*From the Department of Radiology, Duke University Medical Center, Durham, NC.
Correspondence to: H. Page McAdams, MD, Duke University Medical Center, Department of Radiology, Box 3808, Durham, NC 27710
A 27-year-old woman presented with a history of
chronic cough and wheezing, recurrent lower respiratory tract
infections, and progressive shortness of breath. Asthma had been
diagnosed 1 year previously in this patient, and her therapy included
an inhaled β-adrenergic agonist and glucocorticoid.
On admission, her temperature was 38.5°C, pulse rate was 86
beats/min, respiratory rate was 17 breaths/min, and BP was 126/70 mm
Hg. Auscultation of her lungs revealed diffuse rhonchi, and palpation
revealed no abdominal organomegaly. Findings from the examination of
the CNS and skin was normal. Leukocytosis was revealed in a WBC count
of 24.5 × 109 cells/L with 85%
neutrophils. The platelet count and hemoglobin levels
were normal. Arterial blood gas indexes included a
Po2 of 68 mm Hg,
Pco2 of 47 mm Hg,
HCO3− of 31 mEq/L, and
pH of 7.43.
Pulmonary function tests were indicative of obstructive airways
disease: an FVC of 1.88 L/min (50% of predicted), an
FEV1 of 0.84 L (28% of predicted), and a
residual volume of 2.08 L (142% of predicted). The diffusing capacity
of the lung for carbon monoxide was reduced to 54% of the predicted
The chest radiograph at admission showed heterogeneous pulmonary
opacities in the right lower lobe consistent with pneumonia. Both lungs
had peribronchial wall thickening of the segmental bronchi, and the
left lung had perihilar subsegmental atelectasis (Fig 1
). A chest CT scan revealed diffuse narrowing and soft-tissue thickening
of the walls of the trachea (not shown), the main bronchi (arrows), and
the segmental bronchi (arrowheads) (Fig 2
Bronchoscopy revealed diffuse nodular thickening of the walls of the
airways, a narrowing of the left upper lobe bronchus, and mucosal
friability. Purulent secretions were present in the right lower lobe. A
transbronchial biopsy specimen was diagnostic.
differential diagnosis of diffuse narrowing and tracheobronchial wall
thickening includes relapsing polychondritis, sarcoidosis, Wegener’s
granulomatosis, tracheopathia osteoplastica, and tracheobronchitis
associated with ulcerative colitis. In tracheopathia osteoplastica,
multiple nodules (often calcified) are visualized protruding from the
anterior and lateral walls of the central airways. The posterior walls
do not contain cartilage; therefore, nodules and calcification are not
present.1 Relapsing polychondritis, a recurrent
inflammation of cartilage, involves the laryngeal and tracheal
cartilages in > 50% of patients.1 A chest CT scan can
show marked diffuse tracheobronchial narrowing that is exacerbated by
The transbronchial biopsy tissue specimens were stained with Congo red
dye and exhibited green birefringence under polarized microscopy, a
finding characteristic of amyloid. The total protein count was 6.1
g/dL, with a mild hypogammaglobulinemia of 0.4 g/dL. Serum
immunoelectrophoresis revealed a small IgM monoclonal spike.
Bone marrow aspiration and biopsy specimens demonstrated a normal
cellularity (without plasmacytosis) and a normal histology. The liver
and myocardial biopsy specimens were normal, consistent with the
diagnosis of primary tracheobronchial amyloidosis.2
Relief of symptoms was achieved with antibiotic, oral glucocorticoid,
and nebulized β-adrenergic agonist therapies. The proposed treatment
includes a trial of interferon therapy and an evaluation for future
Amyloidosis refers to a group of diseases characterized by the
extracellular deposition of the complex fibrillar protein amyloid in
one or more organs of the body.2 Amyloidosis is generally
classified either according to the anatomic site of involvement
(localized or systemic) or to coexisting medical diseases, such as
primary or secondary amyloidosis.2–6 This latter
group includes amyloidosis associated with aging, myeloma, Hodgkin’s
disease, chronic infections, or inflammatory disorders.
Pulmonary amyloidosis usually occurs in patients with primary systemic
amyloidosis.7–10 These patients seldom have pulmonary
symptoms, although radiographic abnormalities (reticular and
reticulonodular opacities) are common.3,10–11
Primary pulmonary amyloidosis is uncommon and manifests as focal or
diffuse tracheobronchial or parenchymal
amyloidosis.4,9–10,12 Tracheobronchial disease is usually
multifocal and manifests as submucosal plaques.4,9 In 14%
of patients, the amyloid deposition is solitary and can mimic an
endobronchial neoplasm. Patients with tracheobronchial
amyloidosis are usually in their fifth or sixth decade of life (range,
27 to 85 years old).9–10,12 Common presenting symptoms
include wheeze, chronic cough, hemoptysis, dyspnea, and recurrent
pneumonia.9–10,12 Radiologic manifestations include focal
and diffuse thickening and narrowing of the major airways, chronic
atelectasis, and bronchiectasis.
Parenchymal amyloidosis manifests either as single or multiple
nodules or as diffuse interstitial pulmonary
disease.9–10,12 The nodules range in size from 0.3 to 15
cm (mean, 3 cm), and they can calcify.9,13 Interstitial
pulmonary disease usually manifests as diffuse, small, irregular
reticular and reticulonodular parenchymal opacities.9
Patients are usually > 50 years of age; patients with parenchymal
nodules are typically asymptomatic, and patients with interstitial
disease often present with dyspnea.9–10,12–13
In summary, primary pulmonary amyloidosis is an uncommon disease that
can involve the tracheobronchial tree and the pulmonary parenchyma.
Patients with tracheobronchial and diffuse interstitial amyloidosis are
frequently symptomatic; patients with parenchymal nodules are usually
asymptomatic. CT scans typically show parenchymal nodules or diffuse
tracheobronchial narrowing and wall thickening. The diagnosis is
confirmed by tissue specimens that exhibit green birefringence under
polarized microscopy after staining with Congo red dye.
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