Unusual Combination of Tracheobronchopathia Osteochondroplastica and AA Amyloidosis

Department of Chest Diseases, Faculty of Medicine, University of Dicle, Diyarbakir, Turkey.
Yonsei medical journal (Impact Factor: 1.29). 10/2009; 50(5):721-4. DOI: 10.3349/ymj.2009.50.5.721
Source: PubMed


Tracheobronchopathia osteochondroplastica (TO) is a rare disorder of unknown cause characterized by the presence of multiple submucosal osseous and/or cartilaginous nodules that protrude into the lumen of the trachea and large bronchi. A simultaneous diagnosis of TO and amyloidosis is rarely reported. In this report, a case initially suspected to be asthma bronchiole that could not be treated, was radiologically diagnosed as TO, and also secondary amyloidosis is presented. A 53 years, man patient reported a 3 years history of dyspnea. Pulmonary function tests (PFTs) showed an obstructive pattern. Chest X-rays revealed right middle lobe atelectasis. FOB and CT detected nodular lesions in the trachea and in the anterior and lateral walls of the main bronchi. AA amyloidosis was confirmed by endobronchial biopsy. In the abdominal fat pad biopsy, amyloidosis was not detected. Asthma bronchiole was excluded by PFTs. This case illustrates that it is possible for TO and amyloidosis to masquerade as asthma. TO and amyloidosis should be suspected in patients of older ages with asthma and especially with poorly treated asthmatic patients. Although nodular lesions in the anterior and lateral tracheobronchial walls are typical for TO, a biopsy should be obtained to exclude amyloidosis.

Full-text preview

Available from: PubMed Central
  • Source
    • "Biopsy of TO lesions sometimes can be a challenge due to the hard consistency of the bone nodules [26]. It was reported that histopathologic examination of TO nodules were usually unnecessary [40]. Nevertheless, because of unfamiliarity for many bronchoscopists with this condition and the need to exclude differential diagnoses, the histopathologic confirmation should be essential and reasonable in most cases [37]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Background and Study Aims Tracheobronchopathia osteochondroplastica (TO) is an uncommon disease of the tracheobronchial system that leads to narrowing of the airway lumen from cartilaginous and/or osseous submucosal nodules. The aim of this study is to perform a detailed review of this rare disease in a large cohort of patients with TO proven by fiberoptic bronchoscopy from China. Patients and Methods Retrospective chart review was performed on 41,600 patients who underwent bronchoscopy in the Department of Respiratory Medicine of Changhai Hospital between January 2005 and December 2012. Cases of TO were identified based on characteristic features during bronchoscopic examination. Results 22 cases of bronchoscopic TO were identified. Among whom one-half were male and the mean age was 47.45±10.91 years old. The most frequent symptoms at presentation were chronic cough (n = 14) and increased sputum production (n = 10). Radiographic abnormalities were observed in 3/18 patients and findings on computed tomography consistent with TO such as beaded intraluminal calcifications and/or increased luminal thickenings were observed in 18/22 patients. Patients were classified into the following categories based on the severity of bronchoscopic findings: Stage I (n = 2), Stage II (n = 6) and Stage III (n = 14). The result that bronchoscopic improvement was observed in 2 patients administered with inhaled corticosteroids suggested that resolution of this disease is possible. Conclusions TO is a benign disease with slow progression, which could be roughly divided into 3 stages on the basis of the characteristic endoscopic features and histopathologic findings. Chronic inflammation was thought to be more important than the other existing plausible hypotheses in the course of TO. Inhaled corticosteroids might have some impact on patients at Stage I/II.
    PLoS ONE 07/2014; 9(7):e102068. DOI:10.1371/journal.pone.0102068 · 3.23 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Tracheobronchopathia osteochondroplastica (TO) is a rare large-airway disorder of unknown cause, characterized by the development of multiple submucosal osseous and/or cartilaginous nodules that protrude into the lumen of the trachea and large bronchi. Although most patients are asymptomatic, some may present with nonspecific respiratory symptoms. A chest computed tomographic scan with multiple protruding nodules in the trachea is suggestive but other diagnoses can present with a similar appearance; hence, definitive diagnosis may require bronchoscopic examination. Numerous cases of TO have been reported in association with different diseases, but no definitive causative explanation has been shown with any of the conditions. Chronic airway inflammation is one of the proposed mechanisms to explain the formation of TO nodules. Most of the reported TO patients have a history of frequent airway infections. Patients with IgA deficiency present with frequent sinopulmonary infections. The present case illustrates the first case of TO associated with IgA deficiency.
    Journal of Bronchology and Interventional Pulmonology 12/2012; 19(1):54-6. DOI:10.1097/LBR.0b013e3182446949
  • [Show abstract] [Hide abstract]
    ABSTRACT: Background: Endobronchial obstructions are rarely seen in children and are often misdiagnosed resulting in delay of definitive treatment. A variety of diseases can cause endobronchial obstructions in childhood, but data is limited as to the frequency, distribution and clinical characteristics of endobronchial obstructions diagnosed with flexible bronchoscopy (FB). Objective: To document endobronchial obstructions detected by FB. Methods: FB results from three pediatric pulmonology centers in Istanbul were evaluated. Results: A total of 2,555 children underwent an FB procedure during the study period. Endobronchial obstructions were detected in 10% (n = 256) of the patients. Among FB in patients who had endobronchial obstructions, the four most common indications for bronchoscopy were persistent infiltrations (30%, n = 72), persistent wheezing (28%, n = 70), chronic cough (26%, n = 66) and atelectasis (23%, n = 59). The most common endobronchial obstructions detected in the patients were aspirated foreign bodies (35.9%, n = 92), endobronchial tuberculosis (31.6%, n = 81), mucous plugs occluding airway (16.7%, n = 43) and granulation scars (6%, n = 16). Other pathologies included hydatid cysts (n = 5), hemangiomas (n = 5), tumors (n = 5), submucosal nodules (n = 5) and polyps (n = 4). Endobronchial obstructions were most commonly located in the right bronchus (51%, n = 130) followed by the left bronchus (33%, n = 85), bilaterally (8%, n = 21) and trachea (8%, n = 20). Conclusions: Endobronchial obstructions can be caused by a number of different diseases which require various medical or surgical treatments. In the presence of clinical or radiological findings suggesting an endobronchial obstruction, FB should be performed promptly. Copyright (C) 2012 S. Karger AG, Basel
    Respiration 01/2013; 85(1):43-48. DOI:10.1159/000342339 · 2.59 Impact Factor
Show more