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Abstract: Case Reports |

27 year old female with Common Variable Immunodeficiency, recurrent pneumonia and endobronchial “cobblestoning” due to bronchus associated lymphoid tissue FREE TO VIEW

Adey Tsegaye, MD*; Patricia Villamena, MD; Abhijith Hegde, MD; Ya Ju Chang, MD
Author and Funding Information

Beth Israel Medical Center, New York, NY


Chest


Chest. 2004;126(4_MeetingAbstracts):927S. doi:10.1378/chest.126.4_MeetingAbstracts.927S
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INTRODUCTION:  Common variable immunodeficiency (CVID)predisposes to recurrent sinopulmonary infections, but other thoracic manifestations are unusual. We report a patient with CVID and bronchus associated lymphoid tissue (BALT).

CASE PRESENTATION:  27-year-old female presented with fever, shortness of breath, pleuritic chest pain and productive cough. This patient had asthma since childhood and recurrent ear infections in the last five years. Two years prior to admission, she had a left-sided pneumonia. Twenty days prior to admission she presented to the Emergency Department (ED) with worsening asthma and fever. A chest x-ray (CXR) done at that time showed a lingular pneumonia, and she was discharged on oral moxifloxacin, fluticasone/salmeterol and prednisone. The patient returned to the ED three weeks later with no improvement in symptoms, and the CXR showed extension of the pneumonia into the left lower lobe. The patient never smoked, used illicit drugs or used alcohol. On physical exam, the patient appeared comfortable. The temperature was 37°C, pulse 72 beats/minute, blood pressure 108/65, respirations 18/minute, O2 saturation 96% on room air. The head, ears, eyes, nose and throat were normal. The heart had no murmurs, rubs or gallops. She had diminished breath sounds at the left base with crackles in the left lower and mid-lung zones, with diffuse mild end-expiratory wheezing. The abdomen and extremities were normal. The white blood count was 14,500/mm3 with 80% granulocytes. Serum chemistries and liver function tests were normal. She was HIV seronegative. Computerized tomography of the chest showed multifocal airspace infiltrates, some with a nodular appearance and others, showing variable degrees of consolidation and ground glass opacity. The lingula and basal segments of the lower lobe are affected asymmetrically. No definite underlying anatomic abnormalities were seen nor, was there evidence of bronchiectasis or congenital deformities. Immunoglobulin assays were performed and serum IgA, IgE, IgM and IgG (including all subclasses) were all markedly reduced. Complement ( CH50 ) and Tcell abnormalities (CD4, CD8, CD4/CD8 ratio and Candidal Anergy panel) were negative. As patient has recurrent sinopulmonary infections with depressed immunoglobulin levels, a diagnosis of CVID was established. Flexible bronchoscopy revealed a diffuse bilateral cobblestone appearance of the airway at and below the level of the main carina and, purulent secretions in the lingula. Endobronchial biopsy revealed BALT.

DISCUSSIONS:  CVID is characterized by low levels of immunoglobulins and an increased susceptibility to infections. It typically occurs in the second or third decade of life, presenting with recurrent sinopulmonary infections that my lead to bronchiectasis. CVID is associated with lymph node and spleen enlargement and occasionally with enlargement of gut-associated lymphoid tissue (Peyer’s patches). CVID is also associated with an increased incidence of lymphoma, gastric cancer, inflammatory bowel disease, and autoimmune disorders. Sarcoid-like granulomas can occur in the skin, gut and other viscera; in this case, the bronchoscopic appearance of the airways looked like that of sarcoidosis. BALT is a localized collection of lymphocytes in the subepithelial area of the bronchi. Unlike Peyer’s patches, which occur in normal gut mucosa, BALT is not present in normal adult lungs, but may appear in patients with chronic respiratory infection and autoimmune disorders. BALT is associated with the development of extranodal low-grade B-cell lymphomas.

CONCLUSION:  To our knowledge, this is the only report of diffuse endobronchial BALT in association with CVID.

DISCLOSURE:  A. Tsegaye, None.

Monday, October 25, 2004

4:15 PM - 5:45 PM

References

Rosen FS, Cooper MD, Wedgwood RJP. The primary immunodeficiencies.N Engl J Med1995;333:431–440. [CrossRef]
 
Takafumi S, Chida K, Hayakawa H, et al. Development of bronchus associated lymphoid tissue in chronic hypersensitivity pneumonitis.Chest1999;115:357–363. [CrossRef]
 
Lee DK, Im J-G, Lee KS, et al. B-Cell lymphoma of bronchus associated lymphoid tissue: CT features in 10 patients.J Comp Assist Tomog2000;24:30–34. [CrossRef]
 

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References

Rosen FS, Cooper MD, Wedgwood RJP. The primary immunodeficiencies.N Engl J Med1995;333:431–440. [CrossRef]
 
Takafumi S, Chida K, Hayakawa H, et al. Development of bronchus associated lymphoid tissue in chronic hypersensitivity pneumonitis.Chest1999;115:357–363. [CrossRef]
 
Lee DK, Im J-G, Lee KS, et al. B-Cell lymphoma of bronchus associated lymphoid tissue: CT features in 10 patients.J Comp Assist Tomog2000;24:30–34. [CrossRef]
 
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