Case Presentation: Case PresentationA 49 years old Moroccan female with past medical history of chronic sinusitis and rheumatoid arthritis on Humira presented with the chief complaints of cough and low-grade fever for 1 week. She also had progressive worsening of cough which was productive with dark yellowish/greenish colored phlegm. It was associated with low-grade fever. Initially the patient attributed these episodes to the worsening of sinusitis, but lately patient also started to have shortness of breath.Initially the patient was hypoxic with tachypnea and tachycardia. Blood pressure was holding steady. On exam, the patient was in moderate respiratory distress with normal chest expansion, but reduced breath sounds in the lower lung fields. Neither adventitious sound nor neuropathy was noted. CTA chest showed dense bilateral pneumonia with bilateral pleural effusions. The Patient was initially admitted to the medical floor for the treatment of pneumonia with IV Zosyn and IV azithromycin. However, the patient continued to have hypoxic respiratory failure and minimal relief in cough despite broad spectrum antibiotics. On day 3 of hospitalization, patient had to be transitioned to BiPAP. . Patient’s clinical status continued to deteriorate as she was requiring FiO2 of 50% and PaO2/FiO2< 200 concerning for ARDS. The patient was empirically started on IV methylprednisolone. Bronchoscopy was performed which showed no evidence of intraluminal growth, mass or stenosis, but bronchoalveolar lavage studies revealed a high eosinophil count of 64%. It raised the suspicions for Fungal/Mycobacterial infections, ABPA, EGPA. AFB and fungal staining were negative. ANCA levels and total IgE levels were sent. On day 5 of admission patient's PaO2/FiO2 ratio improved. A repeat chest x-ray showed partial clearing of infiltrates. The patient was extubated on day 6 and the patient was discharged on tapering doses of steroids over a period of 6 months. The patient was followed outpatient with pulmonary medicine and patient's ANCA levels came out to be positive with elevated total IgE levels and lung biopsy revealed eosinophilic infiltration with areas of necrosis-confirming the diagnosis of EGPA.Discussion

Discussion: Eosinophilic granulomatosis with polyangiitis is a pleiotropic systemic vasculitis which can present with multisystem involvement. It can mimic allergic and asthmatic symptoms. A clinician should have a high level of suspicion, especially if the patient presents with persistent sinusitis or poorly controlled asthma with high level of eosinophil count. Treatment can be as simple as early initiation of steroids and other immunosuppressants which usually has favorable outcome and expectancy.

Conclusions: Eosinophilic granulomatosis with polyangiitis formerly called as Churg Strauss syndrome is an extremely rare necrotizing vasculitis condition of small and medium sized blood vessels. The first or allergic stage marked by airway inflammation in patients experiencing asthma or allergic rhinitis. The second stage is characterized by peripheral hyper eosinophilia causing tissue damage most commonly in the lungs and digestive tract. The last stage encompasses a vasculitic phase eventually leading to sudden death and can be life-threatening. Not all patients develop all the 3 stages or progress from one stage to the next in the same order.