Colitis as the Initial Presentation of Eosinophilic Granulomatosis with Polyangiitis

Abstract

A male patient in his early sixties with recurrent diarrhea was transferred to our hospital. The patient did not have any pulmonary or upper respiratory symptoms. He was noted to have peripheral eosinophilia. Further workup revealed a negative antineutrophilic cytoplasmic antibody titer but a positive myeloperoxidase antibody and positive proteinase 3 antibodies. A colon biopsy also revealed eosinophilic-rich granulomas in the mucosa, confirming a diagnosis of eosinophilic granulomatosis with polyangiitis. On cardiac imaging, eosinophilic myocarditis was also discovered. To treat active severe EGPA, the patient received high-dose corticosteroids and intravenous cyclophosphamide. The occurrence of gastrointestinal involvement as an initial manifestation of eosinophilic granulomatosis with polyangiitis is infrequent, emphasizing the significance of its recognition. This case underscores the importance of identifying and diagnosing such atypical presentations to facilitate timely and appropriate management.

Figure 1

Eosinophilic granulomatosis with polyangiitis. (A) Area of patchy erythema and decreased mucosal vascularity in the rectum seen on colonoscopy. (B) Colon mucosal biopsies: low power magnification showed eosinophilic necrotizing granulomatous vasculitis involving the submucosal vessel (×40). (C, D) High-power magnification showed a dense eosinophilic infiltrate destroying the vessel wall, a classic presentation of granulomatous vasculitis (×400).

Figure 2

Suspected acute eosinophilic myocarditis. (A, B, C, F) Multiplanar late gadolinium enhancement (LGE) imaging is demonstrated in the 4-chamber (A), short-axis (B, C), and 2-chamber long-axis (F) planes. Unlike most contrast-enhanced sequences routinely utilized in both CT and MRI, these images are obtained in 5–15 minutes following intravenous administration of the contrast agent (gadolinium, allowing for washout of contrast from not only the intravascular space but also from most soft tissues (including normal/viable myocardium)). These cardiac-specific sequences further utilize an “inversion” radio frequency pulse before image acquisition to further “null” (decrease/darken) the signal from the myocardium. The resultant image then depicts normal myocardium as a nulled low signal (black) and acute myocardial necrosis or scar regions as a relatively high signal (gray/white). (A) In a four-chamber LGE image, the thick black arrow points to a viable, appropriately “nulled” septal myocardium. In contrast, the adjacent thin black arrow points to one of the several focal regions of epicardial hyperenhancement involving the septum. (B) Correlative short-axis findings of patchy, predominantly epicardial enhancement resulting in an irregular appearance of the septal epicardium (black arrows) and adjacent confluent hyperenhancement of the more severely involved inferior epicardium (curved white arrow). (C) A short-axis LGE view reveals additional near transmural involvement of the basal inferior wall. A color “map” plotting specific T2 and T1 values at each voxel is depicted in (D, E), respectively. Increased signal values (appearing as shades of purple in (D) and orange/yellow in (E)) suggest disease involvement/myocardial edema. The yellow arrow in (D), a two-chamber long-axis T2 map, points to the focal near transmural edema of the LV septum. (D) In a short-axis T1 map, multiple regions of abnormal predominantly epicardial signal correlate with/confirm the suspected multifocal myocardial necrosis and scar (hyperenhancement) depicted in LGE images (A, B, C, F).