A 32-year-old Caucasian female presents to your office with persistent cough and shortness of breath. She has had three episodes of pneumonia over the last year. She had severe sinusitis one year ago, and an episode of bloody diarrhea that required hospitalization and IV antibiotic therapy six months ago. She does not smoke or consume alcohol. She denies any illicit drug use. She is currently not taking any medications. All her immunizations are up-to-date. Her blood pressure is 130/80 mmHg and heart rate is 90/min. Physical examination reveals fine crackles over the right lower lung lobe. No lower extremity edema is present. Neck palpation does not reveal any lymph node enlargement. The chest x-ray shows right lower lobe infiltrates and left upper lobe fibrosis. The ECG reveals non-specific ST segment and T wave changes. What is the best next step in the management of this patient?

Question

A 32-year-old Caucasian female presents to your office with persistent cough and shortness of breath.  She has had three episodes of pneumonia over the last year.  She had severe sinusitis one year ago, and an episode of bloody diarrhea that required hospitalization and IV antibiotic therapy six months ago.  She does not smoke or consume alcohol.  She denies any illicit drug use.  She is currently not taking any medications.  All her immunizations are up-to-date.  Her blood pressure is 130/80 mmHg and heart rate is 90/min.  Physical examination reveals fine crackles over the right lower lung lobe.  No lower extremity edema is present.  Neck palpation does not reveal any lymph node enlargement.  The chest x-ray shows right lower lobe infiltrates and left upper lobe fibrosis.  The ECG reveals non-specific ST segment and T wave changes.  What is the best next step in the management of this patient?

Quizplus · Accepted Answer

Explanation: This patient has a history of recurrent bacterial infections that is highly suggestive of immunodeficiency with involvement of humoral immunity. Selective immunoglobulin deficiency that includes IgA and/or IgG subfractions is a possible diagnosis. For example, selective deficiency of IgG3 alone is more common in adult females and is associated with recurrent sinopulmonary as well as gastrointestinal infections. Food allergies and autoimmune diseases may also be present. IgA deficiency and common variable immunodeficiency are also possible. The latter may be associated with suppressed cell immunity and increased risk of malignancy. Quantitative measurement of serum immunoglobulin levels helps to establish the diagnosis. (Choice D)Cystic fibrosis is also a possibility, but it usually manifests earlier in life and does not cause gastroenteritis. (Choice A)Alpha-1-antitrypsin deficiency is an inherited disorder that leads to emphysema and liver damage, but does not increase the risk of recurrent infections. (Choices B and E)Methacholine challenge is used to diagnose asthma, and V/Q testing is employed in patients suspected to have pulmonary thromboembolism; both these diagnoses are unlikely in this case. Educational Objective: Recurrent bacterial infections in an adult patient may indicate a humoral immunity defect. Quantitative measurement of serum immunoglobulin levels helps to establish the diagnosis.

A 32-Year-Old Caucasian Female Presents to Your Office with Persistent