A 50-year-old Caucasian female with a history of difficult-to-control asthma since 1994 and chronic rhinitis presented to the hospital with severe jaw and ear pain in late February of 2009. She had been suffering from intermittent pain for a few months and underwent bilateral myringotomy tube placement about a month prior for recurrent otitis media with some benefit. The pain was distributed over the rami of the mandible bilaterally with radiation to her ears. She denied any fever, night sweats, weight loss, purulent nasal discharge, odynophagia, dysphagia, or shortness of breath. No significant history of travel or sick contact including contact to TB patients. The patient was a past smoker with 15 pack year history of smoking.
Vital signs on admission showed a BP of 101/63 mm Hg, pulse of 105 beats/minute, temperature of 97.9 F, respiratory rate of 18 breaths/min, and SPO2 of 96% on room air. Physical examination revealed a patient in moderate distress. Bilateral tenderness was elicited while palpating the mandibular rami. The myringotomy tubes were intact without and significant drainage. The nasal mucosa appeared normal without any evidence of erythema, epistaxis, or discharge. The rest of the physical examination was unremarkable. The laboratory data are shown in Table 1.
Urinalysis showed trace proteinuria and no RBC cast. Electrocardiogram and a chest X-ray were normal. She underwent a CT scan of her neck with contrast which was unremarkable except left maxillary sinus thickening. However, the apical part of the lungs showed multiple nodules bilaterally. A dedicated high-resolution CT scan of the chest revealed multiple bilateral nodules, 5-11 mm in diameter. The largest nodule was noted in the lingula that measured 11 x 9 mm. There was also evidence of pericarditis and small pericardial effusion. Given her long standing history of uncontrolled asthma, upper airway symptoms, eosinophilia, and multiple pulmonary nodules, a clinical diagnosis of EGPA was made and the patient underwent an extensive rheumatologic workup which is shown in Table 2.
The patient underwent an open lung biopsy of the lingular nodule as well as a pericardial biopsy. The histopathology was consistent with necrotizing granulomatous vasculitis with extravascular eosinophilic infiltrate. The histopathology slides are shown in Figures 1 and 2. The diagnosis of EGPA was confirmed.
The patient was started on high-dose prednisone and azathioprine in March 2009. She initially did well, but in August 2009 the patient was admitted to the hospital with pulmonary edema secondary to heart failure with reduced ejection fraction (HFrEF). The echocardiogram showed an EF of 25-30% with biventricular dilatation and global hypokinesis. No wall motion abnormality was identified. Cardiac catheterization was negative for coronary artery disease. No endomyocardial biopsy was performed. Given the high incidence of cardiac involvement in EGPA and a negative coronary angiogram, the myocardial dysfunction was thought to be secondary to EGPA and she was started on IV cyclophosphamide. Over the course of next 6 months, the patient completed 6 cycles of IV cyclophosphamide and started on mycophenolate. Steroid taper was continued. Her EF recovered completely, but she suffered from multiple vertebral body compression fractures and developed cushingoid appearance secondary to chronic steroid therapy. Between September 2009 and July 2011, she suffered from 2 episodes of sinusitis which was treated with short course of antibiotic. Her disease remained well controlled with normal inflammatory markers.
During an office visit in July 2011, the patient was noted to have depressed nasal ridge (Figure 3). Physical examination showed nasal mucosal erythema. The patient underwent reconstruction of her nasal bridge in July 2013. Since 2011, the patient has been managed with mycophenolate without any incidence of exacerbation and is doing well currently.