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Albany Medical Review - January 2002

Case Report-
A 34-year-old woman with worsening of poorly controlled asthma


Mark A. Rasmus MD, Albany Medical Center, Albany, NY

G. Scott Worthen MD, National Jewish Medical and Research Center, Denver, CO

 

PRESENTING COMPLAINT

The patient is a 34-year-old woman who presented for an evaluation of her life-long asthma. Her asthma was poorly controlled since childhood; her symptoms seemed to worsen over the past three years. Over that period she has wheezed daily, had numerous emergency department visits, required monthly visits to her physician and required daily prednisone. Her symptoms were minimal at night and were rarely exacerbated by exercise. The patient was particularly concerned about longer recovery periods between attacks. These attacks required hospitalization four times in the five months preceding referral. She was diagnosed with radiologically confirmed pneumonia three times in the year prior to referral. The patient was evaluated at National Jewish Medical and Research Center to confirm her diagnosis of asthma and to evaluate for atopy and other allergic triggers.

Past medical history is significant for eczema. The patient reported allergies since childhood, with anaphylactic reactions to specific foods including nuts, fish and raw eggs. There is no history of aspirin sensitivity or nasal polyposis. She also has a history of gastroesophageal reflux disease diagnosed by endoscopy. She suffers from osteopenia and cataracts related to her chronic corticosteroid use.

Her surgical history is remarkable for sinus surgery to correct her deviated septum as well as debulking of concha bullosa.

Medications on admission included prednisone, fluticasone MDI, salmeterol diskus, albuterol MDI/ nebs, montelukast, theophylline, alendronate, calcium, lansoprazole and azelastine nasal spray.

Family history is significant for a sister with asthma, one child with atopic dermatitis and both parents have allergic rhinitis.

The patient is a lawyer, married and living in a suburb of Phoenix with two children. She is a life-long nonsmoker, and rarely drinks alcohol which does not exacerbate her symptoms. She has no pets and she follows many hypoallergenic precautions in her home.

PHYSICAL FINDINGS

Physical exam revealed a blood pressure of 122/68, pulse of 82, respiratory rate of 14 and oxygen saturation of 97% on room air.

Patient was a pleasant white woman with a cushingoid facies. Her HEENT exam was significant for pale nasal mucosa without polyps and no conjunctival erythema was noted. Oropharyngeal exam was unremarkable without palpable lymphadenopathy. Cardiac exam was normal. Her chest was symmetric and normal to percussion as well as palpation. On ascultation her lungs had expiratory wheezes that were most prominent over the sternal notch. The wheeze was loudest with forced expiration. No crackles were noted and her inspiratory to expiratory ratio was within normal limits. Her skin revealed numerous ecchymoses predominantly on her lower extremities and striae on her abdomen. Abdominal, neurologic and extremities exam were normal.

TEST DATA

Hemogram and biochemical panels were normal. Immunoglobulins were normal, and an A.M. cortisol level of 4.7 mcg/dL was consistent with her corticosteroid use. Steroid kinetics revealed normal absorption, conversion and clearance of prednisone. Lymphocyte proliferation assay revealed a suboptimal response to hydrocortisone with normal lymphocyte suppression to dexamethasone, budesonide and fluticasone. Skin testing revealed marked atopy to numerous grasses, trees, weeds and household allergens, and an immediate response to aspergillus fumigatus.

Pulmonary function tests an isolated increased residual volume at 150% predicted. Flows were within normal limits and did not change following bronchodilator therapy. Diffusing capacity was above normal. Methacholine challenge revealed no significant change in FEV1 or specific airways conductance. Fiberoptic laryngoscopy was performed immediately following the methacholine challenge, with normal vocal cord function despite audible wheezing on forced expiration.

A high-resolution CT scan was performed and this revealed mild bronchial wall thickening, with no bronchiectasis, air trapping or other deformities.

Bronchoscopy revealed friable, irritated mucosa and normal left sided airways. The right middle lobe orifice was quite small, and the superior segment of the right lower lobe was significantly deformed and was only able to be entered with a deep inspiratory maneuver. Bronchoalveolar lavage of the right middle lobe revealed normal cell count and differential; however electron microscopy revealed an increased number of Langerhan’s type macrophages with pleomorphic structures noted to be attached to them. These are ultrastructurally consistent with Mycoplasma pneumoniae.

 

BRONCHOMALACIA PRESENTING IN ADULTHOOD

Discussion

Bronchomalacia is defined as narrowing of the airway lumen by greater than 50 percent. Severe airway collapse can lead to airflow limitation, chronic infection and bronchiectasis. Frequently, the airflow limitation is demonstrated by an expiratory notch during spirometry.

Bronchomalacia can be categorized as either congenital or acquired and can be either diffuse or localized. The congenital form usually presents as an isolated finding in infancy, and most patients are asymptomatic by two years of age. The acquired form usually affects adults and is most commonly associated with relapsing polychondritis, tracheostomies, endotracheal tubes, syphilis, crushing chest trauma, and anastamotic sites following lung transplantation. It is suggested that the acquired form is a result of a congenital weakness of the fibrous tissue combined with chronic inflammatory irritation. It is certainly possible that the abundant Langerhans cells on BAL signify chronic inflammation.

Our patient exhibited near total collapse of her affected airways during bronchoscopy, as demonstrated in figures 1-4; however, some of the classic spirometric findings were absent. The near normal spirometry in our patient is most likely due to the distal, localized nature of her disease.

Treatment strategies include surgical stent placement for localized proximal lesions, and other techniques aimed to prevent airway collapse. Pursed lip breathing, continuous positive airway pressure (CPAP), and expiration through a resistor apparatus have all been used.

A combination of flutter valve use, pursed lip breathing during exercise and nasal CPAP has allowed our patient to be weaned from her corticosteroids and asthma medications. She continues to remain free from hospitalizations and episodes of pneumonia during her six months following her diagnosis.

Conclusion

Bronchomalacia can simulate asthma. Suggestive features include persistence of wheezing despite aggressive bronchodilator therapy, poor response to glucocorticoids, and negative methacholine challenge.

Diagnosis can be made with either bronchoscopy or dynamic (inspiratory/expiratory) spiral CT scan. Conservative treatment designed to improve airway secretion clearance and stent collapsable airways can help limit the complications of this disease, mainly chronic infection and bronchiectasis or long-term corticosteroid use due to a misdiagnosis as asthma.

Figure 1 - Right middle lobe orifice during inspiration.

 

Figure 2 - Right middle lobe orifice during forced expiration

 

Figure 3 - Narrowed orifice of superior segment of right lower lobe

 

Figure 4 - Electron microscopy of BAL; Langerhans type macrophages
(click on image for larger and closer view)

 

References

Jokinen K, Palva T, Sutinen S, Nuutinen J. Acquired tracheobronchomalacia. Annals of Clinical Research 1977; 9:52-57.

Lavins BJ, Hamilos DL. Distal airway bronchomalacia resulting in severe obstructive airway physiology. Chest 1990; 97(2):489-91.

Cohn J. Localized bronchomalacia presenting as worsening asthma. Annals of Allergy 1985; 54(2):222-23.

Arakawa H, Webb RW. Imaging of Obstructive Pulmonary Disease. Radiologic Clinics of North America 1998; 36(1): 189-209.

Stelio LM, Sterman DH. Interventional Pulmonology. New England Journal of Medicine 2001; 344(10): 740-749.

Ferguson G, Benoist J. Nasal continuous positive airway pressure in the treatment of tracheobronchomalacia. American Review of Respiratory Disease 1993;147:457-61.

 

 

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30.01.2002


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