Anca Negative Eosinophilic Granulomatosis With Polyangiitis: 4 Cases Report And Literature Review

Background and Aims: To improve the understanding of antineutrophil cytoplasmic antibody (ANCA) negative eosinophilic granulomatosis with polyangiitis (EGPA) which primary manifestations were respiratory involved. Methods: The clinical data of 4 cases of ANCA negative EGPA from February 2017 to April 2017 in our hospital was analyzed, including clinical features, procedure of diagnosis and treatment options. Relevant literatures of EGPA were reviewed. Results: 3 cases were diagnosed as EGPA and 1 was EGPA combined with allergic bronchopulmonary aspergillosis (ABPA). The age of onset was 28 to 59 years, with the average age of 44 years, and the course of disease was ranged from 1 month to 3 years. ANCA was negative of all those 4 patients. Airway hyperresponsiveness (AHR) of these patients varied from cough variant asthma (CVA) to refractory asthma. Patients were confirmed with chronic sinusitis by computer tomography (CT) scan, whereas clinical symptoms were nearly absent. Gastrointestinal, skin and neurological system were involved as extra-pulmonary manifestations. Eosinophilia of peripheral blood and induced sputum, pulmonary infiltration and peripheral neuropathy were found in 3 patients. 3 cases with elevated serum total IgE before using systemic glucosteroid. Eosinophilic infiltration in extravascular and intravascular space was verified by tissue biopsy in 3 cases with chronic inflammation in 1 case. Conclusion: EGPA patients who first suffered from respiratory symptoms are tend to have the negative result of ANCA. The degree of AHR is associated with the course of the disease. Patients with asthma complicated with eosinophilia and sinusitis should be aware of ruling out of EGPA.