Isolated Vasculitis of the Stomach: A Novel or Rare Disease with a Difficult Differential Diagnosis

• Introduction
• Case Report
• Discussion
• Diagnosis
• Treatment
• Acknowledgment
• References

In up to 80 % of patients with vasculitis, signs of the disease are also seen in the gastrointestinal tract. However, no cases of exclusively gastric vasculitis have previously been reported. We report here the case of a 45-year-old woman with upper abdominal discomfort (no arthropathy), with gastroscopic and endoscopic ultrasound (EUS) findings that mimicked scirrhous gastric carcinoma. Gastroscopy revealed giant gastric folds and a suspicious antral ulcer (with histological findings suggesting chronic active pangastritis). EUS showed a concentric, thickened gastric wall (8 mm) with ”pseudolamellation“ and more than five enlarged lymph nodes in the paragastric region (lesser curvature). On the basis of suspected scirrhous gastric carcinoma, the patient underwent a four-fifths gastric resection of the altered parts of the gastric tissue. The postoperative course was uneventful. Histological examination of the specimen revealed severe obliterative panvasculitis of the stomach. During a 22-month follow-up period, no signs or symptoms of systemic primary or secondary vasculitis were found in the patient’s medical history, symptoms, laboratory parameters, or imaging.
This case shows for the first time that a specific gastric panvasculitis can occur, either as a preliminary stage of the condition or as a distinct manifestation of vasculitis associated with the stomach alone. Gastric resection appears to be indicated in patients with isolated obliterative gastric vasculitis, since it avoids the side effects of long-term immunosuppressive therapy and provides prognostic information that takes account of the differential diagnosis of scirrhous gastric carcinoma.

Introduction

The incidence of the rare condition of systemic vasculitis, affecting all vessels and organs, is approximately 20 per 10⁶ population per year [1]. Gastrointestinal manifestations, which occur in up to 80 % of the patients [1] [2], can become symptomatic [3]. They are more likely to develop after a considerable period of time and may then be associated with more specific changes in the skin, joints, kidneys, and lungs. No cases of exclusively gastric manifestation or of the initial appearance of vasculitis in the stomach have previously been reported. We report here a case of isolated obliterative vasculitis of the stomach, which mimicked a localized scirrhous gastric carcinoma in its symptoms as well as in the findings on endoscopy, transabdominal ultrasonography, and endoscopic ultrasonography (EUS).

Case Report

A 45-year-old woman was referred due to discomfort in the upper abdomen that had persisted for 5 weeks. Her medical history was only notable for type-1 diabetes mellitus, treated with an insulin pump. The physical examination showed that her general condition was slightly reduced; there was some tenderness in the upper abdomen, but no tumor mass. Laboratory tests only showed abnormal values for blood glucose (7.6mmol/l) and iron (4.0 μmol/l); she was positive for antibodies to the hepatitis B surface antigen (HBsAg) but negative for hepatitis B surface antigen. Upper gastrointestinal endoscopy showed giant gastric folds with a granular mucosal relief and coloring (Figure [1 a]) and a suspicious antral ulcer (Figure [1 b]); the histological assessment showed chronic active pangastritis and a fibrin-coated antral ulcer. Transabdominal ultrasonography and EUS showed a concentric thickening of the entire gastric wall to 8 mm and a disturbed layer configuration (“pseudolamellation”). In addition, there was hypoechoic thickening of the fourth layer (the muscularis propria), mixed echogenic thickening of the third (submucosal) layer with gyrus-like structures, a partly blurred margin and contour in the fifth (serosal) layer at the outer side, and more than five tumor-like lesions with grouped and enlarged lymph nodes in the paragastric region at the lesser curvature (Figure [2]).

The patient underwent an exploratory laparotomy, which showed a macroscopic appearance resembling scirrhous gastric carcinoma, with considerable thickening of the gastric wall. Because of the macroscopic findings, a four-fifths gastric resection removing the altered parts of the stomach was carried out (the fresh frozen section did not confirm carcinoma), with a side-by-side gastrojejunostomy and Braun jejunojejunostomy. The postoperative course was uneventful.

The histological examination showed dense infiltrates consisting of lymphocytes (T-cells), plasma cells, and granulocytes (with only a few eosinophiles) within the walls of the vessels and occluding fibrinoid necroses within the lumens of the arteries, veins, and capillaries in the serosal layer, muscularis propria, and submucosal layer. There were no giant cells, granulomas, or carcinoma cells. The final histological diagnosis was severe obliterative vasculitis of the stomach (Figure [3]).

After a follow-up period of 22 months, the patient did not report any symptoms, problems, or discomfort, and no further signs or symptoms were found. In addition, there was no evidence in the patient’s medical history, no suspicious laboratory parameters, and no pathological findings in the imaging procedures that would suggest systemic primary or secondary vasculitis (negative for the American College of Radiology criteria). Angiography was declined by the patient.

Discussion

This case demonstrates for the first time that obliterative gastric vasculitis can be exclusively limited to the stomach, with no precise distinction possible between whether this represents an early stage of systemic vasculitis or a separate gastric disorder. Since there was no historical, clinical, laboratory, or radiographic evidence of systemic primary or secondary vasculitis, the findings suggest a diagnosis of primary obliterative gastric vasculitis as a novel, distinct, and unique disease in the vasculitis group, which has not previously been described.

Isolated vasculitis of the stomach is very rare [4]. There has only been one case report of eosinophilic granulomatous vasculitis of the stomach, which was interpreted as an atypical Churg-Strauss syndrome [5].

This unusual case also reflects the following specific aspects and problems involved in the perioperative management.

Diagnosis

Similarities with and differences from known benign or malignant diagnoses need to be considered, such as:

• The giant gastric folds observed can occur in both benign and malignant diseases.

• Histological examination of the gastric biopsies showed both routine findings suggesting benign disease and findings in the suspicious antral ulcer that were suggestive of cancer.

• Transabdominal ultrasonography and EUS, with findings such as thickening of the gastric wall including the fourth layer, which is typical of malignant and infiltrative tumor growth in the gastric wall [6] [7] [8] [9], suggested the conclusion that there was a scirrhous gastric carcinoma or lymphoma.

• The ”pseudolamellation“ of the gastric wall and the texture of the submucosal (third) layer also supported a diagnosis of scirrhous gastric carcinoma.

• A deep biopsy plus histological examination, which have a detection rate of only 50-70 %, can be avoided during the diagnostic work-up if EUS (with a detection rate of more than 90 %) provides an indicative finding.

Treatment

As it is usually indicated in systemic vasculitis, immunosuppressive treatment could also be considered in obliterative gastric vasculitis. However, this might be fatal in case of a scirrhous gastric carcinoma (the differential diagnosis), as the two conditions have similar morphological and imaging features. The risk of inappropriate treatment based on an incorrect diagnosis can be circumvented by elective gastric resection.

Exploratory laparotomy is indicated when there are imaging findings suspicious for scirrhous gastric carcinoma and the results of the histological examination are negative for tumor. The case reported here suggests that gastric resection or even gastrectomy can be considered as a curative approach in patients with isolated obliterative gastric vasculitis (endoscopic follow-up examinations are required). This avoids the adverse effects of long-term immunosuppressive medication and provides prognostic information that takes account of the differential diagnosis of scirrhous gastric carcinoma.

Acknowledgment

The authors are grateful to Prof. D. Katenkamp of the University of Jena for providing an expert opinion on the histopathological diagnosis and specific findings in the surgical specimen.

References

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U. Will, M. D.

Dept. of Gastroenterology · Municipal Hospital · Gera · Germany

Fax: +49-365-8282402 ·

Email: uwe.will@wkg.srh.de

References

• 1 Müller-Ladner U. Vasculitides of the gastrointestinal tract.  Best Pract Res Clin Gastroenterol. 2001;  15 59-82
  CrossrefPubMedSearch in Google Scholar
• 2 Geboes K, Dalle I. Vasculitis and the gastrointestinal tract.  Acta Gastroenterol Belg. 2002;  65 204-212
  PubMedSearch in Google Scholar
• 3 Bailey M, Chapin W, Licht H, Reynolds J C. The effects of vasculitis on the gastrointestinal tract and liver.  Gastroenterol Clin North Am. 1998;  27 747-782
  PubMedSearch in Google Scholar
• 4 Burke A P, Sobin L H, Virmani R. Localized vasculitis of the gastrointestinal tract.  Am J Surg Pathol. 1995;  19 338-349
  CrossrefPubMedSearch in Google Scholar
• 5 Premaratna R, Saparamadu A, Samarasekera D N. et al . Eosinophilic granulomatous vasculitis mimicking a gastric neoplasm.  Histopathology. 1999;  35 479-481
  CrossrefPubMedSearch in Google Scholar
• 6 Will U, Zinsser E, Raabe G, Bosseckert H. Value of endosonography in diagnosis of diffusely growing stomach carcinomas; in German.  Z Gastroenterol. 1998;  36 151-157
  PubMedSearch in Google Scholar
• 7 Okanobu H, Hata J, Haruma K. et al . Giant gastric folds: differential diagnosis at US.  Radiology. 2003;  226 686-690
  CrossrefPubMedSearch in Google Scholar
• 8 Chen T K, Wu C H, Lee C L. et al . Endoscopic ultrasonography in the differential diagnosis of giant gastric folds.  J Formos Med Assoc. 1999;  98 261-264
  PubMedSearch in Google Scholar
• 9 Songür Y, Okai T, Watanabe H. et al . Endosonographic evaluation of giant gastric folds.  Gastrointest Endosc. 1995;  41 468-474
  CrossrefPubMedSearch in Google Scholar

U. Will, M. D.

Dept. of Gastroenterology · Municipal Hospital · Gera · Germany

Fax: +49-365-8282402 ·

Email: uwe.will@wkg.srh.de