Diffuse panbronchiolitis: an underdiagnosed disease? Study of 4 cases in Brazil

Souza, Rogério; Kairalla, Ronaldo Adib; Santos, Ubiratan de Paula; Takagaki, Teresa Yae; Capelozzi, Vera Luíza; Carvalho, Carlos Roberto Ribeiro

doi:10.1590/s0041-87812002000400007

Cited by 12 publications

(6 citation statements)

References 26 publications

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“…However, some cases have been described in non-Asian Brazilians. (61,62) The disease is predominantly seen between the second and fifth decade of life. Chronic sinusitis is common.…”

Section: Diffuse Panbronchiolitismentioning

confidence: 99%

Padrões tomográficos das doenças intersticiais pulmonares difusas com correlação clínica e patológica

et al. 2008

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High-resolution computed tomography (HRCT) is the radiological imaging technique best suited to revealing changes in lung structure. Various HRCT findings, taken together, can represent typical patterns. These patterns, in conjunction with the anatomical distribution of findings and with clinical data, can narrow the differential diagnosis of diffuse interstitial lung disease and, in many cases, indicate the correct diagnosis with a high degree of accuracy. The most common HRCT patterns seen in cases of diffuse interstitial lung diseases are the nodular pattern, linear/reticular opacities, cystic lesions, ground-glass opacities and consolidations. This article reviews the correlations between HRCT patterns and pathologic findings, summarizing the most common causes, as well as detailing the methods of investigation employed in order to diagnose the most common types of chronic diffuse lung disease.Keywords: Lung diseases, interstitial/pathology; Tomography, X-Ray computed; Diagnostic techniques, respiratory system. ResumoA tomografia de alta resolução (TCAR) é a técnica de imagem radiológica que reflete mais de perto as alterações da estrutura pulmonar. Os vários achados tomográficos podem ser combinados para formar padrões típicos. Estes, conjuntamente com a distribuição anatômica dos achados, e com os dados clínicos, podem estreitar o diagnóstico das doenças intersticiais pulmonares difusas, e em vários casos sugerir o diagnóstico correto com alto grau de acurácia. Os padrões mais comuns das doenças intersticiais pulmonares difusas na TCAR são o nodular, linear e reticular, lesões císticas, opacidades em vidro fosco e consolidações. Este artigo revisa as correlações entre os padrões tomográficos na TCAR e os achados patológicos e resume as causas mais comuns e os métodos de investigação para se atingir um diagnóstico nas doenças pulmonares crônicas difusas mais comuns.Descritores: Doenças pulmonares intersticiais/patologia; Tomografia computadorizada por raios X; Técnicas de diagnóstico do sistema respiratório.* Study carried out in the

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“…However, some cases have been described in non-Asian Brazilians. (61,62) The disease is predominantly seen between the second and fifth decade of life. Chronic sinusitis is common.…”

Section: Diffuse Panbronchiolitismentioning

confidence: 99%

Padrões tomográficos das doenças intersticiais pulmonares difusas com correlação clínica e patológica

et al. 2008

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“…It was originally reported in Japan (2). However, an increasing number of cases have been reported in Europe (3,4) and America (5)(6)(7)(8)(9), as well as in Asia (10), including Thailand (11), India (12), and China (13)(14)(15)(16)(17)(18)(19)(20).…”

Section: Introductionmentioning

confidence: 99%

Effect of Azithromycin on Patients with Diffuse Panbronchiolitis: Retrospective Study of 51 Cases

Zhou

Feng

et al. 2011

Intern. Med.

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Background Patients with diffuse panbronchiolitis (DPB) are routinely treated with erythromycin, clarithromycin, and roxithromycin. The clinical effect of azithromycin on DPB has not been confirmed in a large cohort. Objective The present study was undertaken to investigate the clinical effects of azithromycin on patients with DPB. Methods Fifty-one patients with DPB treated with azithromycin in Shanghai Pulmonary Hospital, China, from July 2001 to April 2007 were analyzed retrospectively. Azithromycin (500 mg a day) was administrated intravenously in the first 1-2 weeks, taken orally (500 mg, once a day) for 3 months, and tapered to 3 times a week for 6-12 months. The patients were followed up until September 1, 2009. The therapeutic effect, according to their clinical and radiological findings, arterial gas analysis, lung function, and sputum bacterium before and after the therapy, was categorized into the following five grades: 1) cured; 2) improved; 3) no response; 4) aggravation, and 5) relapse. Results With azithromycin therapy, 14 (27.5%) patients with DPB were completely cured. The symptoms were eliminated to certain degrees for the other 36 cases (70.6%) of DPB. Five-year survival in this cohort was 94.1%. Conclusion Azithromycin is effective and well tolerated for patients with diffuse panbronchiolitis.

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“…In the years following the initial description of DPB in Japan, cases were recognised in other parts of Asia, including Taiwan, Korea, China, Malaysia, Thailand and Singapore. More recently, DPB has been encountered in Caucasian, Hispanic and African-American populations in Italy, France, the UK, Germany, Norway and the USA [8][9][10][11][12][13][14][15][16][17][18][19][20]. One case has been reported in a child [21].…”

mentioning

confidence: 99%

Diffuse panbronchiolitis

Poletti¹

2006

European Respiratory Journal

126

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Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, well recognised in Japan and principally affecting the respiratory bronchioles, causing a progressive suppurative and severe obstructive respiratory disorder. If left untreated, DPB progresses to bronchiectasis, respiratory failure and death.It was first described in the early 1960s. Subsequently, in 1969, the disease was named DPB to distinguish it from chronic bronchitis. ''Diffuse'' refers to the distribution of the lesions throughout both lungs, and ''pan-'' refers to the involvement of inflammation in all layers of the respiratory bronchioles.The distinctive imaging and histological features, the coexisting sinusitis, and the isolation of Haemophilus influenzae and Pseudomonas aeruginosa in the sputum enhance disease recognition. Histologically, DPB is characterised by chronic inflammation, localised mainly in the respiratory bronchioles and adjacent centrilobular regions, with characteristic interstitial accumulation of foamy histiocytes, neutrophils and lymphocyte infiltration. Neutrophils and Tlymphocytes, particularly CD8+ cells, together with the cytokines interleukin-8 and macrophage inflammatory protein-1, are believed to play key roles in the development of DPB.A significant improvement in the prognosis of this potentially fatal disease has been recently reported thanks to the use of long-term therapy with macrolide antibiotics, the effect of which is attributed to an anti-inflammatory and immunoregulatory action.

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Diffuse panbronchiolitis: an underdiagnosed disease? Study of 4 cases in Brazil

Cited by 12 publications

References 26 publications

Padrões tomográficos das doenças intersticiais pulmonares difusas com correlação clínica e patológica

Padrões tomográficos das doenças intersticiais pulmonares difusas com correlação clínica e patológica

Effect of Azithromycin on Patients with Diffuse Panbronchiolitis: Retrospective Study of 51 Cases

Diffuse panbronchiolitis

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