Archived case 201 · April 29-May 6, 2016
50-year-old female, no acute symptoms.
What is your differential diagnosis? CT 1 is from 2005, CT 2 and radiographs are recent.
OBSERVATIONS Radiographs demonstrate bibasilar reticulonodular opacities. CT images in the lung window show centrilobular nodules, ground glass opacities and cylindrical bronchiectasis. Expiratory image shows air trapping. CT image of the abdomen (soft tissue window) demonstrates splenomegaly. No enlarged lymph nodes. Answer Diagnosis: Follicular bronchiolitis (pathology proven) in a patient with common variable immunodeficiency (CVID).
Follicular bronchiolitis (pathology proven) in a patient with common variable immunodeficiency (CVID).
Answer
Diagnosis: Follicular bronchiolitis (pathology proven) in a patient with common variable immunodeficiency (CVID).
DISCUSSION
Follicular bronchiolitis is defined as polyclonal peribronchilar lymphoid hyperplasia. Many cases of follicular bronchiolitis are associated with various systemic illnesses including connective tissue diseases ( Sjogren syndrome, rheumatoid arthritis ), immunodeficiency states (CVID, AIDS ), hypersensitivity disorders associated with peripheral eosinophilia. It can be also familiar or idiopathic. As opposite to lymphoid interstitial pneumonia, follicular bronchiolitis is limited to the airways without diffuse interstitial involvement.
The differential diagnosis for CT findings includes: infection, hypersensitivity pneumonitis, and respiratory bronchiolitis. Surgical lung biopsy is usually necessary to establish the diagnosis. The prognosis is generally good, however younger patient may develop progressive disease.
CT findings of follicular bronchiolitis include small centrilobular nodules 1 to 12 mm in size, usually less than 3 mm, occasionally with a tree-in-bud configuration. In addition, peribronchial nodules, nodules along the septa and pleura, ground glass opacities, and cysts may be seen. Infrequent findings include bronchiectasis, bronchial wall thickening, and peribronchovascular consolidation.
CVID is the most common symptomatic primary immunodeficiency characterized by hypogammaglobulinemia (usually also decrease of IgA and/or IgM antibodies) and recurrent infections (most frequent with H. influenzae, S. pneumoniae and S. aureus). CT chest findings include bronchiectasis possibly with interstitial lung disease, pulmonary nodules, ground glass opacities, as well as intrathoracic or intra-abdominal lymphadenopathy and/ or hepatosplenomegaly.
Selected Readings:
1. Howling SJ, Hansell DM, Wells AU et al. Follicular bronchiolitis: thin-section CT and histologic findings. Radiology. 1999;212 (3): 637-42
2. Sirajuddin A, Raparia K, Lewis VA et al. Primary Pulmonary Lymphoid Lesions: Radiologic and Pathologic Findings. RadioGraphics 2016 36:1, 53-70
3. Travis WD, Galvin JR. Non-neoplastic pulmonary lymphoid lesions. Thorax 2001;56(12):964–971.
4. Maglione PJ, Overbey JR, Radigan L et-al. Pulmonary radiologic findings in common variable immunodeficiency: clinical and immunological correlations. Ann. Allergy Asthma Immunol. 2014;113 (4): 452-9.
5. Hare SS , Souza CA , Bain G , et al. The radiological spectrum of pulmonary lymphoproliferative disease . Br J Radiol 2012 ;85(1015): 848 – 864 .
6. Pipavath SJ, Lynch DA, Cool C et-al. Radiologic and pathologic features of bronchiolitis. AJR Am J Roentgenol. 2005;185 (2): 354-63.
Original case written by its authors at Virginia Commonwealth University and published at this address as part of a weekly teaching collection. Reproduced here as an archive.