraaortic and
aortocaval regions at level of renal hila, with nodes measuring as large as 1
cm in diameter.
|
|
View larger version (154K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1B. —68-year-old man who presented with mastoiditis, pulmonary
infiltrates, fever, and weight loss. Photomicrograph obtained at fine-needle
aspiration shows multinucleated giant cells and collection of histiocytes.
(Diff Quik [Dade Behring, Newark, DE], x200)
|
|
View larger version (121K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1C. —68-year-old man who presented with mastoiditis, pulmonary
infiltrates, fever, and weight loss. Photomicrograph from core biopsy shows
extensive necrotizing inflammation, glomerular involvement with focal
sclerosis, and fibrinoid necrosis of small blood vessel. (H and E,
x100)
|
|
Discussion
This case illustrates Wegener's granulomatosis of the kidney presenting as
a renal mass that mimicked a neoplastic process similar to lymphoma or renal
cell carcinoma. The patient's illness was characterized by sinus, ear, and
lung findings consistent with Wegener's granulomatosis. However, involvement
in these organ systems was overshadowed by prominent constitutional symptoms,
particularly the weight loss. These constitutional symptoms, combined with the
CT finding of a renal mass, strongly suggested an underlying malignancy.
Biopsy of the renal mass revealed not the expected renal cell carcinoma, but
rather the classic pathologic triad of Wegener's granulomatosis: granulomatous
inflammation, fibrinoid necrosis, and vasculitis.
Although renal disease is present in only 20% of patients at the time of
diagnosis, kidney involvement eventually occurs in approximately 80% of
patients with Wegener's granulomatosis
[1]. The usual presentation of
Wegener's granulomatosis in the kidneys is rapidly progressive
glomerulonephritis with an "active" urine sediment, particularly
RBC casts. In this acute setting, the kidneys usually have fewer abnormal
findings on imaging. The onset of renal involvement signals the development of
severe, life-threatening disease, requiring urgent intervention with
immunosuppressive therapy. The diagnosis of Wegener's granulomatosis is rarely
made by cytopathology except for occasional fine-needle aspirations of
pulmonary nodules. We are unaware of any descriptions in the medical
literature of Wegener's granulomatosis in the kidneys diagnosed by
cytopathology. In this patient, the fine-needle aspiration diagnosis was
critical because it led to successful medical treatment. The core renal biopsy
supplemented the cytopathologic diagnosis in this case, showing suppurative
granulomas, necrotizing inflammation, fibrinoid necrosis of blood vessels, and
glomerulonephritis (glomerular obsolescence).
A recent case-control study found an association between Wegener's
granulomatosis and renal cell carcinoma, with an odds ratio of 8.7 compared
with a group of rheumatoid arthritis controls
[2]. Simultaneous occurrence of
these two diseases has been reported in several cases
[3,4,5,6,7,8],
emphasizing the importance of confirming the diagnosis of suspected Wegener's
granulomatosis by biopsy and of repeating imaging studies to confirm the
resolution of mass lesions after appropriate treatment.
References
-
Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener's granulomatosis:
an analysis of 158 patients. Ann Intern Med
1992;116:488
-498
-
Tatsis E, Reinhold-Keller E, Steindorf K, Feller AC, Gross WL.
Wegener's granulomatosis associated with renal cell carcinoma.
Arthritis Rheum
1999;42:751
-756[Medline]
-
Villa-Forte A, Hoffman GS. Wegener's granulomatosis presenting with
a renal mass. J Rheumatol
1999;26:457
-458[Medline]
-
Maguire R, Fauci AS, Doppman JL, Wolff SM. Unusual radiographic
features of Wegener's granulomatosis. AJR
1978;130:233
-238[Abstract]
-
Schapira HE, Kapner J, Szporn AH. Wegener's granulomatosis
presenting as renal mass. Urology
1986;28:307
-309[Medline]
-
Schydlowsky P, Rosenkilde P, Helin P, Braendstrup O. Wegener's
granulomatosis presenting with a tumor-like lesion in the kidney.
Scand J Rheumatol
1992;21:204
-205[Medline]
-
Smith DJ, Milroy CM, Chapple CR. An unusual renal mass: Wegener's
granulomatosis. Br J Urol
1993;72:980
-981[Medline]
-
Boubenider SA, Akhtar M, Nyman R. Wegener's granulomatosis limited
to the kidney as a mass-like lesion. Nephron
1994;68:500
-504[Medline]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
N. Takahashi, A. Kawashima, J. G. Fletcher, and S. T. Chari
Renal Involvement in Patients with Autoimmune Pancreatitis: CT and MR Imaging Findings
Radiology,
March 1, 2007;
242(3):
791 - 801.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Introduction
Case Report
