Open Journal of Respiratory Diseases, 2012, 2, 57-59
http://dx.doi.org/10.4236/ojrd.2012.22008 Published Online May 2012 (http://www.SciRP.org/journal/ojrd)
Asthma-Like Tracheo-Br onchial Amyloid osis
Fatma Chermiti Ben Abdallah1*, Hanene Smadhi1, Ridha Mahouachi1, Amel Chtourou1,
Sofia Taktak1, Ayda Ayadi2, Faouzi El Mezni2, Ali Ben Kheder1
1Pneumology IV Department, Abderrahmen Mami Hospital, Ariana, Tunisia
2Anatomy-Pathology Department, Abderrahmen Mami Hos pital, Ariana, Tunisia
Received October 20, 2011; revised November 25, 2011; accepted December 10, 2011
ABSTRACT
Localized bronchial Amyloidosis is an uncommon disease of unknown origin. Clinical signs are not specific. Tracheo-
bronchial symptoms are the most frequent. We report a case of a 31 year-old man complaining of asthma-like dyspnea.
Bronchoscopy was performed because of ineffectiveness of antiasthmatic treatment, showed a submucosal infiltration
with stenosis of both right and left upper bronchi and a complete stenosis of intermediate troncus. Multiple biopsies
were performed and concluded to Amyloidosis of AL type. Oral corticosteroids were indicated with clinical improve-
ment.
Keywords: Amyloidosis; Biopsy; Histo logy; Corticosteroids
1. Introduction
Tracheobronchial amyloidosis (TBA) is rare and it con-
stitutes approximately 1% of benign tumors in these ar-
eas. It is characterized by deposition of amyloid within
the airway walls, manifesting as submucosal tumors.
Symptoms are related to bronchial obstruction leading to
a misdiagnosis in some cases. Consequently, the diagno-
sis is delayed and made through bronchoscopic biopsy.
No consensus exists with regard to its op timal treatment,
resulting in different modalities, used to manage this
condition.
We report a case of localized bronchial amyloidosis
diagnosed after a history of asthma-like dyspnea.
2. Case Report
A 31-year-old man, non -smoking complained of asthma-
like wheezing dyspnea during six months, with a recent
worsening during one month. He was treated with in-
haled corticosteroids and bronchodilators, but without
improvement. He was admitted to our department. His
clinical examination showed a respiratory rate of 28/min,
a pulse rate of 90/min and blood pressure of 110/80
mmHg. Pulmonary examination revealed diffuses sibi-
lant rales. All routine biological examinations were nor-
mal. The arterial blood gas levels in room air were as
follows: PaCO2 of 38.1, PaO2 of 62.3, and pH of 7.47.
Pulmonary function tests showed an obstructive pattern:
FVC of 2.33 l (50% of predicted), FEV1 of 1.33 l (34%
of predicted) and FEV1/FVC ratio of 57%.
Chest X-ray revealed atelectasis in the middle lobe and
a right hilar opacity (Figure 1). Bronchodilators, corti-
costeroids and antibiotics were administrated for two
weeks but without improvement. A Compued tomogram-
phy (CT) scan of the chest was performed, showing air-
way narrowing caused by circumferential thickening of
the bronchial wall and atelectasis in the left upper lobe
(Figure 2).
Fiberoptic bronchoscopy was then performed reveal-
ing severe narrowing of the right and the left main bron-
chi with mucosal irregularities and a complete sten osis of
intermediary bronchus and the left upper bronchus (Fig-
ure 3). Pathological study of bronchial biopsy demon-
strated an extensive deposit in the submucosa of a hya-
line substance shown to be amyloid. The epithelium over
the deposit was intact. No squamous metaplasia was
demonstrated. Some giant foreign-body cells were pre-
sent in relation to the amyloid and small calcifications
were seen. Histopathological analysis of the tissue sam-
ple, with Congo red staining, demonstrated apple-green
birefringence when viewed under polarized light (Figure
4).
The diagnosis of bronchial amyloidosis was made and
several investigations were undertaken. The results of
urine analysis (for 24-h proteinuria and creatinuria) and
protein profiles were within normal limits. An electro-
cardiogram, echocardiography and abdominal ultrasound
showed no abnormalities.
The diagnosis of TBA was then made and the patient
*Corresponding author.
C
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F. C. B. ABDALLAH ET AL. 58
Figure 1. Chest X-ray showing deviation of the trachea and
atelectasis in the middle lobe.
Figure 2. Chest CT scan showing airway narrowing and
wall thickening with stenosis of the intermediary and left
upper bronchi.
received oral corticosteroids (0.5 mg/Kg/day) with pro-
gressively decreased doses over 7 months. We noted
clinical improvement of dyspnea but persistent obstruc-
tive pattern in pulmonary function tests.
3. Discussions
Amyloidosis is a rare condition; fewer than 150 cases
were reported in the literature. It’s characterized by ab-
normal extracellular deposition of amyloid an d autologus
fibrillar protein material, which binds with Congo red
revealing green birefringence under polarized light [1].
All organ systems can be infiltrated by amyloid.
Figure 3. Broncoscopy showing patchy areas of erythema
and mucosal irregularities causing stenosis of intermediary
bronchus and left upper br onchus.
Figure 4. Bronchial biopsy tissue showing eosinophilic
masses of amyloid material; Congo, red staining demon-
strating apple-green birefringence of the tissue studied un-
der polarized microscopy.
Lesser described in 1877 three forms of respiratory
amyloidosis: focal or diffuse TBA, nodular parenchymal
and diffuse parenchymal amyloidosis [2]. TBA is still th e
most common manifestation of primary pulmonary amy-
loidosis which occurs exclusively in the absence of sys-
temic infiltration [3].
It affects males more than females. The median onset
age is 50 - 60 years old with ranging from age 27 to 85
years [4,5]. Common symptoms include dyspnea, non-
productive cough, haemoptysis and hoarseness [6]. The
disease has insidious course and its clinical presentation
is not specific; thus TBA is often misdiagnosed. The pa-
tient presented in this report was treated by short acting
bronchodilators and inhaled corticosteroids during 6
months because of asthma-like presentation. Consequently
diagnosis was delayed.
Chest X-ray is normal in half of. Imaging especially
CT-scan is helpful for diagnosis. Radiographic findings
are various including bronchial wall thickening, irregular
narrowing of the airway lumen, calcified nodules within
the airway lumen, airway obstruction with atelectasis,
postobstructive pneumonia and distal lung air trapping [5,
7]. Our patient had post obstructive pneumonia partially
improved by antibiotics. In these cases other diagnoses
have to be discussed such as neoplastic diseases, tuber-
culosis, other granulomatous disease and tracheobron-
chopathia osteochondroplastica. Diagnosis confirmation
of TBA requires fiberoptic bronchoscopy which allows
better visualization of lesions and biopsies. Positive
specimen with Congo red staining with a characteristic

F. C. B. ABDALLAH ET AL. 59
apple green birefringence with polarized light is the
proof of amyl oidosis di sease [ 4, 5].
The treatment of localized TBA is essentially symp-
tomatic [8]. Strategies depend on the site and extent of
the disease. In fact, only observation can be indicated in
asymptomatic patients. However, local and or systemic
therapies are recommended in patients with severe air-
way obstruction [9]. Drugs inhibiting the synthesis,
deposition and degradation of amyloid in tissues, espe-
cially colchicine, prednisone and melphalan were used
but with a limited effect [4,9]. Varied methods of bron-
choscopic treatment have been reported such as endo-
bronchial Nd-YAG laser, tracheobronchial stents, bal-
loon dilation and bronchoscopic ablation of intraluminal
amyloid deposits. These methods are associated with a
risk of bleeding and recurrences [4]. Surgery may be
necessary when airway involvement is extensive [5]. Re-
cently, some investigators demonstrated that external
beam radiation therapy (EBRT) may provide an objective
improvement [10]. Also M. Truong found in his study
that EBRT prevents progressive amyloid deposition in
90% of patients with localized airway amyloidosis and it
is well tolerated with minimal toxicity [11].
Although TBA is a localized process, it is associated
with poorer prognosis. Authors reported that overall sur-
vival ranges from 31% to 43% at 4 to 6 years. In absence
of curative treatment of TBA, researchers are still con-
tinuously trying o ther therapeutic alternatives to improve
the prognosis of these patients.
4. Conclusion
Diagnosis of TBA has to be considered in patients with
asthma-like dyspnea in whom anti-asthmatic treatments
are ineffective. The diagnosis can be suggested by radio-
graphic findings but it is confirmed essentially through
bronchoscopic biopsies, with appropriate Congo red
staining of the bronchial tissue samples obtained. At
present, there are no therapeutic options proved to be
completely successful. However, bronchoscopic man-
agement often proves to be temporarily effective, while
EBRT still need to be further evaluated. Prognosis of
TBA remains poor.
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