Jou rn al of the Korean Radiological Society, 1994; 30 (6) : 1039 - 1044

Bronchiectasis in Diffuse Panbronchiolitis: High Resolution CT Assessment1

Kun 11 Kim, M.D., Ki Nam lee2, M.D., Jae Ryang Juhn3, M .D. , Woo Hyun Ahn4, M.D., Soon Kew Park5, M.D., Byung Soo Kim, M .D .

Purpose: To evaluate the characteristics of the bronchiectasis in diffuse panbronchiolitis using HRCT. Materials and Methods: We retrospectively studied 12 H RCT scans and two bronchography of 12 patients with diffuse panbronchiolitis(DPB). According t 。 Akira et al., DPB was classified into four types: small nodules around the end of bronchovascular branchings(CT type 1), small nodules in the centrilobular area connected with small branching linear opacities(CT type 11). nodules ac­ companied by ring -shaped or small ductal opacities connected to proximal bronchovascular bundles(CT type 111). large cystic opacities accompanied by dilated proximal bronchi( CT type IV). We compared the type and the extent of bronchiectasis, CTtypes of DPB, and pulmonary function test. Results: Bronchiectasis was defined in 12 cases with the tubular type predominantly involving small and medium-sized bronchi. These bronchiectasis involved the proximal bronchi of the centrilobular lesions of DPB. Among eight cases of advanced DPB(CT type 111 & IV) which extended to both upper lobes, seven showed tu bu lar bronchiectasis at the same area. Cystic bronchiectasis was shown in eight cases predominantly involving right middle lobe(n=7) . There was no linear correlation between the values of p 비 mona ry function test and CT types of DPB. Conclusion: Characteristic feature of the bronchiectasis in DPB is the tubular ectasis predominantly involving the small and medium -sized bronchi.DPB with associated tubular bronchiectasis can involve whole lung field in advanced cases. HRCT is useful not only to depictthe findings of DPB but also to demonstrate the extent of lesion.

Index Words: Bronchiectasis Bronchiolitis Lung ,CT Computed tomog raphy( CTl , h i 9 h-resol uti on

Diffuse panbronchiolitis(DPB) is a chronic inflamma­ airflow limitation ; and histologically by typical bron­ tory disease ofthe respiratory bronchioles of unknown chiolar lesions(1). Chest radiograph and high resol­ etiology characterized clinically by chronic cough, ex­ ution CT have shown specific findings of the bron­ pectoration , and dyspnea; physiologically by chronic chiolar lesions(2, 3). However, recent studies have shown that DPB cau­ ‘Department of Diag nostic Radiology. Coll ege of Medicine. Pusan National Uni ses inflammation and dilatation of not only the bron­ versi ty chioles but also the bronchi(4-7). Although there is no 2Depa rtemen tof Diag nos itic Radio logy, Coll ege of Med icin e, Do ng-AUni ve rsity doubt that the major pathologic findings are present in 3Depa rtment ofD iagnostic Radio logy , Col lege of Medicin e, Inje University and around the bronchi 이 ar walls, it is not known ' De partment ofRadiology, Wallace Memorial Hospital 5Department of Internal Medicine , Coll ege 이 Medicine, Pusan National Unive r­ whether the repiratory bronchioles are primarily or sity secondarily affected(8). Furthermore, the relationship Rece ived december13 , 1993 ; Accepted April7 , 1994 of DPB to bronchiectasis, if there is any, remains to be Add ress reprin t requests to:K un- II Kim, M.D., Department of Rad iology , Col lege of Medicine , Pusan National Uni versity í 1-10, Am i-dong , Seo-gu, Pusan, elucidated(7) . 602-739 Korea , Tel (051) 240 - 7373 Fax. (051) 244 -7534 We began this study to find the characteristics of 1039 - Journ al of the Korean Radiologica l Society, 1994 : 30 ( 6) : 1039-1044 the bronchiectasis in DPB and to determine if there is in diffuse panbronchi 이 itis with assessment of the any suggestion about the nature of the airway disease findings of HRCT.

Table 1. Summ ary 0112 Pati ents with Diffuse Panbronchiolitis Bronchiectasis Case PFT CT Extent Sizeol Age/Sex ABGA type* 。 IDPB site type bronchi Others

U * + RML tubular La * - FEVl :39 % 1.57 / F 3 / 2 * M * ++ Ls * tubular Me * + + 17 years * FVC :34 % L *+ + + BLL tubu lar Sm * + + + U++ La + FEVl : 27 % Whole * tubular Bronchography 2. 33 1 M 4 13 M + ++ Me ++ FVC :40% RML cystlc 13 years L+++ Sm ++ + FEVl : 58 % U- Ls tubular La - Bronchography 3. 21 / M FVC : 62 % 2 / 1 M+ BLL tubular Me ++ RML lobectomy Pa02 :65% L ++ RML cystic Sm ++ 10 years U + + La - Pseudomonas Pa0 2 :38 % Whole tubular 4. 48 1 F 4 / 4 M + + Me ++ Inlection PFTNA * RML cystic L + ++ Sm +++ 20 years U + La - FEVl : 61 % Whole tubular 5. 24 1 F 3 / 2 M + Me + 8 years FVC: 65 % RML cystic L ++ Sm + ++ U- RML tubular La - FEVl ’ 58 i 6. 42 / M ’ 2 13 M + LLL tubular Me ++ 20years FVC ‘ 64 % L +++ LLL cystic Sm ++ U + Whole tubul ar La - FEVl : 55 % 7.32 / F 3 12 M ++ RML cystic Me+ + 20 years FVC : 60 % L +++ RML varlcose Sm ++ U- La - FEVl ‘ 49 % 2 / 3 Pathologic DPB 8. 26 1 M M- RLL tubular Me + FVC ’ 54 % Rt ) Lt * 15 years L +++ Sm + U ++ La - FEVl : 25 % Whole tubular 9. 20 1 M 4 14 M + ++ Me + 8 years FVC :31 % RML cystic L+++ Sm ++ + U- La - FEVl : 52 % 2/3 10. 20 1 F M+ LLL tubular Me + 7 years FVC: 47 % Lt ) Rt * L ++ Sm + FEVl : 42 % U + + La - 11 . 51 1 F FVC : 51 % 4 / 4 M ++ Whole tubular Me + 3 years Pa02 :63% L ++ + Sm + U + + Whole tubular La - FEVl : 66 % 12. 26 1 F 3 / 4 M ++ + RML cystic Me + 9 years FVC : 62 % L +++ RUL cystic Sm + Abb reviations * U, M, L : Upper, Middle, Lower lung lield * La, Me, Sm ,: Large, Medium , Small-sized bronchi * Ls : Lingular segment BLL : both lower lobes * Whole: Whole lobes * Years Duration 01 sym ptoms such as productive coughing and exertional dyspnea * NA : Not Applicable due to severe dyspnea * Rt ) Lt, Lt ) Rt : Right or Left predominant inv 이 vement 01 lung * +, + + , + + +: mild, moderate, severe involvement * CT type 1: small nodules around the end 01 bronchovascular branchings CTtype 11 : small nodules in the centrilobular area connected with small branching linear opacities CT type 111 : nodules accompanied by ring-shaped or small ductal opacities connected to proximal bronchovascular bundles CT type IV : large cystic opacities accompanied by dilated proximal bronchi * CT type 3/2: Predominant CT type/Subsidiary CT type - 1040 - Kun 11 Kim , et al : Bronchiectasi s in Diffuse Panbronchi 이 itis

dium -sized bronchi. These bronchiectasis involved MATERIALS and METHODS the proximal bronchi of the centrilobular lesions of DPB . In two cases of unilateral predominant involve­ The study group consisted of 12 patients(tive men ment of DPB , ipsilateral involvement of tubular and seven women) , who ranged in age from 20 to 57 bronchiectasis and thickening of bronchial wall were years(mean 33 years). The diagnosis was based on the found(one in the left lower lobe, the other in the right cl inical , functional , and radiologic criteria of Homma et lower lobe). Among the eight cases(CT type 111 and IV) al (1) and HRCT(Table 1). AII cases had chronic pan­ involving both upper lung fields with DPB , seven cases sinusitis. Pseudomonas aeruginosa was detected con­ showed tubular bronchiectasis. Tubular bronchiecta­ tinuously in the sputum of one case. Histologic proof sis was not found in the lobar or segmental area which was obtained by means of open lung biopsy in one was not involved with bronchiolar lesion case. The CT scans were obtained on a Somatom Plus In addition , there were cystic bronchiectases in eight S(Siemens) and GE 9800(General Electric) with 1.0 to cases predominantly involving right middle 10be(RML) 1.5 mm collimation at 1.5-2.0 cm interval from the (n =7) (Fig. 2). Other sitesof cystic bronchiectasis were apex to the base ofthe lung during breath holding after left lower 10be(LLL) in one case and right upper lobe full inspiration. A high spatial frequency algorithm was (RU L) anterior segment in another( combined RML used for all patients. The high - resolution images were lesion). The cases with cystic bronchiectasis had CT displayed at window levels appropriate for pulmonary type 11 in two, CT type 111 in three, and CT type IV in three parenchyma (1000-15001 -700 - -600). In two cases, cases. Large bronchi were involved with tubular we reviewed the bronchography films taken one year bronchiectasis only in one case, but all cases(n=12) before the diagnosis of DPB. Two diagnostic radio­ showed thicken ing of the bronchial wall. log ists were participated in reading of the HRCT films. In two cases, previous bronchography showed mild The CT findings of DPB were graded with CT types tubular bronchiectasis at the medium - sized bronchi , reported by Akira et aI( 2) . Several CT types seen bronchiolectasis, stenosis of long segment of small concurrently in each case were categorized using airways, tapered obstruction ofthe small and medium slash(Predominant CT type/Subsidiary CT type, e. g. sized bronchi , and absence of the acinar filling in the CT type 111/11). The extent of the involvement of DPB was involved area. (Fig. 2, 4) divided to upper, middle and lower third lung field. We There was no linear correlation between CT types observed the laterality of the lesions. Bronchiectasis and FEV1 %(p= -0.526, p=0.09) , % FVC(p= -0.477, and the thickening of the bronchial wall were assessed p=0.13) , and FEV1 IFVC(p= -0.481 , p=0.13) though according to the size ; large(main stem , lobar, and seg­ there was a tendency that the values of pulmonary mental bronchi) , medium, and small(within the range function test decreased as CT type increased. With of the diameter of accompanying pulmonary artery higher CT types, DPB and associated bronchiectasis from 1 to 2 mm) bronchi by our arbitrary classification involved the upper lung field(P=0.01). The extent(involved lobes or segments), the type(cys­ tic, varicose and tubular), and the laterality of involve­ DISCUSSION ment of bronchiectasis were also assessed. We correlated the findings of DPB and associated bronchi­ Clinical DPB based on the diagnostic criteria accord- ectasis with p 비 monary function test and CT types of DPB. Wilcoxon rank sum test and Mantel - Haenzel chi ­ square test, and Spearman correlation coefficient were used in analysis of data.

RESULTS

Of the 12 cases with DPB , four cases had CT type 11 (CT type 1111 in one, 111111 in three), four had CTtype III(CT type 111 111 in three, III /IV in one) , and four had CTtype IV (CT type IV/III in one, IV/IV in three). None was in the CT type 1. The extent of DPB was predominant in lower lung zone, butthere were upper lung zone extension in eight cases all of which had advanced disease(CT type 111 and IV). Bilateral symmetrical involvement was found in 10 cases(Fig. 1,2) , and there were two cases of Fig . 1. Case 4, Bilateral symmetrical involvement 01 both upper unilateral predominant involvement(Fig, 3) . lung lield with bronchiol itis and dilatation 01 small bronchi and/or Bronchiectasis was defined in 12 cases with the bronchiolectasis(large arrows). Some secondary p 비 monary tubular type predominantly involving small and me- lobules are spared Irom air-trapping(small arrows) - 1041 - Journal of the Korean Radiological Society, 1994: 30 (6) : 1039 - 1044 ing to Homma et al. (1) can include the case with not on DPB showed that typical HRCT findings are enough only pathologic DPB but also unclassified bronchiolitis to diagnose DPB when correlated with clinical fea­ and bronchiolectasis(7). But several recent studies ture(2, 3). Open lung biopsy is not neccessary for the diagnosis of DPB when typical HRCT features, clinical findings and p 비 mon ary function test(1) reaults exist. The HRCT findings of DPB include centrilobularly distributed, small rounded areas of attenuation ; branched linear areas of attenuation, contiguous with the small rounded areas; dilated airways with thick walls, also common outside secondary p 비 monary lobules ; and decreased lung attenuation in peripheral areas due to air trapping caused by the bronchiolar ob­ struction(8). Bronchial wali thickening was thought to be due to inflammation of bronchial wall or retained se­ cretion(8). Figure 3 shows one case of crescentic wall thickening on dependent portion which can be due to retained secretion Cystic bronchiectasis was found in 8 cases which af- a

j Fig. 3. Case 10, Predominant left lung involvement 01 DPB with b ipsilateral mild tubular bronchiectasis in medium-sized airways with thickening of bronchial wal l. The thickened portion of bron ch i probably suggests the retained secretion because th e thickening is only seen in the dependent portion(arrow) Centrilobular lesions are seen in peripheral lung field 01 RML and LLL

c Fig. 2. Case 2, a. Cystic bronchiectasis in RML. Th ere is also di­ latation ofmedi um-sized bronchi with thi ckened wall(arrows) b. Bron chograp hy one year before. There are tubular bronchi­ ectasis in righ t lower lobe an d abrupt narrowi ng of small airway sug gesting long segmentstenosis(arrow). Fig. 4. Case 3, Bronchography shows tubular branching linear c. Left lower lob e shows poor lilling 01 acini inspi te 01 adjacent shadows about 5 mm apart from pleural surface compatible with normal acinarfilling. bronchiolectasis

- 1042 Kun 11 Kim, et al: Bronchiectasis in Diffuse Panbronchiolitis

fected predominantly the right middle lobe known to be Akira et al(2) seem to be too simplified to be in accord­ the vulnerable site due to its anatomical orientation(9). ance with the clinical severity of DPB as there were Considering the specific features of DPB which is bilat­ several CT types in most patients in our study. Prob­ eral , symmetrical and diffuse, we consider that cystic ably not only the CT types but also the extent of involve­ bronchiectasis in DPB is more likely to be due to recur­ ment of DPB should be considered in the correlation rent secondary infection. We propose that the pres­ with P 비 monary function test. We suggest that the CT ence of cystic brçmchiectasis should not influence the types should be cautiously applicated in clinical prac­ diagnosis of DPB in the reading ofthe HRCT films tice, and larger number of subject should be studied to Tubular bronchiectasis involving the small and me­ determine clinical usefulness of the application of the dium - sized bronchi is considered as the characteristic CTtype. feature of the bronchiectasis in DPB . With the view In conclusion, the characteristic bronchiectasis in point of the bronchiectasis, DPB can be considered as DPB occurs specifically in the small and medium - sized a cause of the diffuse bronchiectasis (such as cystic fi­ bronchi , mainly with the tubular type. At first, the dis­ brosis and immotile cilia syndrome etc.), although the ease begins in basallung fields. These bronchiectasis relationship of DPB and bronchiectasis was not and corresponding bronchiolitis can involve whole elucidated. In our cases, there was no early DPB(CT lung field as the disease process advances. HRCT is type 1) which had only centrilobular small nodules with­ useful to depict the centrilobular lesions and out bronchiolectasis or bronchiectasis. However, it is associated proximal bronchiallesions in DPB , and also suggested that the bronchiectasis would eventually de­ useful to demonstrate the extent of DPB. To clarify the velop in vicinity of the bronchiolar lesions and prog­ relation between the centrilobular lesions and dilated ress proximally if the patient is neglected as shown in bronchi in DPB , prospective long-term studies and our cases. studies for the etiology are needed. It is possible but not proved that the dilatation of the proximal bronchi is secondary due to bronchiolar ob­ REFERENCES struction in DPB as supposed by Akira et al. We have

shown two cases of DPB examined with bronchogra­ 1. Homma H, Yamanaka A, Tanimoto S, et al. Diffuse panbron­

phy which was done 1 year before under the suspicion chiolitis ‘ a disease of the transitional zone of the lung. Chest of bronchiectasis. These cases show somewhat un­ 1983 ; 83 : 62-69 usual bronchographic features for usual bronchiecta­ 2. Akira M, Kitatani F, Lee YS , etal. Diffuse panbronchiolitis: evalu­ sis such as irregular stenosis and dilatation of the ation with high-resolution CT1. Radiology 1988 ‘ 168 : 433-438 small airways, areas of the bronchiolectasis, and diffi­ 3. Choo SW, 1m JG , Kim DY, et al. Diffuse panbronchiolitis: chest culty of acinar filling at the involved area despite radiograph and HRCT findings in 8 patients. Journal o( Korean forceful inspiration. These findings were reported pre­ Radiology Society.1992; 28(4): 553-557 4. Maeda M, Saiki S, Yamanaka A. Serial section analysis of the viously from Japan but considered as nonspecific for lesions in diffuse panbronchiolitis. Acta Pathol Jpn 1987 ; 37 DPB(10, 11). With long term follow- up of the broncho­ 693-704 graphies in DPB , they found that the bronchiectasis in 5. Kitaichi M. Pathology o( diffuse panbronchiolitis (rom the view medium - sized bronchi is the findings of advanced point o( differential diagnosis. In: Grassi C, Rozzatp , Pozzi E, cases in DPB(1 이 which could have been the theoretical eds. Sarcoidosis and other granulomatous disorders. Ams­ basis insisting that the proximal bronchiectasis is sec­ terdam : Elseier , 1988 ; 741-746 ondary to bronchiolar obstruction. But two factors are 6. Kitaichi M, Nishimura K, Izumi T. Di(use panbronchip/itis. In required for the occurence ofthe bronchiectasis : bron­ Sharma OP , ed. Lung diseases in the tropics. New Yor~ ‘ Pekker, chial wall demage and a dilating force(12). It is not 1991 ; 479-509 clear whether the proximal bronchiolar or bronchial di­ 7. Izumi T. Diffuse panbronchi 이 itis. Chest1991 ; 100: q9p-597 latation can be caused by just physiologic dilating force 8. Nishimura K, Kitaichi M, Izumi T, Itoh H. Diffuse panbrpflchiolitis correlation of high-resolution CT and pathologic findings. Radi­ produced by the distal bronchiolar narrowing or ob­ ology 1992 ; 184 : 779-785 structi on(8). 9. Scott AR , Carl ER , Carles EP , et al. Peripheral Middle Lobe Syn­ In our study, there was no linear correla drome. Radiology 1983; 149: 17-21 10. Nakata K, Tanimoto H. Diffuse panbronchiolitis. Jpn JClin R~diol 1981 ; 26 : 1133-1142 11. Homma H. Diffuse panbronchi 이 itis. Jap J Thorac Dis 1975; 13 383-395 12. Jack L. Westcott. Bronchiectasis . Radiol Clin North Am 1991 ; 29 (4): 1009(4) :1031-1042

- 1043 - Journal of the Korean Radiological Society, 1994: 30(6) : 1039-1044

대 한 방사 선 의 학회 지 1994 : 30( 6): 1039 - 1044

미만성 세기관지염에서의 기관지확장증:고해상 CT 소견

김건일 .01 기남 1. 전제량 2 • 안우현 3 • 박순규 4 • 김병수

부산대학교 의과대학 방사선과학교실 동아대학교 의과대학 진 단방사선과학교실 2 인제대학교 의과대학 진 단방사선과학교실 3 침례병원 방사선과 4 부산대학교 의과대학 내과학교실

목 적 : 미만성세기관지염 (Diffuse panbronchiolitis, DPB) 에서 잘 동반되는 기관지확장증의 특징을 알고자 하였다. 대상 및 방법 : DPB로 진 단된 환자 12 엽의 고해상 전산화 단층촬영 (HRCT ) 12 예와 기관지조영술 2 예을 후향적으로 분석하 여 기관지확장증의 형태와 분포앙상을 DPB 으| 진행정도와 침범부위 및 페 기 능검사치 ( FEV1 ， FVC ) 와 비교하였다. DPB의 진행

정도는 Akira 등의 분류에 따라 HRCT상 type 1 은 기관지혈관분지부의 끝에 소결절이 있는 것， type 11 는 중심소엽성 소결질이

소분지음영과 연결된 것， type 川는 걸절이 근위부 기관지혈관대에 연결된 환형 혹은 관상형 음영과 동반될 [[H , type IV 는 큰

난형음영이 확장된 큰위부기관지와 동반될 때로 분류하였다.

결 과 : 12 예 모두에서 범발성의 소형 혹은 중형 기관지를 침범하는 관상형 기관지확장증이 보였다. 이들은 모두 세기관지

병소의 근위부 기관지에 나타났고 세기관지병소가 침범한 엽과 소엽부위에서 보였다. 병변이 진행된 8여 I(CTtype 111 and IV )

중 7 예에서는 양측상엽에서도 같은 특징의 기관지 확장증이 보였다.8 예에서는 낭형 기관지확장증이 보였며 이늠 우중엽에

7 여|로 많았다. CT typeOI 진행할수록 폐기능검사치가 감소하는 경향은 보였으나 유의한 상관관계는 보이지 않았다.

결 론 :DPB 으| 특징적인 기관지 확장증은 주로 중소형 기관지의 관상형 기관지확장이다. 이 질환은 세기관지 뿐만아니라

근위부 중소형 기관지의 염증과 확장을 동반하며 폐기저부에서 병소가 시작하지만 치료없이 진행하면 전 폐야를 침범한다.

HRCT는 이 질환의 진단 및 진행정도 뿐만아니라 침범부위를 아는데 유용하다.

- 1044-