Inflammatory bowel disease (IBD) is a chronic and re- latively common disorder of uncertain etiology (1). IBD can be associated with a variety of respiratory disor- ders (2). A link between pulmonary disease and IBD was suggested nearly 40 years ago (3). Both screening studies and the cumulative volume of case reports sug- gest that respiratory system may be involved in IBD more frequently than it is generally appreciated (4).
The colonic and respiratory epithelia both share embr- yonic origin from the primitive foregut (5). Although, many of the reported pulmonary diseases associated with IBD have cryptic etiologies, the causes or mecha- nisms of respiratory tract involvement in IBD remain poorly understood (2).
A 51 years old female patient was admitted to our cli- nic with the complaints of cough, fever and sputum production for 10 days and abdominal pain, bloody stool and diarrhea for two days. The patient was a life- long non-smoker and had no history of occupational or environmental exposure relevant to lung disease. Des- pite empirical antibiotic treatment, there was no clini- cal improvement.
Chest X-ray revealed infiltration at the right lower zone.
Pulmonary function test was normal. Connective tissue markers including ANA, anti-dsDNA, RF were negati-
ve. Erytrocyte sedimentation rate (ESR) and C-reacti- ve protein (CRP) were high. Contrast-enhanced com- puted tomography revealed segmental atelectasis on the right lower lobe and infiltration around atelectasis (Figure 1). A bronchoscopy was performed; there was endobronchial polypoid lesion on the posterior wall of the posterior segment of right lower lobe. Bronchosco- pic biopsy revealed subepithelial fibrosis and under the
Tüberküloz ve Toraks Dergisi 2011; 59(3): 312-315
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Yazışma Adresi (Address for Correspondence):
Dr. Evrim Eylem AKPINAR, Ufuk Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Konya Yolu No: 88/86 Balgat, Ankara - TURKEY
e-mail: [email protected]
Editöre mektup/Letter to the editor
Thorax as an extraintestinal target for inflammatory bowel disease
Evrim Eylem AKPINAR1, Meral GÜLHAN1, Halil DEĞERTEKİN2, Ömür ATAOĞLU3
1 Ufuk Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Ankara,
2 Ufuk Üniversitesi Tıp Fakültesi, Gastroenteroloji Bilim Dalı, Ankara,
3 Mikro-pat, Patoloji Laboratuvarı, Ankara.
Figure 1. Thorax CT before mesalazine treatment.
bronchial epithelium, a mixed type inflammation is composed of mononuclear cells admixed with poly- morphs and eosinophils were observed (Figure 2). Ex- tensive search for bacterial (including mycobacteri- um), fungal and parasitic infectious agents were all ne- gative.
A colonoscopy was also performed. Because there we- re ulcerations and pseudopolips on transverse colon mucosa, multiple biopsies were taken. Focal inflam- mation was seen on pathological examination of colo- nic biopsies (Figure 3). When colonoscopy and patho- logic examination considered together, patient was di- agnosed as Crohn’s disease. Endobronchial lesion of the lung was evaluated as Crohn’s disease after other causes of endobronchial lesions including neoplasms and infectious causes were excluded. Oral mesalazine (500 mg for three times a day) was started. Both res- piratory symptoms and abdominal symptoms of the
patient were totally improved at the first month of the mesalazine treatment. On laboratory examination ESR and CRP levels were in normal limits. Complete radi- ological and bronchoscopic resolution were also reve- aled in follow up examination.
Respiratory system may be involved in very different patterns in Crohn’s disease (6). However, to our know- ledge, this is the first case of Crohn disease’s pulmo- nary involvement as endobronchial polypoid lesion.
Overt and clinically significant lung involvement of Crohn’s disease is rare. However, lung function test ab- normalities were seen more often especially during at- tack periods of the disease (6-8). The decrease in car- bon monoxide diffusion capacity (DLCO) is the pulmo- nary function test abnormality that is most often seen in IBD, especially in active phase of the disease, both in pediatric and adult patients (6,9,10). Small airway dysfunction, hyperinflation with increased functional residual capacity and residual volume bronchial hyper- reactivity was also detected among patients with IBD (6,8,10,11). Pulmonary function test and DLCO of our patient were normal, possibly due to localized segmen- tal involvement.
Clinically significant lung involvement of Crohn’s dise- ase is rare, but quite variable. Respiratory system may be affected from upper airway to alveoli. The large air- ways are the most common location of IBD involve- ment (2,12,13). Stridor may be a presenting symptom due to subglottic inflammation and stenosis. Tracheob- ronchitis with or without upper airway obstruction and laryngitis in different studies as pulmonary involve- ment of Crohn’s disease have been shown (2,12-14).
Severe tracheobronchial stenosis in a case was also re- ported by Kuzniar et al. (15). All these patients with up- per airway disease responded dramatically to oral or inhaled steroid treatment.
Bronchiectasis is mostly known pulmonary manifesta- tion of IBD involving large airways. Acute or chronic bronchitis, suppurative airway disease may also be se- en as large airway disease in IBD patients. Surprisingly, a high proportion of these patients are non-smokers (6,16).
Unlike other forms of lung involvement, small airway involvement in IBD commonly starts before gastroin- testinal symptoms as in our case. Pathologically, altho- ugh bronchiolitis and peribronchial granuloma formati- on are fairly common, endobronchial polypoid lesion at segmental level has not been reported before our case.
Interstitial lung involvement has been reported to ac- company both clinical IBD entities, Ulcerative colitis Akpınar EE, Gülhan M, Değertekin H, Ataoğlu Ö.
313
Tüberküloz ve Toraks Dergisi 2011; 59(3): 312-315 Figure 3. Granulation tissue and fibrosis compatible withulcer ground, HE x200.
Figure 2. Under the bronchial epithelium, a mixed type inf- lammation is composed of mononuclear cells admixed with polymorphs and eosinophils are observed, HE x200.
and Crohn’s disease (17). In addition to bronchial eosi- nophilic infiltration, pulmonary infiltrates with eosinop- hilia, nonspesific interstitial pneumonia, desquamative interstitial pneumonitis also were reported as pulmo- nary involvement of IBD (2,18). Treatment with corti- costeroids or with appropriate medication such as sul- fasalazine or mesalamine for the basic gastrointestinal disease appeared to be satisfactory for both diseases.
Pneumonitis, in contrast, due either to sulphasalazine or mesalamine is a well-recognized adverse drug reac- tion in these patients (19).
Infrequently, Crohn’s disease may present as sterile multiple necrobiotic nodules even though the disease was in remission with mesalazine treatment (20).
Pleura may also be affected in IBD, but rarely. Pleural involvement is almost always unilateral and pleural fluid is usually exudative in nature (21). Pleural fibro- sis has also been reported in a patient with Crohn’s disease (22). Colobronchial fistula is another rare complication of Crohn’s colitis. Different forms of pulmonary involvement in IBD were presented in Table 1 (7).
Thorax as an extraintestinal target for inflammatory bowel disease
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Table 1. Different forms of lung involvement in inflammatory bowel disease.
Mean age ± Extraintestinal
Site of involvement SD years Female gender (%) manifestations (%)
Upper airway 40.5 ± 14.8 40 33.3
Trachea Larynx/glottis
Large airways 42.6 ± 7.4 64.2 52
Bronchiectasis Chronic bronchitis Suppurative airway disease Acute bronchitis
Small airways 28.9 ± 14.4 47 18.1
Bronchiolitis
Bronchiolitis obliterans Diffuse panbronchiolitis
Parenchyma 38.8 ± 21.2 57.5 32.5
BOOP Nodules
Intertitial lung disease not otherwise specified Pulmonary interstitial emphysema
Desquamative interstitial pneumonia Fibrosing alveolitis
Eosinophilic pneumonitis Sarcoidosis
Alfa-1 antitrypsin deficiency
Pulmonary vasculature 29.3 ± 13.9 50 30
Wegener granolomatosis Churg-Strauss syndrome Microscopic polyangitis
Pulmonary vasculitis not otherwise specified
Serosa 29 ± 14 37 31.8
Pleural disease Pericardial disease
Akpınar EE, Gülhan M, Değertekin H, Ataoğlu Ö.
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Tüberküloz ve Toraks Dergisi 2011; 59(3): 312-315 In conclusion; Crohn’s disease may involve respiratorysystem in variable forms, even as endobronchial lesion.
In differential diagnosis of almost all pulmonary dise- ases including benign endobronchial polypoid lesions, the patient’s history about gastrointestinal symptoms should be evaluated carefully Crohn’s disease should be considered.
ACKNOWLEDGEMENT
The authors would like to express their appreciations to Dear Gulten Ortac for proofreading.
CONFLICT of INTEREST None declared.
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