ORIGINAL ARTICLE
Integration of 2-deoxy-2-[18F] fluoro-D-glucose PET/CTinto clinical management of patients with Wegener’sgranulomatosis
Ozlem Ozmen • Ebru Tatci • Atila Gokcek •
Deniz Koksal • Yeliz Dadali • Esra Ozaydin •
Nuri Arslan
Received: 7 May 2013 / Accepted: 29 August 2013 / Published online: 15 September 2013
� The Japanese Society of Nuclear Medicine 2013
Abstract
Objective Wegener’s granulomatosis (WG) is a rare dis-
order characterized by granulomatous necrotizing vasculi-
tis which mainly affects small- and medium-sized vessels.
While the classical triad of involvement is upper and lower
respiratory system and glomerulonephritis, WG may
involve any organ or system in the body. The aim of our
study was to investigate the role of positron emission
tomography/computerized tomography (PET/CT) both in
the initial evaluation and follow-up of patients with WG.
Methods We retrospectively evaluated PET/CT data from
13 patients (6 males; 7 females) with a mean age of
45 ± 12.4 years (range 28–63) who underwent either ini-
tial evaluation (n = 12) or response evaluation (n = 2) by
conventional imaging methods and FDG with PET/CT.
PET/CT images were both visually and quantitatively
evaluated. The demographic data, clinical and laboratory
findings of each patient were also recorded from the hos-
pital files.
Results Lung (n = 13), parapharyngeal space (n = 8),
nose (n = 8), and ear (n = 3) were the most common
disease sites detected on PET/CT. The entire initial eval-
uation patients had either solitary or multiple pulmonary
nodular/mass lesions with marked increased FDG uptake
(mean SUVmax 12 ± 4, range 3.53–19.51) on PET/CT.
There was no significant pathological FDG uptake in
patients consistent with complete treatment response after
appropriate immunosuppressive therapy. PET/CT clearly
demonstrated unexpected disease sites besides the respi-
ratory system, with WG involvement except kidneys.
Possibly due to physiological urinary excretion of FDG,
urine analysis, BUN and creatinine levels were accepted
still the best way for diagnosis of renal involvement.
Conclusion FDG with PET/CT is a valuable tool in the
management of patients with WG for a more accurate
clinical evaluation regarding disease extension and treat-
ment response.
Keywords PET/CT � Wegener’s granulomatosis �Vasculitis
Introduction
Wegener’s granulomatosis (WG) is a rare disorder char-
acterized by chronic granulomatous necrotizing vasculitis
which mainly affects small- and medium-sized vessels [1].
WG has a broad clinical spectrum ranging from localized
disease of respiratory tract to severe life-threatening dis-
ease with involvement of multiple organs [2]. Although,
O. Ozmen (&) � E. Tatci
Department of Nuclear Medicine, Ataturk Chest Diseases
and Thoracic Surgery Training and Research Hospital,
Sanatoryum Cad., 06280 Kecioren, Ankara, Turkey
e-mail: [email protected]
A. Gokcek � Y. Dadali
Department of Radiology, Ataturk Chest Diseases and Thoracic
Surgery Training and Research Hospital, Ankara, Turkey
D. Koksal
Department of Chest Diseases, Ataturk Chest Diseases and
Thoracic Surgery Training and Research Hospital, Ankara,
Turkey
E. Ozaydin
Department of Pathology, Ataturk Chest Diseases and Thoracic
Surgery Training and Research Hospital, Ankara, Turkey
N. Arslan
Department of Nuclear Medicine, Gulhane Military Medical
Academy and Medical Faculty, Ankara, Turkey
123
Ann Nucl Med (2013) 27:907–915
DOI 10.1007/s12149-013-0769-6
WG is usually presented with a classical triad of involve-
ment of upper and lower respiratory system and glomeru-
lonephritis, it may involve systemically any part of the
body [3].
The diagnosis of WG is based on a combination of
clinical and laboratory findings: the presence of upper
respiratory tract symptoms, laboratory findings indicative
of glomerulonephritis, and the presence of circulating an-
tineutrophil cytoplasmic antibodies (ANCA) directed
against proteinase 3 (c-ANCA) or myeloperoxidase (p-
ANCA). While ANCA positivity is the most essential
laboratory finding for definitive diagnosis, c-ANCA usually
distinguishes WG from other granulomatous disease.
However, while c-ANCA is positive in 85–90 % of
patients with active WG, its positivity rate may decrease up
to 60 % in patients with limited disease in lungs [4, 5].
Besides that, while ANCA test positivity confirms the
diagnosis, negative results do not exclude the disease [6, 7].
Therefore, it may need to identify necrotizing granuloma-
tous vasculitis on biopsy specimens to confirm the disease.
Lung nodules and masses are the most common radio-
logical findings seen at presentation in up to 90 % of
patients with WG. While, the size of nodules can range
from a few millimeters to more than 10 cm in diameter,
most of the lung nodules are greater than 2 cm in size.
These nodules are usually presented with cavitations, thick
walls and irregular inner margins on computerized
tomography (CT). Primary and metastatic lung neoplasm
as well as granulomatosis disease is the most common
differential diagnosis for those lung nodules.
WG is associated with considerable morbidity and
mortality due to irreversible organ damages caused by
inflammatory injury and long-term immunosuppressive
therapy. Irreversible organ damages caused by inflamma-
tory injury may lead to death in untreated or inadequately
treated patients. Therefore, early diagnosis of WG as well
as accurate assessment of the disease extend for an
appropriate treatment is of great importance. While corti-
costeroids, methotrexate and azathioprine are adequate in
limited disease localized to the upper airways, cyclophos-
phamide is the first-line therapy in advanced cases [8]. In
addition, in order to minimize the adverse effects of
immunosuppressive therapy, the degree of immunosup-
pression is determined by the severity of disease and
c-ANCA titers.
2-Deoxy-2-[18F] fluoro-D-glucose (FDG) with positron
emission tomography/computerized tomography (PET/CT)
is a metabolic imaging technique which has become an
essential tool for the management of patients with various
types of cancer. Although, the use of PET/CT in infection
and inflammation is not uncommon, the clinical impact of
it has not been well documented as is the case in patients
with malignant disease. So, a few case reports and limited
number of patient series have been presented, showing that
WG lesions exhibit relatively high FDG uptake which
diminish after successful treatment [9, 10]. In this study we
aimed to investigate the role of PET/CT in the evaluation
and follow-up of patients with WG.
Materials and methods
Patients
We retrospectively evaluated 14 PET/CT studies and rel-
evant laboratory data from 13 patients (6 males; 7 females)
with a mean age of 45 ± 12.4 years (range 28–63) with
WG between 2009 and 2012. 12 patients were referred for
the evaluation of lung nodule(s) or mass lesions, and later
on diagnosed as WG. One patient was referred for the
evaluation of immunosuppressive therapy response for
WG, because of residual parenchymal lung lesions. There
was only 1 patient (Case 11) who had initial and a follow-
up PET/CT study 27 months later after the diagnosis. PET/
CT findings were visually evaluated by two radiologists
and two nuclear medicine specialist. On rare occasions
when the readings differed, consensus was obtained based
on a discussion with the help of another nuclear medicine
specialist. SUVmax values were automatically calculated
by drawing area of interest of detected lesions. PET/CT
findings were correlated with existing radiographic exam-
inations such as ultrasound, contrast-enhanced CT and
MRI. ANCA titer measurements and other laboratory
studies (serum creatine, urine analysis) were done within
2 weeks of PET/CT imaging. The serologic and histopa-
tologic findings and referring reason for PET/CT are shown
in Table 1.
The diagnosis of patients with WG in our study group
was confirmed by clinical, laboratory findings, and histo-
pathologic examinations based on the criteria of American
College of Rheumatology [11]. The demographic data,
clinical and laboratory findings of our patient population
were recorded from the hospital files.
Informed consent was deemed as a retrospective study
using existing hospital records, documents and data, which
belong to patients referred to FDG PET for initial staging
due to lung masses highly suspicion for lung cancer. This
study was approved by Institutional Review Board
Committee.
FDG PET/CT imaging
PET/CT evaluation was carried out with an integrated PET/
CT scanner Siemens, Biograph-6-HI-REZ (Siemens Med-
ical Solutions, Knoxville, TN, USA). Patients were
instructed for fasting at least 6 h before the examination.
908 Ann Nucl Med (2013) 27:907–915
123
After confirmation of a normal peripheral blood glucose
level (\180 mg/dL), patients were received an intravenous
injection of 370–555 MBq (10–15 mCi) FDG and rested
for 60 min before the scan. Oral contrast material was used
in all patients for better visualization of the intestinal
lumens. PET data were acquired from the top of skull to the
upper thigh with the arms up position. The maximum
standardized uptake value (SUVmax) corrected for body
weight was computed by standard methods from the
activity in the most intense voxel in the three-dimensional
tumor region from the transaxial whole-body images on
attenuation-corrected PET/CT images.
Statistical analysis
SPSS for windows release 19.0 package program was used
to carry out the descriptive statistics of the study subjects
that were expressed in terms of frequency, mean and
standard deviation.
Results
Lung (n = 12), parapharyngeal space (n = 8), nose
(n = 8) and ear (n = 3) were the most common disease
sites having increased FDG uptake on PET/CT in initial
evaluation patients. Besides commonly seen involvement
sites for those patients with WG, PET/CT showed addi-
tional organ involvements such as great vessels, ear,
spleen, duodenum, trachea, skin and adrenals which were
not documented before by conventional methods. These
were great vessels (n = 3), kidney (n = 3), spleen (n = 2),
duodenum (n = 1), trachea (n = 1), skin (n = 1) and
adrenals (n = 1) showing increased metabolic activity with
FDG (Table 2).
Radiological imaging (and/or ultrasound, contrast-
enhanced CT) before or after PET studies were re-evalu-
ated for the involvement of kidney, adrenals and spleen.
Splenic involvement detected with FDG PET/CT was
confirmed by MRI after completion of PET/CT (Fig. 1a,
b). Furthermore, endoscopy which was performed for
detection of possible gastrointestinal bleeding revealed
duodenal hemorrhage consistent with increased duodenal
FDG uptake on PET/CT. However, large vessel involve-
ment could not be demonstrated by conventional imaging
methods in this patient. Because of ethical concerns, his-
topathologic confirmation was not possible in every
patient/site and performed only if it was clinically
indicated.
All of the untreated patients with pulmonary system
involvement have either solitary (8 %) or multiple (92 %)
nodular/mass lesions on CT. Most of these pulmonary
lesions (83 %) were presented with cavitations. The largest
diameter of the pulmonary lesions was 9 cm in size (size
range 1.7–9 cm). Between the untreated patients, 4 (33 %)
consolidation, 5 (42 %) ground glass opacity and 2 (17 %)
atelectasis were present. Radiographic features of pul-
monary pathologies and quantitative FDG PET/CT results
are given in Table 3. Nine of 12 (75 %) patients in initial
evaluation studies showed increased FDG uptake in lymph
nodes consistent with WG on PET/CT. On the other hand,
there was only one patient showing increased FDG uptake
in trachea consistent with WG (Fig. 2).
All disease sites in patients showed moderate to the
marked FDG uptake on PET/CT before initiation of any
treatment with mean SUVmax value of 12 ± 4 (range
Table 1 Clinical, serologic and histopathological findings and PET/CT results in patients with Wegener’s granulomatosis
Case no. Age (years) Sex Biopsy site ANCA serology Referring reason for PET/CT
1 44 F Nose c-ANCA Diagnosis
2 47 M TTB c-ANCA Diagnosis
3 56 F TTB p-ANCA Diagnosis
4 37 M FOB c-ANCA Diagnosis
5 30 F – c-ANCA Diagnosis
6 56 M Skin c-ANCA Diagnosis
7 44 M Nose c-ANCA Diagnosis
8 63 M – c-ANCA Diagnosis
9 35 F TTB c-ANCA, p-ANCA Diagnosis
10 47 F TTB Negative Therapy response
11 28 F FOB p-ANCA Diagnosis
Negative Therapy response
12 57 M TTB p-ANCA Diagnosis
13 63 F TTB c-ANCA Diagnosis
TTB transthoracic tru-cut biopsy, FOB fiberoptic bronchoscopic biopsy
Ann Nucl Med (2013) 27:907–915 909
123
Table 2 Non-pulmonary disease sites and FDG PET/CT results in patients with Wegener’s granulomatosis
Case
no.
Age
(years)
Non-pulmonary disease sitesb Non-pulmonary disease sites detected only with FDG
PET/CT
SUVmax valuesc
1 44 Ear, nose, eye, facial nerve,
kidney
Parapharyngeal space (7.42)
2 47 Nose, kidney Parapharyngeal space/great vessels (9.82)/(13.46)
3 56 Kidney Parapharyngeal space/nose/great vessels (3.97)/(4.66)/(7.48)
4 37 Nose, kidney, spleen Parapharyngeal space/ear/great vessels (11.89)/(8.12)/(4.42)
5 30 Nose, kidney
6 56 Kidney, skina Parapharyngeal space, nose, spleen, duodenum (9.29)/(3.57)/(3.19)/
(6.43)
7 44 Nose Parapharyngeal space, trachea, skin (4.76)/(9.59)/(3.46)
8 63 Kidney Parapharyngeal space, ear, nose (4.11)/(9.89)/(7.75)
9 35 Kidney
10 47
11 28 Parapharyngeal space, adrenals (5.68)/(13.03)
12 57 Kidney
13 63 Kidney Nose (6.91)
a Lesions outside the viewing areab Detected with clinical, laboratory, radiological or histopathological resultsc Non pulmonary disease sites only detected with FDG PET/CT
Fig. 1 a At axial PET/CT image, a large hypointense hematoma and
septal densities (white arrow) in the spleen and increased FDG uptake
in the periphery of it are observed (dark arrow). b Hypo-intense
appearance of lesions in the spleen (white arrow) on corresponding
post-contrast T1-WI enhancement plan axial MRI sequences
910 Ann Nucl Med (2013) 27:907–915
123
3.53–19.51). Although, upper respiratory system involve-
ment was clinically evident in 5 patients (45 %), a total of
11 patients (92 %) had pathological metabolic activity
consistent with WG involvement on PET/CT. Besides that,
while pulmonary system involvement was equally detected
on CT and PET/CT scans, SUVmax values helped us to
differentiate active inflammation from chronic parenchy-
mal pathologies as a marker of metabolic activity. While
10 patients (83 %) had renal involvement based on the
laboratory findings such as urine analysis, BUN and cre-
atinine levels, PET/CT could detect only 3 (25 %) of them.
Two patients referred for the evaluation of treatment
response with PET/CT. The first patient (Case no. 11,
Table 1) had follow-up PET/CT scan after immunosup-
pressive therapy. All disease sites with high FDG uptake on
previous PET/CT were completely resolved on follow-up
PET/CT scan consistent with treatment response. The
second patient (Case no. 10) who was on maintenance
therapy had a pulmonary mass lesion 4.5 cm in diameter
with a low FDG activity (SUVmax 2.2). The comparison of
conventional clinical and radiological methods and FDG
PET/CT in the evaluation of organ and systems regarding
involvement of Wegener’s granulomatosis are given in
Table 2.
Discussion
FDG activity in a lesion is the indicator of high glycolytic
activity, which is a property of not only malignant lesions,
but also the inflamed tissues. On the other hand, detection
of extent and severity of disease status may be very
important for most of the non-oncologic diseases, such as
vasculitis. On the contrary to anatomical changes in vessel
Table 3 Radiologic findings of pulmonary pathologies and qualitative FDG PET/CT results in patients with Wegener’s granulomatosis
Case
no.
Largest
lesion
size
(cm)a
SUVmax Number of
pulmonary
nodules
Cavitation Consolidation Distribution Ground
glass
opacity
Mediastinal
adenopathy
Atelectasis Tracheal
involvement
1 9 12.20 Solitary ? – Bronchovascular – – – –
2 5.4 14.14 Multiple – – Apical ? – – –
3 1.7 3.53 Multiple ? – Peripheral – ? ? –
4 3.1 14.38 Multiple ? – Random – ? – –
5 3.7 19.51 Multiple ? ? Apical – ? ? –
6 8 10.31 Multiple ? – Apical ? ? – –
7 6.7 8.96 Multiple ? ? Random ? ? – ?
8 6.4 13.31 Multiple ? – Random – ? – –
9 2.5 12.92 Multiple ? – Random – ? – –
10 4.5 2.20 Solitary ? ? Apical – – ? –
11 2.2 14.10 Multiple – ? Random ? – – –
– aN/A – – – – – ? –
12 3.3 8.72 Multiple ? – Random ? ? – –
13 2.4 8.26 Multiple ? ? Random – ? – –
a N/A: not applicable: parenchymal band formation with no discrete nodular lesion
Fig. 2 An axial PET/CT image showing markedly increased FDG
uptake (SUVmax 9.59) in the trachea (dark short arrows) and
adjacent soft tissue corresponding to the irregular densities seen on
CT slices (white long arrows) in a patient with tracheal involvement.
There is also a pulmonary nodule with markedly increased FDG
uptake on the right upper lobe (dark long arrows)
Ann Nucl Med (2013) 27:907–915 911
123
walls, inflammation in the vessel wall cannot be detected in
the early phase of vasculitis due to lack of substantial
anatomical changes by conventional imaging methods.
Therefore, it is difficult to accurately diagnose, and eval-
uate the extension and severity of disease in patients with
vasculitis by conventional imaging methods. It is also
problematic to distinguish active inflammatory lesions
from residual anatomical changes secondary to previous
inflammation. However, FDG PET/CT imaging is reported
to be a valuable method in diagnosing and monitoring of
patients with vasculitis such as giant cell arteritis, polym-
yalgia rheumatica, and Takayasu’s arteritis [12]. Walter
et al. [13] reported that FDG-PET imaging had a sensitivity
of 99.7 % and specificity of 99.8 % for the evaluation of
disease extension in large vessel vasculitis. Recently FDG-
PET is used as a sensitive and specific imaging tool for
large vessel vasculitis, especially when performed in
patients not receiving immunosuppressive drugs [14].
Besides that, Beggs and Hain [15] reported that FDG-PET
can assess response to therapy in patients with WG before
any decrease in size of the lesion seen on CT, and can also
detect the disease recurrence before the conventional
imaging methods. In our study, two patients were referred
for the evaluation of treatment response with PET/CT. All
disease sites with high FDG uptake on previous PET/CT
were completely resolved or low uptake on follow-up PET/
CT scan consistent with treatment response. Although, we
do not have pretreatment PET/CT scan and any informa-
tion about the previous FDG activity, this low-level FDG
activity was concluded as treatment response based on our
clinical experience and literature data.
All patients with high FDG uptake on PET/CT were also
ANCA positive in our study group. On the other hand, the
patients who were referred for follow-up PET/CT scan
showed decreased or ceased metabolic activities of lesions,
parallel to diminishing clinical symptoms and ANCA lev-
els after the treatment. PET/CT is of great importance for
diseases accompanied with infection and inflammation
which can cause serious complications if appropriate and
effective treatment is not applied. Disease extension and
severity is one of the most important elements for the
choice of treatment according to the ‘‘BHPR BSR guide-
lines’’ which is developed to serve the management of
patients with ‘‘ANCA associated vasculitis’’ [16]. PET/CT
scan has the ability to detect unexpected sites of involve-
ment allowing a more accurate evaluation of disease
extension and help to institute the appropriate treatment.
Upper respiratory tract involvement is almost present in
all patients with WG [17]. Although, it is preceded by otitis
media, mastoiditis and facial nerve paralysis as in one case
in our patient group (Case no. 1, Table 2), inflammation in
the nasopharyngeal cavity and paranasal sinuses is usually
the first symptom. In some patients who have limited
disease in upper respiratory system that are resistant to
conventional therapies, the diagnosis of vasculitis could not
be possible with only help of laboratory and radiological
findings. On the other hand, Maranhao et al. [18] reported
that the early detection of the upper respiratory tract
involvement may prevent complications such as mastoid-
itis and facial palsy in patients with WG. Similar to pre-
vious reports, 92 % (11/12) of our untreated patient
population showed FDG uptake consistent with upper
respiratory tract involvement (Fig. 3). So, PET/CT scan
could be helpful in demonstrating unexpected disease sites
other than upper respiratory tract as in our study. In this
study, FDG PET/CT determined in 46 % of patient popu-
lation unexpected involvement areas such as great vessels,
ear, spleen, kidney (Fig. 4), duodenum, trachea and skin
which were difficult to detect by conventional imaging
procedures.
Despite clinical and laboratory findings, a tissue diag-
nosis is usually needed for a definitive diagnosis for WG.
However, a definitive histopathological diagnosis could not
be obtained for more than 50 % of cases. So, a PET/CT
scan may help to determine the appropriate biopsy site with
the highest metabolic activity representing active inflam-
mation. WG may develop high mortality and morbidity as
a result of irreversible organ damage due to inflammation
or long-term use of immunosuppressive agents. Moreover,
an unfavorable prognosis was observed in almost up to
90 % of all untreated patients for the first 2 years following
the diagnosis [19]. Perivascular soft tissues in the great
vessels, aneurysm, dissection and/or rupture have been
observed in ANCA-associated vasculitis such as WG [20].
Besides that, about 15 % of patients with WG may show
valvular stenosis and regurgitation on echocardiography.
Concordant with the literature, 3 of 13 patients in our study
group had increased perivascular FDG uptake consistent
with inflammation on the vessel wall and perivascular soft
tissue. PET/CT scan was also found to be suspicious for
vessel dissection in one patient (Fig. 5).
It is well known that the evaluation of renal system
involvement on PET/CT is challenging due to physiologic
urinary excretion of 18F-FDG for those patients with
malignant disease. Similarly, intense physiological FDG
uptake may mask any focal increased FDG uptake in
central nervous system. Therefore, the sensitivity of PET/
CT may not be high enough for patients either having small
renal lesions or limited disease in central nervous system
with WG. On the other hand, one should be aware that a
focal-increased FDG uptake in kidney may be an indicative
of a greater parenchymal destruction associated with
hemorrhage. Decreased renal uptake due to renal failure
may provide the ability to distinguish renal involvement
(Fig. 4). In the present study PET/CT suggested the
involvement of renal system in three patients who have
912 Ann Nucl Med (2013) 27:907–915
123
significantly increased parenchymal renal FDG uptake.
While 2 of 3 patients had renal failure, only one of them
had hematuria and proteinuria indicating subsequent renal
failure. Contrast-enhanced examinations can help detect
lesions. However, due to the existing or possibly
developing renal insufficiency, contrast-enhanced examin-
ations are generally problematic in this group.
Evaluation of response to treatment in patients with WG
is another problematic issue. Herein, we described a patient
(Fig. 6) with increased FDG uptake in the adrenal gland
Fig. 3 Destruction of the nasal septum (white arrow) and intense FDG uptake (SUVmax 16.95) in nasal cavity with ill-defined borders
extending paranasal soft tissue (dark arrow) on corresponding axial PET/CT slices
Fig. 4 FDG PET/CT image showed moderately increased FDG
uptake in posterior upper cortical area of the left kidney which was
separated from physiological urinary activity (dark arrow). Contrast
material is seen in renal pelvis remaining of previous study at CT
imaging (short white arrows). On the other hand, contrast was not
observed at the lesion area. Therefore, urinary activity could be easily
excluded. Consistent with all, hypodense lesion area is seen on
corresponding CT slice (long white arrow)
Fig. 5 Markedly increased FDG uptake in thoracic aorta (dark arrow) and perivascular soft tissue consistent with inflammation on the vessel
wall and perivascular soft tissue (white arrow)
Ann Nucl Med (2013) 27:907–915 913
123
consistent with WG involvement at the diagnosis, resolved
after successful treatment. To best of our knowledge, this is
the first case reported to have adrenal gland involvement
shown by PET/CT scan as well as treatment response.
In conclusion, we may conclude that PET/CT is a useful
modality in the management of patients with WG regarding
initial evaluation for determining the biopsy site as well as
detection the extensiveness of disease to choose an
appropriate treatment, and also monitoring response to
therapy.
Acknowledgments The authors wish to thank Dr. Aysenaz Ozcan,
Dr. Belgin Samurkasoglu, Dr. Nermin Capan, Dr. Nilgun Kalac, Dr.
Sema Canbakan, Dr. Sukran Atikcan, Dr. Yurdanur Erdogan for
referring their patients to our department and cooperation. The
abstract of the manuscript has also been accepted to be presented at
26th Annual Congress of the EANM in October 2013.
Conflict of interest On behalf of all authors, the corresponding
author states that there is no conflict of interest.
References
1. Woywodt A, Matteson EL. Wegener’s granulomatosis—probing
the untold past of the man behind the eponym. Rheumatology
(Oxford). 2006;45(10):1303–6.
2. Frankel SK, Cosgrove GP, Fischer A, Meehan RT, Brown KK.
Update in the diagnosis and management of pulmonary vasculitis.
Chest. 2006;129(2):452–65.
3. Fries JF, Hunder GG, Bloch DA, Michel BA, Arend WP, Cala-
brese LH, et al. The American College of Rheumatology 1990
criteria for the classification of vasculitis. Summary. Arthritis
Rheum. 1990;33(8):1135–6.
4. Hagen EC, Daha MR, Hermans J, Andrassy K, Csernok E,
Gaskin G, et al. Diagnostic value of standardized assays for anti-
neutrophil cytoplasmic antibodies in idiopathic systemic vascu-
litis. EC/BCR Project for ANCA Assay Standardization. Kidney
Int. 1998;53(3):743–53.
5. Choi HK, Liu S, Merkel PA, Colditz GA, Niles JL. Diagnostic
performance of antineutrophil cytoplasmic antibody tests for
idiopathic vasculitides: metaanalysis with a focus on antimye-
loperoxidase antibodies. J Rheumatol. 2001;28(7):1584–90.
6. Pretorius ES, Stone JH, Hellmann DB, Fishman EK. Wegener’s
granulomatosis: spectrum of CT findings in diagnosis, disease
progression, and response to therapy. Crit Rev Diagn Imaging.
2000;41(4):279–313.
7. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-asso-
ciated vasculitides. Am J Med. 2004;117(1):39–50.
8. Frankel SK, Cosgrove GP, Fischer A, Meehan RT, Brown KK.
Update in the diagnosis and management of pulmonary vasculitis.
Chest. 2006;129(2):452–65.
9. Levin A, Kasem S, Mader R, Naparstek Y, Friedman G, Ben-
Yehuda A. Wegener granulomatosis with back pain, periaortitis,
and dural inflammation developing while receiving monthly
cyclophosphamide. J Clin Rheumatol. 2006;12(6):294–7.
10. Ito K, Minamimoto R, Yamashita H, Yoshida S, Morooka M,
Okasaki M. Evaluation of Wegener’s granulomatosis using 18F-
fluorodeoxyglucose positron emission tomography/computed
tomography. Ann Nucl Med. 2012.
11. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend
WP. The American College of Rheumatology 1990 criteria for
the classification of Wegener’s granulomatosis. Arthritis Rheum.
1990;33(8):1101–7.
Fig. 6 Bilateral adrenal glands showing moderate to markedly increased FDG uptake (SUVmax 13.03) (arrows) during active disease on pre-
treatment PET/CT (first row) which have completely disappeared after treatment on follow-up PET/CT (second row)
914 Ann Nucl Med (2013) 27:907–915
123
12. Bleeker-Rovers CP, Bredie SJ, van der Meer JW, Corstens FH,
Oyen WJ. F-18-fluorodeoxyglucose positron emission tomogra-
phy in diagnosis and follow-up of patients with different types of
vasculitis. Neth J Med. 2003;61(10):323–9.
13. Walter MA, Melzer RA, Schindler C, Muller-Brand J, Tyndall A,
Nitzsche EU. The value of [18F]FDG-PET in the diagnosis of
large-vessel vasculitis and the assessment of activity and extent
of disease. Eur J Nucl Med Mol Imaging. 2005;32(6):674–81.
14. Fuchs M, Briel M, Daikeler T, Walker UA, Rasch H, Berg S. The
impact of 18F-FDG PET on the management of patients with
suspected large vessel vasculitis. Eur J Nucl Med Mol Imaging.
2012;39(2):344–53.
15. Beggs AD, Hain SF. F-18 FDG-positron emission tomographic
scanning and Wegener’s granulomatosis. Clin Nucl Med.
2002;27(10):705–6.
16. Lapraik C, Watts R, Bacon P, Carruthers D, Chakravarty K,
D’Cruz D. BSR and BHPR guidelines for the management of
adults with ANCA associated vasculitis. Rheumatology (Oxford).
2007;46(10):1615–6.
17. Fries JF, Hunder GG, Bloch DA, Michel BA, Arend WP, Cala-
brese LH, et al. The American College of Rheumatology 1990
criteria for the classification of vasculitis. Summary. Arthritis
Rheum. 1990;33(8):1135–6.
18. Maranhao AS, Chen VG, Rossini BA, Testa JR, Penido Nde O.
Mastoiditis and facial paralysis as initial manifestations of We-
gener’s granulomatosis. Braz J Otorhinolaryngol. 2012;78(2):
80–6.
19. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL,
et al. Nomenclature of systemic vasculitides: proposal of an
international consensus conference. Arthritis Rheum. 1994;37(2):
187–92.
20. Blockmans D, Baeyens H, Van Loon R, Lauwers G, Bobbaers H.
Periaortitis and aortic dissection due to Wegener’s granuloma-
tosis. Clin Rheumatol. 2000;19(2):161–4.
Ann Nucl Med (2013) 27:907–915 915
123