Pulmonary alterations in Behcet's disease

European Journal of Radiology 70 (2009) 317–319

Pulmonary alterations in Behcet’s disease Cetin Celenk a,∗ , Fatma Aydin b , Meftun Unsal c a

Department of Radiology, Faculty of Medicine, Ondokuz Mayis University, Tip Fak¨ultesi, Radyoloji, 55139 Samsun, Turkey b Department of Dermatology, Faculty of Medicine, Ondokuz Mayis University, 55139 Samsun, Turkey c Department of Chest Diseases, Faculty of Medicine, Ondokuz Mayis University, 55139 Samsun, Turkey

Received 28 September 2007; received in revised form 11 January 2008; accepted 28 January 2008

Abstract Purpose: This study aims to demonstrate pulmonary alterations (PA) in patients with Behcet’s disease by using CT. Materials and methods: CTs of 50 patients with Behcet’s disease and 20 others in a control group have been evaluated retrospectively for PA (septal, reticular, nodular, atelectatic opacities). Results: Eight out of 50 patients (16%) with Behcet’s disease showed PA. Three out of 20 (15%) in the control group showed PA. No differences were observed between Behcet’s disease patients and the control group regarding pulmonary alterations (p = 0.917). No differences were observed in the disease duration, ages and sex in either group in those with and without PA. Conclusion: Pulmonary alterations can be seen in patients with Behcet’s disease, but these alterations are not significant. © 2008 Elsevier Ireland Ltd. All rights reserved. Keywords: Behcet; Computerized tomography; Pulmonary alterations

1. Introduction Because patients with Behcet’s disease have no pathognomonic symptoms or findings that would show up in laboratory tests, diagnosis is made according to the criteria set by the International Study Group (ISG) in 1990 [1]. Behcet’s disease is a chronic, relapsing, multisystemic, and inflammatory disease. It causes mucocutaneous, ocular, vascular, articular, gastrointestinal, urogenital, pulmonary, and neurological abnormalities [2,3]. Etiopathogenesis is still a subject of study. Infectious agents, immune mechanisms, and genetic factors are held responsible. However, Behcet’s disease is described as a type of systemic vasculitis. Actual prevalence of PA is unknown in Behcet’s disease because there are no prospective studies where pulmonary symptoms are evaluated in randomly selected patient groups. Although there are frequent pulmonary abnormalities in those with vasculitis, pulmonary parenchymal abnormalities are not ∗

Corresponding author. Tel.: +90 362 4576000/2656; fax: +90 362 4576041. E-mail addresses: [email protected], [email protected] (C. Celenk), [email protected] (F. Aydin), [email protected] (M. Unsal). 0720-048X/$ – see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrad.2008.01.049

frequent in Behcet’s disease. The reported prevalence is between 5 and 10% [4,5]. Some authors have reported the pulmonary parenchymal alterations percentage in Behcet’s disease as 12–38–59% [6–8]. Recently, CT is a preferred monitoring modality as a noninvasive method in the evaluation of all pulmonary parenchyma and pleura [9,10]. The purpose of this study is to evaluate PA and to compare the results with disease duration, age, and sex, using CT. 2. Materials and methods 2.1. Patient population Fifty patients diagnosed with Behcet’s disease, according to the ISG [1] criterion, were included in this study. Patients were chosen from a group of persons having no granulomatous or mycobacterial lung disease findings and those who requested a routine chest CT in a dermatology clinic. Ages, sexes, disease duration and clinical findings of the patients were recorded. The period since the date of the first diagnosis was set as the disease duration.

318

C. Celenk et al. / European Journal of Radiology 70 (2009) 317–319

Table 1 Clinical findings of patients with Behcet’s disease

Table 3 Findings of control group

Clinical findings

Number of cases

Oral ulceration Genital ulceration Eye lesions Arthralgia Skin lesions Pathergy test positive

50 48 15 25 31 16

Patient no. Female/male Age Pulmonary alterations 3 No pulmonary alterations 17

2/1 10/7

49 (40–60) 35 (20–65)

Smoking ±1/2 ±4/13

A control group was made up of persons referred because of hilar enlargement that showed in postero-anterior chest radiography, but CT slices revealed negative hilar pathology. 2.2. CT A multislice Toshiba-Aquillon (TSX-101A-16 slice) scanner at 120 kV and 180 mA S was used to examine the patients. Scanning was performed from the apex of the lung to the costophrenic angles. The matrix size was 512 × 512 pixels. All scans were obtained during suspended respiration at the end of the inspiratory volume. Patients were scanned in a supine position with 100 cm3 IV contrast medium. All images were examined and photographed at a window width of 1000, 1200 HU and a window level of −600, −700 HU. Pulmonary interstitium of patients were evaluated for septal, reticular, nodular, atelectatic opacities, etc. 3. Results Clinical findings of patients are summarized in Table 1; CT findings, sex, age, smoking, and duration of disease in Table 2; and control group findings in Table 3. Eight out of 50 patients with Behcet’s disease showed PA (Fig. 1). Three out of 20 in the control group showed PA. No differences were observed between Behcet’s disease patients and the control group regarding pulmonary alterations (p = 0.917). Twenty-two (44%) of the patients were male, 28 (56%) were female, with an average age of 38 (14–60); mean age of 37.92 ± 9.64 years. Sixteen (32%) of these patients were smokers; mean consumption was 4.26 ± 7.58 packs per year, median 13 (0–29) packs per year. No significant differences were observed in the CT findings of smoking and non-smoking groups. Again, it has been found that findings in groups with or without CT abnormalities were irrelevant with alterations connected to smoking. The mean age of patients with PA was 43.70 ± 9.99 years, with a median age of 43 (29–60) years, and the mean age of

Fig. 1. Chest CT of a 50-year-old man shows irregulaire, reticulo-nodulaire opacities, parenchymal bands and interlobulaire septal prominence at right middle zone.

patients without PA was 36.48 ± 9.12 years with a median age of 36.5 (14–52) years. Mean and median disease duration were, respectively, 9.82 ± 9.15 and 5.50 (1–40) years in the entire study group population. The average disease duration was 10.07 ± 9.7 (1, 25) years in the group with PA and 8.8 ± 6.8 (1, 40) years in the group with no PA. 4. Discussion In our study the aim was to evaluate the PA particularly, not the mediastinal and main vascular structures. The pulmonary manifestations of Behcet’s disease include pulmonary infarcts, pulmonary hemorrhage, atelectasis, fibrosis, and emphysema. Volume loss, wedge shaped or linear shadows, ill-defined nodular or reticular opacities, cryptogenic organizing pneumonia, eosinophilic pneumonia, recurrent pneumonia, and bronchitis, have been described in Behcet’s disease patients with or without pulmonary artery aneurysms [4–9,11–18]. The PA are non-specific and appear as focal and diffuse areas of increased opacity [18]. The most common parenchymal lesions are subpleural alveolar infiltrates and wedge-shaped or illdefined rounded areas of increased opacity, which represent

Table 2 Findings of patients with Behcet’s disease

Pulmonary alterations No pulmonary alterations

Patient no.

Female/male

Median age

Smoking

Duration of disease

8 42

3/5 25/17

43 (29–60) 37 (14–52)

±3/5 ±13/30

10.07 ± 9.7 (1, 25) 8.8 ± 6.8 (1, 40)

C. Celenk et al. / European Journal of Radiology 70 (2009) 317–319

focal vasculitis with hemorrhage, infarction, and inflammation [17]. Reported prevalence of PA are between 5 and 10% [4,5]. However, some other research results reported 12, 38, and 59%. Caglar et al. found that 17 of 29 patients (59%) with Behcet’s disease showed pulmonary involvement by direct radiography, CT, and an intravenous digital subtraction angiography study [7]. Several pathological findings such as pleuroparenchymal fibrotic alterations, ground glass appearance, and thickenings of pleural, peribronchial, bronchial, and interlobular septa were found in 11 of 29 (38%) patients by Uysal et al. [8]. Gunen et al. found that 5 of 42 patients (12%) had pulmonary involvement [6]. In our study, 8 out of 50 patients (16%) with Behcet’s disease showed PA. Three out of 20 (15%) in the control group showed PA. Some researchers claim that PA seen in groups of Behcet’s disease patients result because the groups is made up of a particular kind of patient. Namely, if a patient group is made up of those who have respiratory symptoms, we can expect PA in the radiographies of those patients. In conclusion, PA that are seen in Behcet’s disease are nonspecific changes and could be due to various reasons such as inflammation, pneumoconiosis, pulmonary infarct, etc. References [1] International Study Group for Behcet’s Disease. Criteria for diagnosis of Behcet’s disease. Lancet 1990;335:1078–80. ¨ [2] Behcet H. Uber rezidivierende, apht¨ose, durch ein Virus verursachte Geschw¨ure am Mund, am Auge und an den Genitalien. Dermatologische Wochenschr 1937;105:1152–7. [3] Gamble CN, Wiesner KB, Shapiro RF, Boyer WJ. The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behcet’s disease. Am J Med 1979;66(6):1031–9.

319

[4] Dahnert W.Radiology review manual. 4th ed. Maryland: Williams and Wilkins; 1999. p. 384. [5] Erkan F. Pulmonary involvement in Behcet’s disease. Curr Opin Pulm Med 1999;5:314–8. [6] Gunen H, Evereklioglu C, Kosar FErH, Kizkin O. Thoracic involvement in Behcet’s disease and its correlation with multiple parameters. Lung 2000;178(3):161–70. [7] Caglar M, Ergun E, Emri S. 99Tcm-MAA lung scintigraphy in patients with Behcet’s disease: its value and correlation with clinical course and other diagnostic modalities. Nucl Med Commun 2000;21(2): 171–9. [8] Uysal H, Balevi SE, Okudan NH, Gokbel H. The relationship between HRCT and pulmonary function in Behcet’s disease. Lung 2004;182(1):9–14. [9] Hiller N, Lieberman S, Chajek-Shaul T, Bar-Ziv J, Shaham D. Thoracic manifestations of Behcet disease at CT. Radiographics 2004;24(3): 801–8. [10] Murata K, Khan A, Herman PG. Pulmonary parenchymal disease: evaluation with high-resolution CT. Radiology 1989;170(3 Pt 1):629–35. [11] Efthimiou J, Johnstan C, Spiro SG, Turner-Warvick M. Pulmonary disease in Behcet’s syndrome. Q J Med 1986;58(227):259–80. [12] G¨ul A, Yilmazbayhan D, B¨uy¨ukyabani N, et al. Organizing pneumonia associated with pulmonary artery aneurysms in Behc¸et’s disease. Rheumatology 1999;38:1285–9. [13] Tunaci A, Berkmen YM, Gokmen E. Thoracic involvement in Behcet’s disease: pathologic, clinical, and imaging features. AJR 1995;164(1): 51–6. [14] Grenier P, Bletry O, Cornud F, Godeau P, Nahum H. Pulmonary involvement in Behcet disease. AJR 1981;137(3):565–9. [15] Ahn JM, Im JG, Ryoo JW, et al. Thoracic manifestations of Behc¸et syndrome: radiographic and CT findings in nine patients. Radiology 1995;194:199–203. [16] Tunaci A, Berkmen YM, G¨okmen E. Thoracic involvement in Behc¸et’s disease: pathological, clinical and imaging features. AJR 1995;164(1):51–6. [17] Raz I, Okon E, Chajek-Shaul T. Pulmonary manifestations in Behcet’s syndrome. Chest 1989;95(3):585–9. [18] Winer-Muram HT, Gavant ML. Pulmonary CT findings in Behcet disease. J Comput Assist Tomogr 1993;13:346–7.