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Amyloidosis involving the respiratory system: 5-year's experience of a multi-disciplinary group's activity.

Scala R, Maccari U, Madioni C, Venezia D, La Magra LC - Ann Thorac Med (2015 Jul-Sep)

Bottom Line: Our experience of a multidisciplinary team confirms that intra-thoracic amyloidosis is an uncommon disorder, representing 1.0% of the cases of rare and diffuse lung diseases referred to our center.The diagnosis of the disease is not always easy and quick as the amyloidosis may involve different parts of the respiratory system (airways, pleura, parenchyma).It is therefore recommended to remind this orphan disease in the differential diagnosis of the wide clinical scenarios the pulmonologist may intercept in clinical practice.

View Article: PubMed Central - PubMed

Affiliation: Pulmonology and Respiratory Intensive Care Unit, Arezzo, Italy.

ABSTRACT
Amyloidosis may involve the respiratory system with different clinical-radiological-functional patterns which are not always easy to be recognized. A good level of knowledge of the disease, an active integration of the pulmonologist within a multidisciplinary setting and a high level of clinical suspicion are necessary for an early diagnosis of respiratory amyloidosis. The aim of this retrospective study was to evaluate the number and the patterns of amyloidosis involving the respiratory system. We searched the cases of amyloidosis among patients attending the multidisciplinary rare and diffuse lung disease outpatients' clinic of Pulmonology Unit of the Hospital of Arezzo from 2007 to 2012. Among the 298 patients evaluated during the study period, we identified three cases of amyloidosis with involvement of the respiratory system, associated or not with other extra-thoracic localizations, whose diagnosis was histo-pathologically confirmed after the pulmonologist, the radiologist, and the pathologist evaluation. Our experience of a multidisciplinary team confirms that intra-thoracic amyloidosis is an uncommon disorder, representing 1.0% of the cases of rare and diffuse lung diseases referred to our center. The diagnosis of the disease is not always easy and quick as the amyloidosis may involve different parts of the respiratory system (airways, pleura, parenchyma). It is therefore recommended to remind this orphan disease in the differential diagnosis of the wide clinical scenarios the pulmonologist may intercept in clinical practice.

No MeSH data available.


Related in: MedlinePlus

Fibrobroncoscopy showing reduction of the 25% of the tracheal lumen in its distal tract extended to the right upper lobe bronchus caused by important mucosal hypertrophy (case 1)
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Figure 2: Fibrobroncoscopy showing reduction of the 25% of the tracheal lumen in its distal tract extended to the right upper lobe bronchus caused by important mucosal hypertrophy (case 1)

Mentions: A 71-year-old non-smoker woman presented at our ambulatory with persistent dyspnea despite many months therapy with systemic steroids and inhaled bronchodilator plus steroids for a previous history of bronchial asthma. Lung function tests showed a plateau in the middle of the expiratory part of the flow-volume curve without significant improvement after short-acting beta2 agonist inhalation, suggesting the presence of intra-thoracic irreversible obstruction [Figure 1]. At the chest X-ray, a relevant latero-lateral stenosis of the distal trachea was evident. Chest computed tomography (CT) scan was then performed showing a diffuse thickening of lateral right tracheal wall until the carina with involvement of right upper lobe bronchus and apical segmental bronchus. Fibrobronchoscopy confirmed the presence of tracheal right deviation with a stenosis of about the 25% of the distal tracheal lumen extended to the right upper lobe bronchus caused by marked mucosal hypertrophy [Figure 2]. Bronchial biopsy was positive to the Congo red stain so the diagnosis of primary tracheo-bronchial amyloidosis (isolated AL) was performed. The presence of extra-pulmonary lesions was then excluded through radiological examination (total-body CT scan) and immunological and serological chemistry tests (immunoglobulins and pro-B-type natriuretic peptid -pro-BNP- measurement, Bence Jones protein lab test, routine blood tests). The patient was successfully treated with the placement of a metallic tracheal stent following a Nd-Yag laser-assisted disobstruction of central airways.


Amyloidosis involving the respiratory system: 5-year's experience of a multi-disciplinary group's activity.

Scala R, Maccari U, Madioni C, Venezia D, La Magra LC - Ann Thorac Med (2015 Jul-Sep)

Fibrobroncoscopy showing reduction of the 25% of the tracheal lumen in its distal tract extended to the right upper lobe bronchus caused by important mucosal hypertrophy (case 1)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4518353&req=5

Figure 2: Fibrobroncoscopy showing reduction of the 25% of the tracheal lumen in its distal tract extended to the right upper lobe bronchus caused by important mucosal hypertrophy (case 1)
Mentions: A 71-year-old non-smoker woman presented at our ambulatory with persistent dyspnea despite many months therapy with systemic steroids and inhaled bronchodilator plus steroids for a previous history of bronchial asthma. Lung function tests showed a plateau in the middle of the expiratory part of the flow-volume curve without significant improvement after short-acting beta2 agonist inhalation, suggesting the presence of intra-thoracic irreversible obstruction [Figure 1]. At the chest X-ray, a relevant latero-lateral stenosis of the distal trachea was evident. Chest computed tomography (CT) scan was then performed showing a diffuse thickening of lateral right tracheal wall until the carina with involvement of right upper lobe bronchus and apical segmental bronchus. Fibrobronchoscopy confirmed the presence of tracheal right deviation with a stenosis of about the 25% of the distal tracheal lumen extended to the right upper lobe bronchus caused by marked mucosal hypertrophy [Figure 2]. Bronchial biopsy was positive to the Congo red stain so the diagnosis of primary tracheo-bronchial amyloidosis (isolated AL) was performed. The presence of extra-pulmonary lesions was then excluded through radiological examination (total-body CT scan) and immunological and serological chemistry tests (immunoglobulins and pro-B-type natriuretic peptid -pro-BNP- measurement, Bence Jones protein lab test, routine blood tests). The patient was successfully treated with the placement of a metallic tracheal stent following a Nd-Yag laser-assisted disobstruction of central airways.

Bottom Line: Our experience of a multidisciplinary team confirms that intra-thoracic amyloidosis is an uncommon disorder, representing 1.0% of the cases of rare and diffuse lung diseases referred to our center.The diagnosis of the disease is not always easy and quick as the amyloidosis may involve different parts of the respiratory system (airways, pleura, parenchyma).It is therefore recommended to remind this orphan disease in the differential diagnosis of the wide clinical scenarios the pulmonologist may intercept in clinical practice.

View Article: PubMed Central - PubMed

Affiliation: Pulmonology and Respiratory Intensive Care Unit, Arezzo, Italy.

ABSTRACT
Amyloidosis may involve the respiratory system with different clinical-radiological-functional patterns which are not always easy to be recognized. A good level of knowledge of the disease, an active integration of the pulmonologist within a multidisciplinary setting and a high level of clinical suspicion are necessary for an early diagnosis of respiratory amyloidosis. The aim of this retrospective study was to evaluate the number and the patterns of amyloidosis involving the respiratory system. We searched the cases of amyloidosis among patients attending the multidisciplinary rare and diffuse lung disease outpatients' clinic of Pulmonology Unit of the Hospital of Arezzo from 2007 to 2012. Among the 298 patients evaluated during the study period, we identified three cases of amyloidosis with involvement of the respiratory system, associated or not with other extra-thoracic localizations, whose diagnosis was histo-pathologically confirmed after the pulmonologist, the radiologist, and the pathologist evaluation. Our experience of a multidisciplinary team confirms that intra-thoracic amyloidosis is an uncommon disorder, representing 1.0% of the cases of rare and diffuse lung diseases referred to our center. The diagnosis of the disease is not always easy and quick as the amyloidosis may involve different parts of the respiratory system (airways, pleura, parenchyma). It is therefore recommended to remind this orphan disease in the differential diagnosis of the wide clinical scenarios the pulmonologist may intercept in clinical practice.

No MeSH data available.


Related in: MedlinePlus