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Chest Imaging

Amyloidosis, thoracic

condition related to the deposition of amyloid material in different organs. Organ dysfunction occurs if sufficient amyloid material accumulates. Amyloidosis may be a systemic disease when deposition is generalized, or be localized to a single organ. Local deposition to an organ may itself be diffuse or focal. Localized disease never becomes generalized. Amyloid is a waxy pink material made of abnormal proteins that stains with Congo red, and has a typical birefringence with polarization microscopy. Amyloidosis may be primary or secondary. Lung involvement in primary amyloidosis occurs rather commonly. Secondary amyloidosis is usually associated with rheumatoid arthritis, suppurative diseases and malignant neoplasm, particularly multiple myeloma. In the thorax, three forms of amyloidosis have been described: tracheobronchial, nodular and diffuse alveolar septal. For further general description, see amyloidosis.

Tracheobronchial amyloidosis

Affected patients are often symptomatic with cough, dyspnoea, haemoptysis and stridor. Recurrent pulmonary infections are common. Amyloid submucosal deposition is more frequently diffuse than focal. When diffuse, it can involve the trachea and proximal (main, lobar and segmental) bronchi together or in part. Radiologically, involvement is seen as multiple concentric or eccentric strictures and mural nodulation. Calcific deposits are frequently present (Fig.1). When amyloid deposition is focal an endobronchial or endotracheal mass may occlude partially or completely airways, with infiltration of the adjacent peritracheal or bronchial tissues, often with foci of calcifications. The appearance may mimic those of both benign and malignant bronchial or tracheal neoplasms. In both diffuse and focal types, lobar atelectasis and recurrent infective consolidation are frequent.

Nodular pulmonary amyloidosis (amyloidoma)

This usually occurs late in life, in the seventh decade. Patients are usually asymptomatic and the prognosis is excellent. The radiographic appearance consists of solitary or multiple nodules and masses. Calcification is common. Cavitation is rare. Nodules grow very slowly.

Diffuse alveolar septal amyloidosis

Infiltration of amyloid material is diffusely distributed in all compartments of the pulmonary interstitium, associated with giant cells and plasma cells. Patients are symptomatic, with progressive dyspnoea leading to respiratory insufficiency. Radiologically, lung infiltration is seen as a diffuse miliary disease, or a nodular or reticulonodular pattern.

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Fig.1

High resolution CT scans (a, b, c) in a patient with tracheobronchial amyloidosis revealed by recurrent bronchial infection, dyspnoea and haemoptysis. There is a diffuse bronchial wall thickening leading to an occlusion of segmental bronchi in the right lower lobe and segmental atelectasis. Calcific deposits are seen within the thickened bronchial wall. Small centrilobular nodules visible within the right middle and lower lobes reflect inflammatory bronchiolitis.
Amyloidosis, thoracic, Fig.1 (a)
Amyloidosis, thoracic, Fig.1 (b)
Amyloidosis, thoracic, Fig.1 (c)