2025 – PAGE 432 – PULMONOLOGY

HYPER-IGE SYNDROME (AKA HYPER IGE SYNDROME)

In hyper-IgE syndrome (AKA hyper IGE syndrome), there is poor neutrophil chemotaxis, which results in recurrent Staphylococcus aureus abscesses and pneumonias. Pneumonias may be associated with pneumatoceles or lung abscesses. Look for a pneumatocele on a chest X-ray. There may also be a history of delayed shedding of primary dentition.

COMMON VARIABLE IMMUNE DEFICIENCY (CVID)

In cases of common variable immune deficiency (CVID), B cells do not change into plasma cells, so there is a deficiency in all of the immunoglobulin subtypes. Look for recurrent upper and lower respiratory tract infections, as well as a history of recurrent viral infections (HSV and VZV infections).

(DOUBLE TAKE) ASPERGILLUS

In immunocompetent patients, Aspergillus may cause a hypersensitivity reaction called Allergic Bronchopulmonary Aspergillosis (ABPA). This is especially true in asthmatics and patients with cystic fibro­sis. Asthmatics seem to have an exacerbation that WORSENS despite “appropriate” treatment with steroids. Look for increased eosinophilia and lung infiltrates. Treat with itraconazole and steroids. In an immuno­compromised patient, Aspergillus can cause INVASIVE pulmonary disease resulting in nodular infiltrates on imaging. Aspergillus can also be an etiology of Chronic Eosinophilic Pneumonia (peripheral infiltrates on X-ray). Treat invasive Aspergillus with amphotericin. Both invasive and noninvasive infections may require long-term steroids. All Aspergillus patients should be in a negative pressure room (as with MTB).

PEARL: Aspergillus is fairly ubiquitous in the world and may be found on cultures as a contaminate. Only choose amphotericin if there is laboratory data supporting Aspergillus infection and it seems that the Asper­gillus is the likely cause of the patient’s symptoms.

CRYPTOGENIC ORGANIZING PNEUMONIA (Formerly known as BRONCHIOLITIS OBLITERANS WITH ORGANIZING PNEUMONIA, or BOOP)

Cryptogenic organizing pneumonia (COP) is associated with recurrent pneumonias that improve a little with antibiotics. Bronchoscopy will show grossly purulent material in the airways, but when the material is cultured it is negative for any growth! An open lung biopsy shows thickened alveolar septa and cell hyperplasia. This usually requires steroids but tends to eventually resolve for most patients. Etiology is unknown, but it’s often found in patients with chronic inflammatory diseases.

INTRAPULMONARY SEQUESTRATION

Intrapulmonary sequestration is a congenital pulmonary malformation that usually presents in teenagers with recurrent pneumonias on the same side. Surgical lobectomy is curative.

MIGRATING PNEUMONIAS

(DOUBLE TAKE) TOXOCARA CANIS

Toxocara canis is a roundworm that causes VISCERAL LARVA MIGRANS. Look for a child presenting with multisystem complaints. Usually affects the LUNGS and the GI TRACT, but can also affect the eyes. May present as abdominal pain in a child who has hepatosplenomegaly and wheezing on exam. Labs will show a HIGH LEUKOCYTOSIS with EOSINOPHILIA. Imaging will show lung infiltrates. The roundworm is found in cats, dogs, and dirt, so look for a kid that likes to eat dirt! It is often self-limited, but can be treated with albendazole.

• PEARL: The words VISCERAL and MIGRANS in an answer choice should help you remember that this is a disease of the deep organs (viscera), and it migrates to multiple organs. Lung infiltrates may be noted to change or “migrate” over time as noted on serial chest X-rays.