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CASE SNIPPET
Year : 2012  |  Volume : 58  |  Issue : 4  |  Page : 302-304

A potentially misdiagnosed suppurative and obstructive airway disease


Department of Pulmonary Medicine, T. N. Medical College, B. Y. L. Nair Hospital, Mumbai, Maharashtra, India

Date of Web Publication4-Jan-2013

Correspondence Address:
J M Joshi
Department of Pulmonary Medicine, T. N. Medical College, B. Y. L. Nair Hospital, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0022-3859.105459

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How to cite this article:
Tirpude S, Karkhanis V, Joshi J M. A potentially misdiagnosed suppurative and obstructive airway disease. J Postgrad Med 2012;58:302-4

How to cite this URL:
Tirpude S, Karkhanis V, Joshi J M. A potentially misdiagnosed suppurative and obstructive airway disease. J Postgrad Med [serial online] 2012 [cited 2020 Sep 20];58:302-4. Available from: http://www.jpgmonline.com/text.asp?2012/58/4/302/105459


A 45-year-old Indian male, who was a security guard and a non-addict, presented with symptoms of cough with copious and foul-smelling expectoration, associated with hemoptysis, which had been ongoing since he was 10 years old. There was history of repeated episodes of fever and increased expectoration. Patient had received empirical antituberculous therapy in the past. Medical history also included recurrent rhinosinusitis since childhood. On examination, his vital parameters were normal, with significant post-exercise oxygen saturation from 92% to 86% and grade 3 clubbing. Respiratory system examination was unremarkable except that on auscultation there were bilateral coarse crackles. Chest radiography revealed bilateral lung cystic opacities [Figure 1]. Sputum specimen was negative for mycobacteria. Pulmonary function test revealed obstructive ventilatory defect mainly affecting the small airways, with air trapping, forced expiratory volume at the end of one second (FEV 1 ) 1.37 L (3.56 L), forced vital capacity (FVC) 2.32 L (4.34 L), and FEV 1 /FVC 59%. A thoracic computed tomography (CT) scan confirmed the presence of extensive bilateral asymmetrical bronchiectasis varying from tubular to cystic, with peribronchial inflammation and thickening, patchy areas of air trapping throughout lung parenchyma [Figure 2]. The serum levels of immunoglobulin A and G subclasses were elevated. Rheumatoid factor was negative. He was started on low-dose Azithromycin therapy (250 mg daily). He experienced significant improvement soon after. Cough and sputum production disappeared and exertional breathlessness decreased. Chest radiography disclosed a significant reduction in the number of small nodular shadows. Repeat CT after 6 months of therapy revealed regression in peribronchiolar thickening and distal branching centrilobular densities; however, the tubular and cystic bronchiectasis remained unchanged [Figure 3]. Spirometry showed improvement in lung functions: FEV 1 1.80 L (51% predicted) increase by 430 ml and 31%, and FVC 3.48 L (80% predicted) increase by 1120 ml and 50%.
Figure 1: Chest radiography showing hyperinflated lung

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Figure 2: High-resolution computed tomography (HRCT) scan shows presence of extensive bilateral asymmetrical bronchiectasis varying from tubular to cystic, with peribronchial inflammation and thickening, patchy areas of air trapping throughout lung parenchyma

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Figure 3: A repeat CT scan after 6 months of low-dose macrolide therapy revealed regression in peribronchiolar thickening and distal branching centrilobular densities, although tubular and cystic bronchiectasis remained unchanged

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Our patient was diagnosed as a case of diffuse panbronchiolitis (DPB) according to clinical diagnostic criteria. [1] Human leukocyte antigen (HLA) Bw54 antigen, however, was not present. This HLA haplotype is not seen in all patients. [2] He showed good response to therapy on subjective and objective assessment.

High resolution computed tomography (HRCT) findings of DPB have four different types of lesions of increasing severity that progressively involve the more central airways. [1],[2] Hyperlucent areas related to air trapping often are noted in the peripheral third of the lung parenchyma. The natural history of DPB is progressive respiratory failure marked by chronic and/or frequent episodes of superimposed bacterial infection. The capacity for gas exchange is reduced, which causes progression of hypoxemia, and later, hypercapnea leading to pulmonary hypertension and cor pulmonale. [3] Macrolides have been shown to be effective therapy [4],[5] in DPB, although the precise mechanism is unknown. Anti-inflammatory actions rather than antimicrobial have been implicated including the reduction of pulmonary levels of pro-inflammatory cytokines interleukin 8, tumor necrosis factor alpha, and leukotriene B 4 , a potent neutrophil chemoattractant. [5]

There is significant overlap among various obstructive diseases. DPB is a suppurative and obstructive lung disease which responds to therapy and is often misdiagnosed due to less awareness. DPB is now reported outside of Asia. [3] The diagnosis may be easily made by clinical and radiological forms in countries where the disease is common, but in countries other than in East Asia, the disease is often unrecognized, underdiagnosed, or misdiagnosed, which leads to lack of appropriate treatment.

 
 :: References Top

1.Jadhav S, Joshi J. Diffuse panbronchiolitis associated with malignant thymoma. Indian J Chest Dis Allied Sci 2010;52:41-2.  Back to cited text no. 1
    
2.Li H, Zhou Y, Fan F, Zhang Y, Li X, Yu H, et al. Effect of Azithromycin on Patients with Diffuse Panbronchiolitis: Retrospective Study of 51 Cases. Intern Med 2011;50:1663-9.  Back to cited text no. 2
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3.Anthony M, Singham S, Soans B, Tyler G. Diffuse Panbronchiolitis: Not just an Asian disease: Australian case series and review of literature. Biomed Imaging Interv J 2009;5:e19.  Back to cited text no. 3
[PUBMED]    
4.Ding K, Liu MB, Wu JL, Ma HQ, Fang XY, Miao GB, et al. Diffuse panbronchiolitis in China: analysis of 45 cases. Chin Med J (Engl) 2007;120:2046-8.  Back to cited text no. 4
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5.Kudoh S, Azuma A, Yamamoto M, Izumi T, Ando M. Improvement of survival in patients with diffuse panbronchiolitis treated with low dose erythromycin. Am J Respir Crit Care Med 1998;157:1829-32.  Back to cited text no. 5
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3]

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2004 - Journal of Postgraduate Medicine
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