Chest
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Patients with common variable immunodeficiency (CVID) can develop granulomatous-lymphocytic interstitial lung disease (GLILD), which is associated with increased morbidity and mortality. Treating GLILD is a significant challenge because it is rare and can be pathologically heterogeneous. ⋯ Based on the pivotal role that B cells play in TLS initiation and maintenance, we hypothesized that using rituximab monotherapy for B-cell depletion alone would be sufficient for the disruption of the pathologic process underlying GLILD. These two cases demonstrate that adapting a strategy of B cell depletion monotherapy may be effective in TLS-associated conditions such as GLILD.
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A previously healthy 45-year-old man was admitted to our ED with a 3-week history of progressive dyspnea on exertion. He also presented with orthopnea, paroxysmal nocturnal dyspnea, and mild ankle swelling, but he showed no fever, wheezing, coughing, or sputum production. Outpatient laboratory studies, performed 1 week after symptom onset, revealed hypereosinophilia (4.100/μL). ⋯ Before symptom onset, he had not been taking any medication. He denied eating raw fish or meat and had not been exposed to mildew. His only exposure to animals was from his two indoor cats.