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Uncontrolled asthma, recurrent rhinosinusitis, and infertility in a young woman

Reviewed By Allergy, Immunology & Inflammation Assembly

Submitted by

P. Scott Meehan MD

Fellow

Division of Pulmonary, Allergy, Critical Care and Sleep Medicine

The Ohio State University Medical Center

Columbus, Ohio

Jennifer W. McCallister MD

Assistant Professor of Internal Medicine

Division of Pulmonary, Allergy, Critical Care and Sleep Medicine

The Ohio State University Medical Center

Columbus, Ohio

Submit your comments to the author(s).

History

A 27 year old Caucasian woman with a history of uncontrolled asthma, allergic rhinitis, gastroesophageal reflux disease and recurrent pneumonias was referred for evaluation in the pulmonary clinic.   She was diagnosed with asthma at age twelve.  In the last six years she had been treated as an outpatient for pneumonia, sinusitis, and bronchitis several times per year and had required systemic corticosteroids several times per year for presumed asthma exacerbations.

Since last year she described persistent dyspnea with exertion, daily cough, and thick, yellow sputum production which had worsened.  This had not improved despite multiple courses of antibiotics, systemic glucocorticoids, and escalation of her inhaled corticosteroids.  She also reported wheezing and recurrent episodes of left sided chest pain.  There was no nighttime cough or limitation in daily activities.  She had not had frequent ear infections, yeast infections, epistaxis or hearing loss.

Her medications included a combination high dose inhaled corticosteroid and long acting beta agonist, montelukast, a proton pump inhibitor, albuterol, a multi-vitamin and methylphenidate.  She had been using short acting beta agonists multiple times per day without relief. 

She had a 5 pack-year smoking history and quit eight years prior.  She was married and had been trying to conceive for about 15 months without success.  She had no known occupational exposures and owned one dog.  There was no known family history of asthma or other respiratory diseases. 

Physical Exam

On physical exam, she was a well developed, obese (Body Mass Index 32 kg/m2) woman in no acute distress.  Her resting oxygen saturation (SpO2) was 97% on room air.  Head and neck exam were normal.  Heart sounds were normal without murmur, rub, or gallop with sounds heard best at right sternal border.  Auscultation of the lungs revealed inspiratory squeaks over the left upper lung field, otherwise clear.  Percussion and diaphragmatic excursion were normal.  The abdomen was soft and non-tender with no hepatosplenomegaly appreciated.  There was no peripheral edema or clubbing.

Laboratory Data

White blood cells 6.2 K/uL
Hemoglobin 12.4 g/dL
Hematocrit  39.4 %
Platelets 285 K/uL
Differential, normal  
   
Alpha 1 Anti-trypsin 117 mg/dL   (80-240 mg/dL)
Immunoglobulin G (IgG) 1001 mg/dL (700-1600 mg/dL)
Immunoglobulin A 312 mg/dL   (70-400 mg/dL)
Immunoglobulin M   116 mg/dL (40-230 mg/dL)
Immunoglobulin E    18.4 IU/mL (7-135 IU/mL)
Total hemolytic complement titer (CH50) 115 units     (51-150 units)
Pneumococcal antibody titer normal  
Tetanus IgG antibody titer normal  
Diphtheria IgG antibody titer normal  

Pulmonary Function Testing

Forced Vital Capacity (FVC)                3.54 L  (109% predicted)
Forced expiratory volume in 1 second (FEV1) 2.94 L (105% predicted)
FEV1/FVC   81.4%
Total Lung Capacity (TLC) 4.5 L    (104% predicted)
Diffusion Capacity of Carbon Monoxide (DLCO) 19 mL/min/mm Hg (82% predicted)
Flow volume loop normal  

Figures


Figure 1: Posterior-Anterior Chest Radiograph—demonstrates dextrocardia and a gastric bubble under the right hemi-diaphragm consistent with situs inversus with a left sided infiltrate and tram tracking along the left heart border. Follow-up imaging with computed tomography (CT) of the chest, abdomen, and pelvis was obtained with selected images provided below:

Figure 2: High resolution computed tomography (CT) of the Chest —demonstrates situs inversus totalis and volume loss of the "left middle lobe" with bronchiectasis, presumably fibrotic.

Figure 3: Computed tomography (CT) of the abdomen—demonstrates situs inversus totalis.

Question 1

What is the most likely diagnosis?

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