Asthma-Like Tracheo-Bronchial Amyloidosis

doi:10.4236/ojrd.2012.22008

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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

Email: *[email protected]

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.

F. C. B. ABDALLAH ET AL.

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.

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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