Shinshu M ed J, 64⑹ :349 〜355, 2016

Usefulness of F‑18 FDG PET/CT in a Case of Relapsing Polychondritis
Shin YANAGISAWA , Tsuyoshi MATSUSHITA , Masanori YASUO , Ryosuke M ACHIDA
Wataru ISHII and Masumi KADOYA
1) Department of Radiology, Shinshu University School of Medicine
2) First Department of Internal Medicine, Shinshu University School of Medicine
3) Department of Rheumatology, Nagano Red Cross Hospital

We report a case of relapsing polychondritis who underwent F‑18 ﬂuorodeoxyglucose (FDG) positron‑emission tomography/computed tomography (PET/CT), and the F‑18 FDG uptake in the larynx, central
airway and rim cartilages was observed. After corticosteroid and immunosuppressant drug therapy, clinicalﬁndings were improved and F‑18 FDG uptake almost disappeared.F‑18 FDG PET/CT is a useful tool in thediagnosis of relapsing polychondritis and the follow‑up after therapy. Shinshu Med J 64 : 349 ―355, 2016
(Received for publication June 2, 2016;accepted in revised form June 29, 2016)
Key words:relapsing polychondritis, F‑18 ﬂuorodeoxyglucose, positron‑emission
tomography/computed tomographyIntroduction

which F‑18 ﬂuorodeoxyglucose (FDG) positron‑
emission tomography/computed tomography(PET/Relapsing polychondritis is a rare autoimmuneCT)demonstrated systemic cartilaginous inﬂamma-disease, characterized by multisystemic inﬂamma-tion, and report on the therapeutic responses totory lesions of cartilaginous tissues, such as thecorticosteroid administration.

external ear, nose, larynx and tracheobronchial
tree . The ﬁrst diagnostic criteria for relapsing

Case Reportpolychondritis,including auricular,nasal,and respi-

A 47‑year‑old woman presented with a persistent

ratory tract chondritis and so on,were proposed bycough with no response to antibacterial therapy for

McAdam et al. . The clinical symptoms caused bysix months before admission to our hospital. Addi-

auricular chondritis are the most frequent, appear-tionally,she had nasal redness and swelling for two

ing in 80% of patients with relapsing polychon-months before admission, but this symptom haddritis.Laryngotracheobronchial and cardiovasculardisappeared when she was admitted. She com-manifestations are life‑threatening symptoms, forplained of severe cough, shortness of breath andexample, obstruction of the airway and ruptured

stridor. She was a smoker, with a 25‑year history.

aortic aneurysm . These various clinical ﬁndings

Her height was 157 cm and weight was 55.7 kg.

are caused bypolychondritis,so systemic evaluation

Blood pressure was 105/80 mmHg, pulse was 82/

is necessary in the diagnosis of relapsing polychon-min,respiratory rate was 16/min,bodytemperature

dritis and the assessment of its severity.

was 36.1 ℃,and oxygen saturation (SpO )was 96%

We report a case of relapsing polychondritis in(room air). Both auricles were normal. She had aﬁnger tremor and proximal interphalangeal joint

Corresponding author:Shin Yanagisawa
Department of Radiology, Shinshu University School ofM edicine, 3‑1‑1 Asahi, M atsumoto, Nagano 390‑8621,

tenderness. The physical examination revealed
wheezes in the lung. Her physiological and bloodJapan

examinations are shown in Table 1.The erythrocyte

E‑mail: shiny＠shinshu‑u.ac.jp

sedimentation rate(ESR)was 116 mm/hr and the C‑No. 6, 2016349

Yanagisawa.Matsushita.Yasuo et al.
Table 1 Laboratory examinationsTPALB
UA
CRE
AST
ALT
γ‑GT
T‑Bil

7.6 g/dl
3.3 g/dl
18.8 mg/dl
0.87 mg
24 IU/l
12 IU/l
15 IU/l
0.54 mg/dl

ALP
LDH

321 IU/l
186 IU/l
75 IU/l

AM Y

NaK
Cl
cCa
KL‑6IgM
IgG
IgE
ESR
CRP
BNP

139 mmol/l
4.0 mmol/l
103 mmol/l
10.0 mg/dl
250 U/ml
94 mg/dl
2004 mg/dl
265 IU/ml
116 mm/1hr
9.27 mg/dl
5.7 pg/ml

WBC
NEU
LYM
MON
EOS
RBC
Hb
HCT
PLT
sIL‑2R

5800/μl
67%
24.0%
7.1%
0%
3.53×10 /μl
10.6 g/dl

RFANA
ACPAM M P‑3
AJo‑1A
PR3‑ANCA
M PO‑ANCA

31.6%
39.7×10 /μl
489 U/ml

AFPCEASCC
PIVKA‑

negative
negative
negative70.5 ng/ml
negative
negative
negative
3.1 ng/ml
1.2 ng/ml
0.8 ng/ml
15 mAU/ml

TP Total protein ;ALB Albumin ;UA uric acid ;CRE creatinine;AST aspartate aminotransferase;ALT alanine
aminotransferase;γ‑GT gamma guanosine triphosphate;T‑Bil total bilirubin ;ALP alkaline phosphatase;LDHlactate dehydrogenase;AMY amylase;Na serum sodium ;K serum potassium ;Cl serum chloride;cCa corrected
serum calcium ;KL‑6 sialylated carbohydrate antigen KL‑6;IgM Immunoglobulin M ;IgG immunoglobulin G ;IgEimmunoglobulin E ;ESR erythrocyte sedimentation rate;CRP C‑reactive protein ;BNP brain natriuretic peptide;
WBC white blood‑cell;NEU neutrophil;LYM lymphocyte;MON monocyte;EOS eosinophil;RBC red blood cell;
Hb hemoglobin ;HCT hematocrit ;PLT blood platelet ;sIL‑2R soluble interleukin‑2 receptor; RF rheumatoid
factor; ANA antinuclear antibody; ACPA anti‑cyclic citrullinated peptide antibody; M MP‑3 matrix metalloproteinase‑3;AJo‑1A anti Jo‑1 antibody;PR3‑ANCA proteinase‑3 anti‑neutrophil cytoplasmic antibody;MPO‑ANCA myeloperoxidase anti‑neutrophil cytoplasmic antibody;sIL‑2R soluble interleukin‑2 receptor;AFPα‑fetoprotein ;CEA carcinoembryonic antigen ;SCC squamous cell carcinoma antigen ;PIVKA‑ protein inducedby vitamin K antagonist‑
Table 2 Pulmonary function tests
FVC

2.22 l

FEV /FVC%FVC
FEV%FEV

81.0%
1.24 l
53.4%

PEFR
V50
V25

55.9 %
1.99 l/s (34.4%)
0.85 l/s (24.3%)
0.56 l/s (38.9%)

FRC

2.48 l (138.5%)

RVTLC
DLco

1.68 l (119.1%)
4.21 l (106.9%)
17.25 ml/min/mmHg

%DLco

80.0%

FVC forced vital capacity;%FVC % forced vital capacity;FEV forced expiratory volume in 1
second ;% FEV % predictive forced expiratory volume in 1 second ;PEFR peak expiratory ﬂow
rate;V50 expiratory ﬂow rate at 50% lung volume position ;V25 expiratory ﬂow rate at 25%
lung volume position ;FRC functional residual capacity;RV respiratory volume;TLC total lungcapacity;DLco diﬀusing capacity for carbon monoxide;%DLco % predictive diﬀusing capacity
for carbon monoxide

reactive protein (CRP)level was 9.27 mg/dl(normal

After admission she underwent a chest X‑raylevel ＜ 0.1). The white blood‑cell count was 5800/

examination,which revealed stenosis of the trachea

μl. Antinuclear factor was normal and matrix

and bilateral main bronchi. Neck and chest CT

metalloproteinase‑3(MMP‑3)was also normal(70.5

showed calciﬁcations in the bilateral ear canals,

ng/ml). Pulmonary function tests showed a low

abnormal tissue around thyroid cartilage, swelling

FEV /FVC value(55.9 %,Table 2).The ﬂow‑volumeof costal cartilages, and thickening of bronchial

curve (Fig. 1)revealed prominently decreased peakcartilage. We considered the diﬀerential diagnosis,

ﬂow. These results were compatible with obstruc-such as relapsing polychondritis, granulomatoustive pulmonary dysfunction due to central airway

polyangiitis, tracheal tumor, and bronchial asthma,stenosis.

and performed a biopsy on the lesions in the bron-350

Shinshu M ed J Vol. 64

F‑18 FDG PET/CT ﬁndings in relapsing polychondritis

Fig. 1

Fig. 2

The ﬂow‑volume curve revealed
central airway stenosis.

Maximum intensity projection (MIP)image of FDG PET in the supine position showed FDG uptake
in the larynx (long arrow), trachea (short arrow), main bronchi (arrowhead) and rim cartilages (openarrow)(a).Axial PET (b)and fusion images (c)showed high FDG uptake in the larynx (arrows).Axial
PET (d)and fusion images (e)showed moderate FDG uptake in the main bronchi (arrowheads).

chial wall by bronchoscopy, however, the specimen

Then,60 min after injection,attenuation correction

did not show inﬂammation of bronchial cartilage.

CT (AC‑CT) and emission scans were obtained

Next, FDG PET/CT was performed to exclude

from the upper thigh to the head using a PET/CT

malignant lesions such as tracheal neoplasms and toscanner (Biograph mCT‑S(40) 4R, Siemens, Ger-attempt to ﬁnd the extent of other inﬂammatorymany).Maximum intensity projection (M IP)images

lesions in order to determine for the focus of theof FDG PET showed increased FDG accumulation

biopsy site.She fasted for 15 hours before receivingin the larynx, trachea, main bronchi, and some rim

an intravenous injection of 190 M Bq F‑18 FDG.

cartilages (Fig. 2). The maximum standard uptake

No. 6, 2016351

Yanagisawa.Matsushita.Yasuo et al.

value (SUV)of the larynx at 60 min was 12.6.In

addition,we performed bone scintigraphywith technetium‑99m hydroxymethylene diphosphonate toevaluate for cartilaginous inﬂammation, whichshowed no abnormal accumulation (Fig. 3). As aresult, we identiﬁed the inﬂammation in the bronchial cartilage by FDG PET/CT and decided toperform a biopsy again from the bronchial wall bybronchoscopy.We obtained cartilage from the bronchial wall and this time the pathological examination revealed inﬂammatory cell accumulation in thecartilage. We referred to the diagnostic criteria ofrelapsing polychondritis proposed by Damiani etal. .The criteria included clinical ﬁndings (bilateralauricular chondritis, nasal cartilage inﬂammation,respiratory tract chondritis,non‑erosive sero‑negative polyarthritis, ocular inﬂammation, andaudiovestibularinvolvement), histologic

conﬁrmation, and positive response to corticosteroids or dapsone. The diagnosis of relapsing polychondritis was based either on three clinical ﬁndings
or at least one clinical ﬁnding and a positive histological conﬁrmation,or two clinical ﬁndings and apositive response to corticosteroids or dapsone. InFig. 4

Fig. 3

Planar image of bone scintigram using
technetium‑99m hydroxymethylene diphosphonate showed no abnormal accumulationin the larynx, trachea, main bronchi and rim
cartilages.

Maximum intensity projection (M IP)image ofFDG PET in the right lateral decubitus position showeddecreased FDG uptake in the larynx (arrow), trachea, main bronchi and rim cartilages (a). Axial PET(b)and fusion images (c)showed moderate FDG uptake in the larynx (arrows).Axial PET (d)and fusion
images (e)showed disappearance of FDG uptake in the main bronchi.

352

Shinshu M ed J Vol. 64

F‑18 FDG PET/CT ﬁndings in relapsing polychondritis

this case, the patient was compatible with theseevaluation of focal inﬂammation of cartilage andcriteria because she had one clinical ﬁnding (respira-arthritis,particularly in detecting perichondrial andtory tract chondritis)and histologic conﬁrmation.

chondroepiphyseal inﬂammation in the early stages

After diagnosis, she received corticosteroid andof the disease process . However, this method

immunosuppressant therapy. Her persistent coughrequires quite a long examination time, and as our

gradually improved with the therapy. The ESR

patient was unable to maintain the supine position

decreased to 29 mm/hr and the CRP level decreased

for long because of dyspnea, we were unable toto 0.01 mg/dl. Five months after the therapy, theperform the necessary MRI examination in thesecond FDG PET/CT showed that the FDG accu-

present case.

mulations in the cartilaginous tissues had almost

In contrast, radionuclide imaging is used fordisappeared (Fig. 4). Additionally, the CT images

detection of chondritis,not onlyfor focal lesions but

showed that the thicknesses of the airway wall andalso for systemic inﬂammation in relapsing poly-rim cartilages were improved. Accordingly, thechondritis. Bone scintigraphy with technetium‑99m

doses of corticosteroid and immunosuppressant

methylene diphosphonate shows increased accumu-

were gradually tapered.

lation in cartilaginous inﬂammation . Borg et al.

Discussion

suggested that bone scintigraphy showed high accumulations such as extensive calciﬁcations of rim

Relapsing polychondritis is an uncommon dis-cartilages, and was useful for selecting the site oforder that mainly involves systemic cartilaginous

biopsy .However,the accumulation of radioisotope

tissues. In particular, auricular chondritis andtracer depends on bone metabolism and amounts ofpolyarthritis are found in 80% of patients with thiscalciﬁcation. Our case showed no abnormal uptake

disease . Laryngotracheobronchial involvement isat calciﬁcations of the bilateral ear canals because

life‑threatening due to obstruction of the airway,soof the slight amounts present. In addition, boneassessment of the involvement of respiratoryorgans

scintigraphy might not properly depict the sites ofis important for patients . In relapsing polychon-cartilaginous inﬂammation. Gallium scintigraphy isdritis, there are no speciﬁc laboratory data for

useful in determining inﬂammatorylesions in relaps-diagnosis. Laboratory data for inﬂammatory activ-ing polychondritis . Okuyama et al. reported gal-ity, such as CRP and ESR, are frequently used forlium scintigraphy was valuable for evaluating

the evaluation of disease activity, however, eleva-inﬂammatory sites such as subglottic lesions .

tion of these factors may be caused by nonspeciﬁc

They also reported that gallium scintigraphy might

inﬂammatory lesions.

be superior to bone scintigraphy in examining for

High‑resolution CT is useful for evaluation of thelesions unrelated to bone.However,this method has

respiratory tract,such as the thickness of bronchiala lower spatial resolution than FDG PET/CT.

cartilage,airway stenosis,and calciﬁcation of carti-

Therefore,the accumulation at small inﬂammatory

laginous tissues . In addition, Lee et al. suggested

lesions, such as in the larynx and bronchial carti-

that the important ﬁndings in diagnosing relapsinglage, might not be seen with this method.

polychondritis were tracheomalacia and air trap-

FDG PET/CT, which is mainly used in the diag-ping with dynamic expiratory CT . However, thisnosis of malignant lesions,was reported to be useful

method alone does not enable evaluation of in-in the evaluation of inﬂammatory tissues

ﬂammatory activity throughout the body. In theshiyama et al. reported the ﬁrst case of relapsingpatient we needed to perform a systemic evaluation

polychondritis with FDG PET examination .Theyof active inﬂammation in order to determine thesuggested that FDG PET might be a more powerfulbiopsy site, so CT was inadequate for our purpose.

tool than conventional radionuclide imaging such as

M agnetic resonance imaging (MRI)is used for thegallium scintigraphy in the diagnosis of relapsing

No. 6, 2016. Ni-353

Yanagisawa.Matsushita.Yasuo et al.

PET/CT

polychondritis because of its higher spatial resolu-

FDG

clearly showed systemic in-tion. In addition, some reports assessed the useful-ﬂammatory lesions, such as in the larynx, bronchi

ness of FDG PET/CT in the diagnosis of relapsing

and rim cartilages in the present case, which werepolychondritis and monitoring the therapeutic

not detected by bone scintigraphy. In addition, thisresponse. Unfortunately, the health insurance

method helped to determine the site of biopsyin thissystem in Japan does not yet support the use of FDGcase. The change in the accumulation of FDG

PET/CT in the diagnosis of inﬂammatory diseases.

furthermore helped in the evaluation of the thera-

In addition, evidence from FDG PET/CT is not

peutic eﬀectiveness of corticosteroid administra-necessarily required in the diagnostic criteria oftion.

relapsing polychondritis.However,FDG PET/CT is

In conclusion, FDG PET/CT was a usefulwell known to be useful in the diagnosis of in-radiological tool for the diagnosis of relapsing poly-

ﬂammatory lesions

chondritis and the evaluation of the therapeutic. In particular, it is a power-

ful tool for detecting active inﬂammatory lesions

response in the present case.

and for determining the site of biopsy for diagnosis.
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