Case Report

A case of Eosinophilic granulomatosis with polyangiitis without Asthma

Harpreet Singh1, Parijat Mathur2, Veera Singh2, Vivek Kak2
1Sutter Gould Medical Foundation, Stockton, California.
2Henry ford allegiance health hospital, Jackson, Michigan

Worldwide Medicine 2019; 1(7): 243-251 | DOI: 10.5455/ww.65674      PDF


Eosinophilic granulomatosis with polyangiitis (EGPA) previously known as Churg Strauss syndrome is a rare necrotizing vasculitis of small and medium sized vessels which classically presents with asthma. As we are trying to understand the pathophysiology of the disease more, cases have been reported without asthma which meet the American rheumatology society requirements for the diagnosis. This case is one of the rarer ones in which the patient presented with involvement of the gastrointestinal tract without asthma.
A 68 year old man with lung cancer presented with petechial rash over extremities, and a marked peripheral eosinophilia after initiation of chemoradiation. It was believed that he had “tumor-associated peripheral eosinophilia” and he was started on a short course of steroids resulting in resolution of his symptoms. He returned to clinic 5 months later, with complaints of left lower quadrant abdominal pain and Computerized tomographic (CT) imaging were consistent with acute appendicitis. Appendectomy was performed and pathology demonstrated tissue eosinophilia with vasculitis. Furthermore, cytoplasmic antineutrophil cytoplasmic antibodies (C- ANCA) were positive at a titer of 1:320. This was consistent with EGPA. We concluded that EGPA should be suspected in patients with marked peripheral eosinophilia even in the absence of typical pulmonary symptoms.

Keywords: churg strauss, eosinophilic granulomatosis, polyangiitis, vasculitis