Abstract | April 6, 2022
An Autoimmune Concoction: The Difficult Diagnosis of a Cavitary Lung Lesion
- Recognize the differential diagnosis of cavitary lung lesions;
- Management of Granulomatosis with Polyangiitis.
Introduction: Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis that affects the smallsized arterial vessels of the ear, nose, throat, airway with common lung, renal, and ophthalmic involvement. Hypereosinophilia is commonly seen in eosinophilic granulomatosis with polyangiitis and a rare finding in GPA. This patient had GPA with eosinophilia complicated by Aspergillosis.
Case Presentation: A 19-year-old female presented with cough and dyspnea for 2-3 days. Medical history included recent lacrimal gland surgery, tympanostomy, and a current 2-week treatment for right middle ear otitis media complicated by otorrhea, hemorrhage and ulceration. The patient had ear pain, diarrhea, fever, and fatigue. Exam was significant for heart rate of 114 bpm, and oxygen saturation of 95% on room air; tachycardia, diminished breath sounds, and injected conjunctivae were also noted. Labs revealed decreased hemoglobin of 9.2 gm/dL, elevated WBC of 14,200/mcl, and elevated platelets of 536,000/mcl. Eosinophil count was 1,400/mcl (10.5%); bandemia (7%) and giant platelets were noted. Rheumatoid factor was 52 iu/mL. Streptococcus pneumoniae urine antigen and human rhinovirus/enterovirus on respiratory viral panel were positive. Urinalysis (UA) showed 25-50 RBC per HPF and 5-10 WBC per HPF. Initial chest x-ray revealed a right parahilar infiltrate. CT showed pneumonia in the right upper lobe with central cavitation and associated multiple patchy areas of interstitial and ground-glass opacities in a peripheral distribution. Ceftriaxone and azithromycin were started. Day 3, the patient developed hemoptysis and epistaxis. Serology revealed antihistone antibodies of 1.0 units, IgE level of 351 KU/L, PR3-ANCA level of 287.5 AI, and a normal MPO-ANCA level <1.0 AI.
Final Working Diagnosis: Granulomatosis with Polyangiitis
Management/Outcome: The patient was started on steroids. Day 4, a repeat UA showed 155 RBC per HPF and 11 WBC per HFP. Day 7, the patient’s hemoptysis resulted in intubation and transfer to ICU. Emergent bedside bronchoscopy was performed. Plasmapheresis was initiated with plans to begin rituximab. However, due to the patient’s rapid decline, she was transferred to receive extracorporeal membrane oxygenation (ECMO). BAL revealed a positive Aspergillus antigen. Since transfer, the patient rapidly improved and was able to be extubated, removed from ECMO and finished her treatment for GPA.
References and Resources:
- King, Jr, MD, Talmadge E. Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on October 5, 2021).
- Falk MD, Ronald J, Merkel MD, MPH, Peter A, King, Jr, MD, Talmadge E. Granulomatosis with Polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on October 5, 2021).