Presentation
Recurrent pneumonia. History of Common Variable Immunodeficiency (CVID)
Patient Data
Lower zone predominant reticulo-nodular opacities with bilateral lung volume loss
Prior CT from 10 years ago
Bilateral reticulonodular opacities with apicobasal gradient
Bibasilar Consolidations
Diffuse thickening of the peribronchovascular interstitium
Lymphadenopathy
Marked progression of the findings since the previous CT. Irregular bronchocentric and peripheral nodules. Central cavitation is at least partly due to dilated bronchi. Confluent opacity in the lower zones with bronchiectasis and interlobular septal thickening. Progressive volume loss.
Mediastinal lymphadenopathy.
Liver and spleen incompletely seen. Possible splenomegaly. Hepatic vein stent.
Pathology Report
Sections show lung fragments covered by visceral pleura with moderate fibrosis and anthracotic tattoo. The lesion is predominantly bronchiolar and peribronchiolar with thickening of the interlobular interstitium due to lymphocyte infiltration and a few plasma cells. There are speckled areas of fibrosis, some subpleural, with architectural distortion, dense fibrosis with lymphoid accumulations, and isolated fibroblastic foci with a UIP-like pattern.
No honeycombing is observed.
Loose granulomas of epithelioid histiocytes and some giant cells with conchoidal bodies (Schaumann bodies) are seen. Some areas of intraalveolar fibroblastic organization with a pattern of organizing pneumonia and peribronchiolar fibrosis.
Diagnosis:
Lung parenchyma with patchy fibrosis peribronchiolar and interstitial loose granulomas with giant cells and Schaumann bodies
Case Discussion
Common variable immunodeficiency (CVID) is a relatively common cause of symptomatic primary immunodeficiency and is characterized by deficient production of antibodies.
In our patient with history of recurrent pneumonia the diagnosis was made several years after the onset of symptoms. The findings in his lung biopsy were initially misinterpreted as hypersensitivity pneumonitis due to the absence of clinical data.
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