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: ozmenozlem@yahoo.com

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