HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Services Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by King, T. K. Articles by Gelbman, B. D. Search for Related Content PubMed Articles by King, T. K. Articles by Gelbman, B. D.

Response to Long-Term Macrolide Therapy in a Patient with Cryptogenic Adult Bronchiolitis

Weill Cornell Medical Center, New York, NY

INTRODUCTION: Cryptogenic Adult Bronchiolitis is a rare clinicopathologic syndrome that represents a diagnostic challenge to clinicians. The pathologic findings are typical for cellular obliterative bronchiolitis, with acute and chronic inflammatory infiltrates in the bronchioles while the alveolar parenchyma is spared.[1] Cases are labeled cryptogenic when cultures and serology for infectious sources are negative and no etiology is elicited. The incidence and pathogenesis are unknown, though the disease tends to occur in middle-aged women who present with non-productive cough for several months.[1] Patients usually have a progressive course and there are no proven therapies. [2] Macrolide antibiotics have been shown to have dramatic effects on survival when used to treat patients with Diffuse Panbronchiolitis (DPB).[3] Given the current lack of effective therapy for Cryptogenic Adult Bronchiolitis, it seems reasonable to try long-term macrolide therapy in this unique patient population.

CASE PRESENTATION: A 46 year-old woman was evaluated in October 2003 for chronic cough of three months duration, without any apparent precipitating event, that did not respond to conventional therapies, including cough suppressants, short course antibiotics (azithromycin for five days), reflux medications and asthma inhalers. She had no significant past medical history and was otherwise in good health. Her clinical exam was normal except for diffuse, end-inspiratory squeaks. Pulmonary Function Tests revealed a restrictive pattern (Fig. 1 ). Initial chest CT showed increased interstitial markings, peripheral bronchiectasis and a mosaic pattern seen only on expiratory views. Laboratory testing was significant for negative ANA and RF. A bronchoscopy with lavage was performed and all subsequent cultures were negative, including viral, legionella, mycoplasma and mycobacterial. The patient was empirically started on prednisone 40mg qd, but after one month of therapy, she denied any symptomatic improvement and her pulmonary function tests were unchanged (Fig. 1). In addition, she developed an elevated fasting glucose and the steroids were discontinued. The patient underwent a surgical lung biopsy that revealed a patchy bronchiolocentric inflammatory process with minimally involved alveolar parenchyma. Airway lumens were distorted and narrowed by lymphoplasmacytic and neutrophilic infiltrates. (Fig 2 ) The clinical and pathologic diagnoses were consistent with Cryptogenic Adult Bronchiolitis. The patient recovered quickly from surgery, but still complained of chronic cough and limited exercise tolerance. She was empirically started on Clarithromycin 250mg bid. After one month of treatment, she stated that her cough had diminished and her dyspnea was improved. Pulmonary function tests were repeated and demonstrated improvement in FEV1, FVC, FEF 25-75%, and TLC (Fig. 1).

View larger version (15K): [in this window] [in a new window]

View larger version (178K): [in this window] [in a new window]

CONCLUSION: We believe this case is the first report using long-term macrolide therapy in patients with Cryptogenic Adult Bronchiolitis. The mechanism of disease and therapy are unknown, but may be related to DPB given the similarity in response. Further studies are warranted.

DISCLOSURE: B.D. Gelbman, None.

REFERENCES

1. Schwartz M, King TE. Interstitial Lung Disease, Fourth Edition, Hamilton: BC Decker;2003 :787 –824.
2. Ryu JH, Myers JL, et al. Bronchiolar Disorders, Am J Respir Crit Care Med,2003; 168 :1277 –1292.
3. Kudoh S, Azuma A, et al. Improvement of survival in patients with diffuse panbronchiolitis treated with low dose erythromycin. Am J Respir Crit Care Med,1998; 157 :1829 –1832.