A 35-year-old Caucasian female with a past medical history of hepatitis C virus, thalassemia minor and ankylosing spondylitis presented to the hospital with worsening dyspnea on exertion accompanied by occasional wheezing for the past month. Her symptoms included substernal pleuritic chest pain, heartburn, recurrent epigastric pain, night sweats, and subjective fevers. She also reported weight loss of 30 pounds over the past 4 months. Review of systems was positive for fatigue, headaches, back pain, recurrent sinusitis, and lower extremity weakness.
Complete blood count (CBC) showed hemoglobin 10.5 g/dL with mean corpuscular volume (MCV) 63, white blood cell (WBC) count 24.4 K/mm3, 75% eosinophils, absolute eosinophil count 18.3 K/mm3, platelets 256 K/mm3. Basic metabolic panel showed sodium 135 mmol/L (136-145 mmol/L), potassium 4.2 mmol/L (3.5-5.1 mmol/L), chloride 99 mmol/L (98-107 mmol/L), bicarbonate 27 mmol/L (21-31 mmol/L), blood urea nitrogen (BUN) 5 mg/dL (7-25 mg/dL), creatinine 0.5 mg/dL (0.6-1.3 mg/dL), glucose 87 mg/dL (70-110 mg/dL). Liver function tests were within normal limits. Urinalysis was unremarkable. Procalcitonin was normal at 0.07 ng/mL (<0.49 ng/mL). Erythrocyte sedimentation rate was elevated at 34 mm/hr (0-20 mm/hr). Serum antinuclear antibody (ANA), antineutrophil cytoplasmic antibodies (ANCA) panel, serum fungal and parasite serologies were all negative as well. Computed tomography (CT) with angiography of the chest revealed ground-glass tree-in-bud opacities within the upper lobes bilaterally, concerning for respiratory bronchiolitis, interstitial lung disease, or atypical pneumonia. Bone marrow biopsy confirmed hypercellular marrow with trilineage hyperplasia and associated moderate eosinophilia.
Pulmonary function testing (PFT) revealed FEV1 1.97 L (63% of predicted), FVC 3.77 L (74% of predicted), FEV1/FVC 85% of predicted; methacholine challenge test was positive. A follow-up high-resolution CT chest showed subtle centrilobular ground-glass nodules along the bronchovascular structures most pronounced within the bilateral mid and upper lungs (Fig. 1). Bronchoscopy with bronchoalveolar lavage showed marked eosinophilia (47%) on cytology, however, bacterial and fungal cultures were negative. Transthoracic lung biopsy was pursued and subsequent histopathological analysis showed prominent eosinophilic infiltrate, granulomatous reaction around small arterioles with occasional multinucleated giant cells and eosinophilic microabscesses, consistent with granulomatosis with polyangiitis and eosinophilia (Fig. 2A and B). She was started on prednisone 60 mg daily for 8 weeks and was found to have improvement in her symptoms.