Caso clinico

 Femmina di 65 anni, non fumatrice.

 Lamenta da circa 6 anni abbondante

produzione giornaliera di
espettorato, a volte, di colore
giallastro.
 Che esami diagnostici faresti?

 Che cosa ti aspetti di trovare?

SINDROME BRONCHIECTASICA

• Definizione
• Epidemiologia
• Eziologia
• Anatomia patologica
• Manifestazioni cliniche
• Diagnosi differenziale
• Trattamento
• Prevenzione
 DEFINIZIONE DI BRONCHIECTASIA

Dilatazione permanente dei bronchi che in condizioni

fisiologiche hanno un diametro interno superiore a 2 mm
NB: a volte sono presenti
manifestazioni cliniche extrapolmonari,
che in alcuni casi possono essere prevalenti
Per convezione questi pazienti quando hanno anche una
sindrome disventilatoria ostruttiva non
vengono considerati BPCO
SINDROMI BRONCHIECTASICHE

• Definizione
• Epidemiologia
• Eziologia
• Anatomia patologica
• Manifestazioni cliniche
• Diagnosi differenziale
• Trattamento
• Prevenzione
EPIDEMIOLOGIA DELLE SINDROMI
BRONCHIECTASICHE IN ITALIA

Mancano dati epidemiologici precisi

Sono abbastanza frequenti

(circa 200 casi osservati dalla Pneumologia
Universitaria nel periodo 2003-Giugno 2009)
SINDROMI BRONCHIECTASICHE

• Definizione
• Epidemiologia
• Eziologia
• Anatomia patologica
• Manifestazioni cliniche
• Diagnosi differenziale
• Trattamento
• Prevenzione
 CAUSES OF BRONCHIECTASIS

• Bronchiectasis is idiopathic in more than 50% of adults

despite aggressive investigation.

• Infection-related bronchial damage is the most common

secondary cause.

• Abnormal host defense and systemic diseases are other

common secondary cases .

• Other secondary causes .

INFECTION-RELATED BRONCHIAL DAMAGE
• Post-infectious 30-60% (childhood pneumonia, measles,
whooping cough).

• Mycobacterium tuberculosis complex infection.

• Mycobacterium avium complex (Lady Windermere

syndrome).

• Allergic bronchopulmonary mycosis (ABPM, mainly

ABPA) 10%

• Swyer-James-McLeod syndrome (Brett's syndrome in

France)
An 81-year-old woman was admitted with weight loss (18 kg in 27 months), hemoptysis, and tubular
and diffuse granular shadows on her chest radiograph (Panel A)

Final diagnosis:
MAC infection
of bronchiectasis

Ebihara T and Sasaki H. N Engl J Med 2002;346:1372

A 26-year-old man who smoked and had a long history of poorly controlled asthma and severe
environmental allergies was admitted for an exacerbation of asthma

Final diagnosis: ABPA

Chen T and Hollingsworth H. N Engl J Med 2008;359:e7

Swyer-James syndrome is an uncommon is a manifestation of postinfectious
obliterative bronchiolitis (after Mycoplasma pneumoniae infection, Streptococcus
pneumoniae infection, and severe RSV infection) in which lung hypoplasia occurs as
a result of diminished vascularity and the arrest of progressive growth and
alveolarization of the lung. Multifocal areas of air trapping may be seen. The
pulmonary parenchymal pattern is similar to that of obliterative bronchiolitis.
The typical radiological appearance of Swyer-James syndrome is that of a
hyperlucent but small lung, with overexpansion of the contralateral lung.
A comparison of progressive radiographs shows failure of growth in the involved
lung. A diffuse pattern of scarring or irregular vessels may be present. Fluoroscopy
shows little change in volume in the involved lung with respiration
 ABNORMAL HOST DEFENSES

• Cystic fibrosis 3%
• Primary ciliary diskinesia (immotile cilia syndrome) 2%
including Kartagener-Afzelius syndrome.
• Young syndrome 1-3%
• B-cell defects (common variable immunodeficiency,
selective IgA deficiency, immunoglobulin G subclass
deficiency) 3-9%
• Chronic granulomatous disease <1%
•Human immunodeficiency infection(controversial)
•Alpha1-antrypsin deficiency (controversial)
Normal lung: chloride into airway lumen; sodium out - keeps mucus moist and thin. Normal CFTR
regulates the sodium channel (inactivates it)

CF lung: chloride does not get into airway lumen; more sodium leaves. More salt in cell and
water comes in. This makes the mucus thick
Cilia in bronchiolar epithelium
 Situs inversus, bronchiectasis, and sinusitis and its relation to immotile cilia:
history of the diseases and their discoverers-Manes Kartagener and Bjorn Afzelius.

The relationship of Kartagener's syndrome to immobile cilia syndrome is a

fascinating merging of clinical observations and basic science in Zurich, Stockholm,
and Toronto. In 1933, Manes Kartagener, a Zurich pulmonary physician, reported
four patients with the triad of sinusitis, bronchiectasis, and situs inversus. In the
following decades, he reviewed reports of hundreds of cases, but the fact that the
male patients with the condition never had offspring eluded his notice. In the 1970s,
Bjorn Afzelius, a Ph.D. ultrastructuralist from Stockholm, reported cilia immotility
in infertile males, some of the cases occurring in families. Half of the cases had
Kartagener's triad. The observation of Afzelius was soon applied to children by
Jennifer Sturgess, a Ph.D. ultrastructuralist, and her medical colleagues in Toronto.
With over 500 MEDLINE references since 1966 on Kartagener's and over 1,000
references on immotile cilia, the causes of the pulmonary infections have become
clearer as the patients demonstrate impaired clearance of mucus with resultant
sinus and bronchial disease. The cause of the situs inversus remains elusive to this
day. It is appropriate to call the condition Kartagener-Afzelius syndrome.

Pediatr Radiol 2004;34:38-42.

DEXTROCARDIA IN KARTAGENER SYNDROME
BRONCHIECTASIS IN KARTAGENER SYNDROME
 SYSTEMIC DISEASES

• Inflammatory bowel disease (mainly ulcerative colitis)

• Rheumatoid arthritis
• Systemic lupus erythematosus
• Ankylosing spondylitis
• Sjögren syndrome
• Relapsing polychondritis
• Systemic sclerosis (uncommon)
• Sarcoidosis
• Yellow nail syndrome (rare)
 OTHER SECONDARY CAUSES
• Aspiration
• Idiopathic tracheobronchomegaly (Mounier-Kuhn
syndrome) (is dilatation of the trachea and major bronchi
because of atrophy or absence of elastic fibers and smooth
muscle cells)
• Williams-Campbell syndrome (constellation of airway
collapse, bronchiectasis, and bronchial cartilage deficiency).
• Aspiration of foreign bodies
• Diffuse panbronchiolitis

• Pulmonary fibrosis or fibrothorax (traction bronchiectasis)

 Mounier-Kuhn syndrome

Mounier-Kuhn P. Dilatation de la trachee: Constatations radio-

graphiques et bronchoscopiques. Lyon Medical. 1932;150:106–9.
SINDROMI BRONCHIECTASICHE

• Definizione
• Epidemiologia
• Eziologia
• Anatomia patologica
• Manifestazioni cliniche
• Diagnosi differenziale
• Trattamento
• Prevenzione
SINDROMI BRONCHIECTASICHE

• Definizione
• Epidemiologia
• Eziologia
• Anatomia patologica
• Manifestazioni cliniche
• Diagnosi differenziale
• Trattamento
• Prevenzione
CLINICAL FEATURES OF BRONCHIECTASIS IN ADULTS

• Cough 90%
• Daily sputum 76%
• Dyspnea 72%
• Hemoptysis 56%
• Pleuritic chest pain 46%
• Crackles 70%
• Wheeze 34%
• Clubbing 3%
• Secondary amyloidosis is a rare complication

Pasteur MC et al. Am J Respir Crit Care Med 2000;162:1277-1284.

LOWER AIRWAY FLORA IN BRONCHIECTASIS

• Haemophilus influenzae 17-55%

• Pseudomonas aeruginosa 24-31%
• Mucoid strain 0-14%
• Streptococcus pneumoniae 4-14%
• Moraxella catarrhalis 5-9%
• Staphylococcus aureus 3-8%
• Coliforms 4-13%
• Aspergillus 1-5%
• Nocardia 0-3%
• MAC 0-17%
• No organism 12-36%
SINDROMI BRONCHIECTASICHE

• Definizione
• Epidemiologia
• Eziologia
• Anatomia patologica
• Manifestazioni cliniche
• Diagnosi differenziale
• Trattamento
• Prevenzione
Rx torace nelle bronchiectasie
HRCT torace è diagnostica nelle bronchiectasie
 Normal pulmonary
artery (pearl)

Dilated bronchus
(ring;1.5 times as wide
as a nearby vessel)

SIGNET-RING SIGN
SINDROMI BRONCHIECTASICHE

• Definizione
• Epidemiologia
• Eziologia
• Anatomia patologica
• Manifestazioni cliniche
• Diagnosi differenziale
• Trattamento
• Prevenzione
 LIVELLI DI EVIDENZA

CATEGORIA FONTE

A Studi randomizzati controllati:

numerosi studi, consistenti
B Studi randomizzati controllati:
pochi studi, inconsistenti
C Studi non randomizzati e studi
osservazionali
D Giudizio di un gruppo di esperti
www.ginasthma.com
TREATMENT OF STABLE BRONCHIECTASIS
• Long-term antibiotic treatment? (level of evidence C)
• Inhaled aminoglycosides in patients colonized with P.
aeruginosa (level of evidence A)
• Bronchodilators? (SABA?, LABA?, AM?, LAM?)
• Chest physiotherapy (inspiratory muscle training)?
• Inhaled glucocorticoids?
• Mucolytics (bromexine level of evidence B)
• Surgery for selected cases of localized bronchiectasis (level
of evidence C)
• LTOT?
•Double lung transplantation (CF)
TREATMENT OF EXACERBATIONS OF BRONCHIECTASIS

• 2-week course of antibiotic treatment (level of evidence C).

• Oral glucocorticoids cycle?
• Human recombinant DNAse harmful (level of evidence B)
• Mucolytics/antioxidants? (level of evidence C)
SINDROMI BRONCHIECTASICHE

• Definizione
• Epidemiologia
• Eziologia
• Anatomia patologica
• Manifestazioni cliniche
• Diagnosi differenziale
• Trattamento
• Prevenzione