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Year : 2022  |  Volume : 65  |  Issue : 3  |  Page : 737-738
Eosinophilic granulomatosis with polyangiitis with gastrointestinal symptoms as the first manifestation

Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, National Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Beijing Key Laboratory for Precancerous Lesion of Digestive Disease, Beijing, PR China

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Date of Submission18-Jun-2021
Date of Decision25-Aug-2021
Date of Acceptance20-Nov-2021
Date of Web Publication21-Jul-2022

How to cite this article:
Cui ZL, Lv FJ, Li P, Wu J. Eosinophilic granulomatosis with polyangiitis with gastrointestinal symptoms as the first manifestation. Indian J Pathol Microbiol 2022;65:737-8

How to cite this URL:
Cui ZL, Lv FJ, Li P, Wu J. Eosinophilic granulomatosis with polyangiitis with gastrointestinal symptoms as the first manifestation. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Aug 15];65:737-8. Available from: https://www.ijpmonline.org/text.asp?2022/65/3/737/351608

Dear Editor,

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune disease involving multiple systems with a variety of clinical manifestations. It is characterized by ANCA-mediated necrotizing vasculitis, eosinophilic infiltration of tissues, and granuloma formation outside blood vessels.[1] Respiratory symptom often appears first, with the progress of the disease, multiple systems become involved. Here we present a case of EGPA with digestive symptoms as the first manifestation.

A 60-year-old man presented with intermittent upper abdominal pain for 4 months without obvious predisposing causes. He had been misdiagnosed with acute gastritis, pancreatitis, and angina. Nevertheless, no significant improvement was observed after treatment. When he was admitted to our hospital, a physical examination showed tenderness in the upper abdomen without other positive signs. Blood analysis revealed an increased EOS (greater than 10% three times in succession) and a positive peripheral-ANCA (P-ANCA). Skull computed tomography (CT) suggested accessory sinusitis. Chest CT scanning showed nodular infiltration of both lungs. Gastroscopy revealed rough-surfaced mucosa with a large number of eosinophil infiltration [Figure 1]a and [Figure 1]b. On an average, though the EOS were about 10/HPF, at areas the same was up to 20/HPF. The carbon 13 urea breath test was negative, and the patient also denied a history of Helicobacter pylori (Hp) infection. No abnormality was detected on colonoscopy. The patient had allergic rhinitis for 3 years without medication. In addition, the patient had a history of hypertension for many years and had taken regular oral antihypertensive drugs for a long time. EGPA was considered as the final diagnosis based on history, examination results, and multidisciplinary consultation, and thereafter oral methylprednisolone (50 mg/day) was started. The patient achieved remission rapidly without relapse. Gastroscopy after 1-year follow-up showed chronic atrophic gastritis without eosinophilic infiltration [Figure 1]c and [Figure 1]d.
Figure 1: Before the treatment, (a) Gastroscopy revealed rough-surfaced mucosa. (b) Endoscopic biopsy showed chronic atrophic inflammation, scattering EOS over the stromal invasion. There is about 10/HPF on average, one of the fields can see 20 EOS. The eosinophils are indicated with the red arrows. (hematoxylin and eosin, original magnification ×400). After the treatment, (c) Gastroscopy showed chronic atrophic gastritis. (d) Pathology findings confirmed that no eosinophilic infiltration was observed in the gastric mucosa. The red box shows the pathological changes of atrophic gastritis. (hematoxylin and eosin, original magnification ×400)

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The diagnostic criteria of EGPA were developed by the American college of rheumatology, the content specifically as follows: (1) Asthma; (2) Eosinophilia >10%; (3) Neuropathy, mononeuropathy, or polyneuropathy; (4) Pulmonary infiltrates; (5) Paranasal sinus abnormality; (6) Extravascular eosinophil infiltration on biopsy findings. A diagnosis can be made if four or more criteria are met.[2] The diagnostic criteria clearly stated that eosinophils should be greater than 10% in peripheral blood, but did not define the cutoff of the eosinophilic cell infiltrate in the tissue. The number of mucosal EOS infiltrate in EGPA though varies between 10 and 30/HPF in the literature, in the index case EOS were identified up to 10–20/HPF, consistent with diagnostic criteria.

Gastrointestinal involvement has been reported in about 20%–30% of EGPA patients.[1] We also considered the possibility of pancreatitis. Pancreatitis is uncommon in EGPA, and only one case of pancreatic eosinophilia and death due to acute pancreatitis was reported in a 5-year-old child.[3] We completed laboratory and abdominal imaging tests that did not support pancreatitis, so no pancreatic biopsy was performed. An endoscopic biopsy can help us easily identify infiltration of EOS in EGPA. However, Histological evidence of vasculitis often is difficult to observe. Therefore, we need additional evidence to identify eosinophilic infiltrates from other diseases. Parasitic infection is a common cause, and sometimes ova can be seen in pathological sections. Eosinophils may be seen in gastrointestinal tissue before and after treatment for Hp infection. Carbon 13 urea breath test and Hp antibody test can confirm the diagnosis. In addition, inflammatory bowel disease can also lead to eosinophilic infiltration.[4]

A comprehensive multisystem examination of such patients is needed and if suspected multipoint or multiorgan biopsies are recommended as in single biopsy the histological changes may be missed. It is worth noting that the pathological characteristics are significant differences at different stages of the disease.[5]

Glucocorticoids are usually used as a basic therapy for EGPA. In the index case features of atrophic gastritis were identified after one year of glucocorticoid therapy. Atrophic gastritis has not been reported in EGPA, and there is no evidence of the association between the two diseases. The pathogenesis of atrophic gastritis is complex, and the influencing factors include pathogen infection, immune level, dietary factors, drug damage, and so on. We suspect that atrophic gastritis is related to the use of glucocorticoids, and to determine whether hormones are directly related to atrophic gastritis, a longer period of follow-up is needed.

In this article, we review the problems in the process of diagnosis and treatment of EGPA, it will provide some help for clinicians to identify this complex and rare disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Supported by the Digestive Medical Coordinated Development Center of Beijing Hospitals Authority, No. XXZ015; Special Scientific Research Fund for Tutor, No. YYDSZX201901; the Science and Technology Development Project of China State Railway Group, No. N2019Z004.

Conflicts of interest

There are no conflicts of interest.

   References Top

Furuta S, Iwamoto T, Nakajima H. Update on eosinophilic granulomatosis with polyangiitis. Allergol Int 2019;68:430-6.  Back to cited text no. 1
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American college of rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-100.  Back to cited text no. 2
Yagi H, Takahashi S, Kibe T, Shirai K, Kosugi I, Kawasaki H, et al. An autopsy case of a 5-year-old child with acute pancreatitis caused by eosinophilic granulomatosis with polyangiitis-like necrotizing vasculitis. Case Rep Rheumatol 2019;2019:9053747. doi: 10.1155/2019/9053747.  Back to cited text no. 3
Walker MM, Potter M, Talley NJ. Eosinophilic gastroenteritis and other eosinophilic gut diseases distal to the oesophagus. Lancet Gastroenterol Hepatol 2018;3:271-80.  Back to cited text no. 4
Gioffredi A, Maritati F, Oliva E, Buzio C. Eosinophilic granulomatosis with polyangiitis: An overview. Front Immunol 2014;5:549.  Back to cited text no. 5

Correspondence Address:
Jing Wu
Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, National Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Beijing Key Laboratory for Precancerous Lesion of Digestive Disease, Beijing - 100 050, PR
PR China
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_625_21

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