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Vol. 17. Issue 1.
Pages 32-40 (January - February 2011)
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Vol. 17. Issue 1.
Pages 32-40 (January - February 2011)
Open Access
Bronquiectasias: Será necessária a investigação etiológica?
Bronchiectasis: do we need aetiological investigation?
Visits
8623
A. Amorima,
Corresponding author
adelinamorim@gmail.com

Autor para correspondência.
, J. Gracia Róldanb
a Serviço de Pneumologia, Hospital São João - EPE, Faculdade de Medicina, Universidade do Porto, Porto, Portugal
b Serviço de Pneumologia, Hospital Universitari Vall d’ Hebron, Barcelona; Universidad Autónoma de Barcelona, Barcelona, Espanha
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Resumo

As bronquiectasias (BE) e caracterizam-se por uma dilatação e destruição irreversível das paredes brônquicas. São a expressão morfológica de uma grande variedade de patologias.

A verdadeira prevalência das BE é desconhecida, contudo sabe-se que são frequentes nos países de Terceiro Mundo devido a infecções pulmonares. A sua prevalência nos países desenvolvidos é também significativa em consequência da maior capacidade de diagnóstico, da sua associação com doenças de prevalência elevada e da maior cronicidade de algumas das patologias subjacentes.

Durante as últimas décadas tem havido pouco interesse na investigação das BE, exceptuando as consequentes à fibrose quística. Uma das razões relaciona-se com o pressuposto de que o tratamento é comum a todos os doentes com BE, independentemente da etiologia subjacente. Vários trabalhos nesta última década demonstraram que a obtenção de um diagnóstico etiológico modifica a abordagem e o tratamento numa percentagem relevante de doentes e consequentemente o seu prognóstico.

Recomenda-se actualmente a investigação sistemática da etiologia, principalmente das doenças que poderão responder a um tratamento específico.

Palavras-chave:
Bronquiectasias
Etiologia
Investigação
Abstract

Bronchiectasis (BE) is characterized by irreversible dilation and damage to the bronchial walls. It is a morphological expression of a large variety of pathologies.

The true prevalence of BE is unknown. It is known, however, that in Third World countries it is common due to lung infections. Its current prevalence in developed countries is rather significant due to a greater capacity for diagnosis, its association with highly prevalent diseases and to a greater chronicity of some of the underlying pathologies.

Over the last few decades there has been little interest in the investigation of BE, unless it is associated with cystic fibrosis. One of the reasons is the presupposition that treatment is the same for all patients diagnosed with BE, regardless of the underlying aetiology. Several works carried out over the last decade show that a diagnosis based on aetiology changes both the approach and the treatment of BE within a relevant percentage of patients, with a consequent change in the prognosis.

Currently, systematic investigation into the aetiology of BE is recommended, particularly in those disorders that respond to specific treatment.

Keywords:
Bronchiectasis
Aetiology
Investigation
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Bibliografia
[1.]
R.T.H. Laennec.
A treatise on the disease of the chest,
[2.]
A.F. Barker.
Bronchiectasis.
N England J Med, 346 (2002), pp. 1383-1393
[3.]
L.M. Reid.
Reduction in bronchial subdivision in bronchiectasis.
Thorax, 5 (1950), pp. 233-247
[4.]
P. Mounier-Kuhn.
Dilatation de la trachée: constatations radiographiques et bronchoscopiques.
Lyon Med, 150 (1932), pp. 106-109
[5.]
D. Farber.
Pancreatic function and disease in early life.
Arch Path, 37 (1944), pp. 328
[6.]
W.H.C. Campbell.
Generalized bronchiectasis associated with deficiency of cartilage in the bronchial tree.
Dis Child, 35 (1960), pp. 182-191
[7.]
P.D. Samman, W.F. White.
The “yellow nail” syndrome.
Br J Dermatol, 76 (1964), pp. 153-157
[8.]
A. Yamanaka, S. Saiki, S. Tamura, et al.
Problems in chronic obstructive bronchial diseases, with special reference to diffuse panbronchiolitis.
Naika, 23 (1969), pp. 442-451
[9.]
D. Young.
Surgical treatment of male infertility.
J Reprod Fertile, 23 (1970), pp. 541-542
[10.]
J.M. Reich, R.E. Johnson.
Mycobacterium avium complex pulmonary infection presenting as isolated lingular or middle lobe pattern: the Lady Windermere syndrome.
Chest, 101 (1992), pp. 1605-1609
[11.]
M. Cohen, S.A. Sahn.
Bronchiectasis in systemic diseases.
Chest, 116 (1999), pp. 1063-1074
[12.]
J.A. Driscoll, S. Bhalla, H. Liapis, et al.
Autosomal Dominant polycystic kidney disease is associated with an increased prevalence of radiographic bronchiectasis.
Chest, 133 (2008), pp. 1181-1188
[13.]
A.F. Barker, E.J. Bardana.
Bronchiectasis: update of an orphan disease.
Am Rev Respir Dis, 137 (1988), pp. 969-978
[14.]
G.J. Brewer.
The orphan drug/orphan disease problem: has it been resolved?.
Pharm Int Dec, 5 (1984), pp. 297-300
[15.]
Ministry of Health.
Chief Medical Officer Reports.
Her Majesty's Stationery Office, (1947),
[16.]
N. Wynne-Williams.
Bronchiectasis: a study centred on Bedford and its environs.
BMJ, i (1953), pp. 1194-1199
[17.]
D. Weycker, J. Edelsberg, G. Oster, et al.
Prevalence and economic burden of bronchiectasis.
Clinical Pulmonary Medicine, 12 (2005), pp. 205-209
[18.]
R. Singleton, A. Morris, G. Redding, et al.
Bronchiectasis in Alaska native children: causes and clinical courses.
Pediatr Pulmonol, 29 (2000), pp. 182-187
[19.]
A.B. Chang, K. Grimwood, E.K. Mulholland, et al.
Bronchiectasis in indigenous children in remote Australian communities.
Med J Aust, 177 (2002), pp. 200-204
[20.]
E.A. Edwards, M.I. Asher, C.A. Byrnes.
Paediatric bronchiectasis in the twenty-first century: experience of a tertiary children's hospital in New Zealand.
J Paediatric Child Health, 39 (2003), pp. 111-117
[21.]
O. Säynäjäkangäs, T. Keistinem, T.O. Tuuponem, et al.
Evaluation of the incidence and age distribution of bronchiectasis from de Finish Hospital Discharge Register.
Cent Eur J Public Health, 6 (1998), pp. 235-237
[22.]
J. Twiss, R. Metcalfe, E. Edwards, et al.
New Zealand national incidence of bronchiectasis “too high” for a developed country.
Arch Dis Child, 90 (2005), pp. 737-740
[23.]
Hospital Episode Statistics 2002–2003. Department of Health. NHLBI. UK; 2003–2004.
[24.]
T. Keistinem, O. Säynäjäkangäs, T. Tuuponem, et al.
Bronchiectasis: an orphan disease with poorly-understood prognosis.
Eur Respir J, 10 (1997), pp. 2784-2787
[25.]
K.M. Eastham, A.J. Fall, L. Mitchell, et al.
The need to redefine non-cystic fibrosis bronchiectasis in childhood.
Thorax, 59 (2004), pp. 324-327
[26.]
M.C. Pasteur, S.M. Helliwell, S.J. Houghton, et al.
An investigation into causative factors in patients with bronchiectasis.
Am J Respir Crit Care Med, 162 (2000), pp. 1277-1284
[27.]
M.J.R. Rock.
Newborn screening for cystic fibrosis.
Clin Chest Med, 28 (2007), pp. 297-305
[28.]
M.A. Martínez-Garcia.
Bronquiectasias no debidas a fibrosis quística. Monografías en Neumología.
Diagnóstico por la imagen en las bronquiectasias, 3 (2008), pp. 47-71
[29.]
A.B. Chang, D. Bilton.
Exacerbations in cystic fibrosis: 4-Non-cystic fibrosis bronchiectasis.
Thorax, 63 (2008), pp. 269-276
[30.]
I.S. Patel, I. Vlahos, T.M.A. Wilkinson, et al.
Bronchiectasis, exacerbation indices, and inflammation in chronic obstructive pulmonary disease.
Am J Respir Crit Care Med, 170 (2004), pp. 400-407
[31.]
A. Shoemark, L. Ozerovitch, R. Wilson.
Aetiology in adult patients with bronchiectasis.
Respiratory Medicine, 101 (2007), pp. 1163-1170
[32.]
A.M. Li, S. Sonnappa, C. Lex, et al.
Non-CF bronchiectasis: does knowing the aetiology lead to changes in management?.
Eur Resp J, 26 (2005), pp. 8-14
[33.]
M. Vendrell, J. Gracia, C. Olveira, et al.
Diagnóstico y tratamiento de las bronquiectasias. Normativas SEPAR.
Arch Bronconeumol, 44 (2008), pp. 629-640
[34.]
B. Rosenstein, G. Cutting.
The diagnosis of Cystic Fibrosis: a consensus statement.
Journal of Pediatrics, 132 (1998), pp. 589-595
[35.]
J.R. Yankaskas, B.C. Marshall, B. Sufian, et al.
Cystic Fibrosis Adult Care Consensus Conference Report.
Chest, 125 (2004), pp. 15-39
[36.]
Cystic Fibrosis Foundation.
Patient Registry 2007 - Annual date report.
Cystic Fibrosis Foundation, (2008),
[37.]
The Cystic fibrosis genetic analysis consortium, CFGAG. http://www.genet.Sickkids.on.ca/cftr. Update Mar 02, 2007.
[38.]
J. De Gracia, F. Mata, A. Álvarez, et al.
Genotype-phenotype correlation for pulmonary function in cystic fibrosis.
Thorax, 60 (2005), pp. 558-563
[39.]
Rafinia MN, Linde L, Keron B. The CFTR gene: structure, mutations and specifi c therapeutic approaches. In CF in the 21st Century. 2005; Karger. p. 2–10.
[40.]
K. Boeck, M. Wilschanski, C. Taylor, et al.
Cystic fibrosis: terminology and diagnostic algorithms.
Thorax, 61 (2006), pp. 627-635
[41.]
Widerman E, Palys B, Palys JR. The phenomenon of adult diagnosis. “Now that I have CF”. Information for men and women diagnosed as adults; Solvay pharmaceuticals. p. 13–47.
[42.]
P.L. Shah, S. Mawdsley, K. Nash, et al.
Determinants of chronic infection in patients with bronchiectasis.
Eur Respir J, 14 (1999), pp. 1340-1344
[43.]
J. De Gracia, A. Álvarez, F. Mata, et al.
Cystic fibrosis in adults: study of 111 patients.
Med Clin (Barc), 119 (2002), pp. 605-609
[44.]
D. Hubert, I. Fajac, T. Bienvenu, et al.
Diagnosis of cystic fibrosis in adults with diffuse bronchiectasis.
Journal of cystic fibrosis, 3 (2004), pp. 15-22
[45.]
S.J. Fowler, J. French, N.J. Screaton, et al.
Nontuberculous mycobacteria in bronchiectasis: prevalence and patient characteristics.
Eur Respir J, 28 (2006), pp. 1204-1210
[46.]
M. Wickremasinghe, L.J. Ozerovitch, G. Davies, et al.
Non-tuberculous mycobacteria in patients with bronchiectasis.
Thorax, 60 (2005), pp. 1045-1051
[47.]
D.E. Griffith, T. Aksamit, B.A. Brown-Elliott, et al.
An official ATS/IDSA statement: diagnosis, treatment, and prevention of nontuberculous mycobacterial diseases.
Am J Respir Crit Care Med, 175 (2007), pp. 367-416
[48.]
D.S. Prince, D.D. Peterson, R.M. Steiner, et al.
Infection with Mycobacterium avium complex in patients without predisposing conditions.
N England J Med, 321 (1989), pp. 863-868
[49.]
J.M. Reich, R.E. Johnson.
Mycobacterium avium complex pulmonary disease presenting as an isolated lingular or middle lobe pattern. The Lady Windermere syndrome.
Chest, 101 (1992), pp. 1605-1609
[50.]
P. Tutor-Ureta, S. Mello-Pita, Yebra-Bango., et al.
Bronquiectasias en el lóbulo medio e infección por Mycobacterium avium complex: síndrome de Lady Windermere.
Enferm Infecc Microbiol Clin, 24 (2006), pp. 590-594
[51.]
E.A. Waller, A. Roy, L. Brumble, et al.
The expanding spectrum of Mycobacterium avium complex-associated pulmonary disease.
Chest, 130 (2006), pp. 1234-1241
[52.]
J.H. Huang, P.N. Kao, V. Adi, et al.
Mycobacterium aviumintracellulare pulmonary infection in HIV- negative patients without pre-existing lung disease.
Chest, 115 (1999), pp. 1033-1040
[53.]
R.D. Kim, D.E. Greenberg, M.E. Ehrmantraut, et al.
Pulmonary nontuberculous mycobacterial disease. Prospective study of a distinct pre-existing syndrome.
Am J Respir Crit Care Med, 178 (2008), pp. 1066-1074
[54.]
S.K. Field, D. Fisher, R.L. Cowie.
Mycobacterium avium complex pulmonary disease in patients without HIV infection.
Chest, 126 (2004), pp. 566-581
[55.]
S.J. Swensen, T.E. Hartman, D.E. Williams.
Computed tomographic diagnosis of Mycobacterium avium-intracellulare complex in patients with bronchiectasis.
Chest, 105 (1994), pp. 49-52
[56.]
M.D. Iseman.
That's no Lady.
Chest, 109 (1996), pp. 1411
[57.]
P. Sexton, A.C. Harrison.
Susceptibility to nontuberculous mycobacterial lung disease.
Eur Respir J, 31 (2008), pp. 1322-1333
[58.]
Y.J. Jeon, K.S. Lee, W.J. Koh, et al.
Nontuberculous mycobacterial pulmonary infection in imunocompetent patients: comparison of thin-section CT and histopathologic findings.
Radiology, 231 (2004), pp. 880-886
[59.]
P. Wood, S. Stanworth, J. Burton, et al.
Recognition, clinical diagnosis and management of patients with primary antibody deficiencies: a systematic review.
Clinical and experimental immunology, 149 (2007), pp. 410-423
[60.]
F.A Bonilla, I.L. Bernstein, D.A. Khan, et al.
Practice parameter for the diagnosis and management of primary immunodeficiency.
Annals of allergy, asthma & immunology, 94 (2005), pp. S1-S63
[60.]
Sorensen RU, Paris K. Selective antibody deficiency with normal immunoglobulins (polysaccharide non-responses). UpToDate online version 17.3 2009.
[62.]
M. Vendrell, J. Gracia, M.J. Rodrigo, et al.
Antibody production deficiency with normal IgG levels in bronchiectasis of unknown etiology.
Chest, 127 (2005), pp. 197-204
[63.]
B.T. Costa-Carvalho, R.R. Cocco, W.M. Rodrigues, et al.
Pneumonias de repetição em paciente com deficiência de anticorpos e imunoglobulinas normais.
J Pneumol, 28 (2002), pp. 155-158
[64.]
J. De Gracia, M. Vendrell, A. Álvarez, et al.
Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency.
Int Immunopharmacol, 4 (2004), pp. 745-753
[65.]
A. Bush, R. Chodhari, N. Collins, et al.
Primary ciliary dyskinesia: current state of the art.
Arch Dis Child, 92 (2007), pp. 1136-1140
[66.]
A. Bush, C. O’Callaghan.
Primary ciliary dyskinesia.
Arch Dis Child, 87 (2002), pp. 363-365
[67.]
P.G. Noone, W. Leigh, A. Sannuti, et al.
Primary ciliary dyskinesia. Diagnostic and phenotypic features.
Am J Respir Crit Care Med, 169 (2004), pp. 459-467
[68.]
T. Ferkol, H.M. Mitchison, C. O’Callaghan, et al.
Current issues in the basic mechanisms, pathophysiology, diagnosis and management of primary ciliary dyskinesia: diagnosis and standards of care.
Eur Resp Mon, 37 (2006), pp. 291-313
[69.]
R. Corbelli, B. Bringolf-Isler, A. Amacher, et al.
Nasal nitric oxide measurements to screen children for primary ciliary dyskinesia.
Chest, 126 (2004), pp. 1054-1059
[70.]
D.R. Taylor, M.W. Pijnenburg, A.D. Smith, et al.
Exhaled nitric oxide measurements: clinical application and interpretation.
Thorax, 61 (2006), pp. 817-827
[71.]
D.G. Parr, P.G. Guest, J.H. Reynolds, et al.
Prevalence and impact of bronchiectasis in a1-antitripsin deficiency.
AM J Respir Crit Care Med, 176 (2007), pp. 1215-1221
[72.]
W.R. Cintron, K. Guntupalli, E. A Fraire.
Bronchiectasis and homozygous (P1ZZ) alpha 1-antitrypsin deficiency in a young man.
Thorax, 50 (1995), pp. 424-425
[73.]
J.H. Scott, C.L. Anderson, P.S. Shankar, et al.
Alpha1-antitrypsin deficiency with diffuse bronchiectasis and cirrhosis of the liver.
Chest, 71 (1977), pp. 535-538
[74.]
G.F. Longstreth, S.A. Weitzman, R.J. Browning, et al.
Bronchiectasis and homozygous alpha1-antitrypsin deficiency.
Chest, 67 (1975), pp. 233-235
[75.]
American Thoracic Society/European Respiratory Society Statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency.
Am J Respir Crit Care Med, 168 (2003), pp. 818-900
[76.]
V. Poletti, G. Casoni, M. Chilosi, et al.
Diffuse panbronchiolitis.
Eur resp J, 28 (2006), pp. 862-871
[77.]
J.H. Ryu, J.L. Myers, S.J. Swensen.
Bronchiolar disorders. State of the art.
Am J Respir Crit Care Med, 168 (2003), pp. 1277-1292
[78.]
E.E. McGrath, A.M. McLaughlin, M.X. Fitzgerald.
Diffuse panbronchiolitis: east meets west.
Eur Respir J, 29 (2007), pp. 817-818
[79.]
N.P. Adams, J. Congelton.
Diffuse panbronchiolitis.
Eur Respir J, 32 (2008), pp. 237-238
[80.]
R. Agarwal.
Allergic bronchopulmonary aspergillosis.
Chest, 135 (2009), pp. 805-826
[81.]
P.A. Greenberger.
Allergic bronchopulmonary aspergillosis.
J Allergy Clin Immunol, 110 (2002), pp. 685-692
[82.]
K.K. Ricaurté, P.A. Greenberger, D.A. Fullerton.
Allergic bronchopulmonary aspergillosis with multiple Streptococcus pneumoniae lung abscesses: an unusual initial case presentation.
J Allergy Clin Immunol, 104 (1999), pp. 238-240
[83.]
A. Shah.
Allergic bronchopulmonary aspergillosis: an Indian perspective.
Curr Opin Pulm Med, 13 (2007), pp. 72-80
[84.]
R. Kumar.
Mild, moderate, and severe forms of allergic bronchopulmonary aspergillosis.
Chest, 124 (2003), pp. 890-892
[85.]
M.A. Martínez-Garcia, M. Perpiña-Tordera, P. Román-Sanchez, et al.
Quality-of-life determinants in patients with clinically stable bronquiectasis.
Chest, 128 (2005), pp. 739-745
[86.]
M.A. Martínez-Garcia, J.B. Soriano.
Phsiotherapy in bronchiectasis: we have more patients, we need more evidence.
Eur Respir J, 34 (2009), pp. 1011-1012
[87.]
V.M. Smith, A. Shoemark, M. Nisbet, et al.
When to think of bronchiectasis and the investigations to perform.
Clinical Pulmonary Medicine, 17 (2010), pp. 7-13
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