The Korean Journal of Helicobacter and Upper Gastrointestinal Research 2021;21(3):226-234.
Published online: August 2, 2021 // DOI: https://doi.org/10.7704/kjhugr.2021.0016
<Case Report> 우리나라에서 발표된 첫 cases report
<Case 1, F/54>
Figure 1. Gastric neuroendocrine tumors in an autoimmune gastritis patient with general weakness. (A) Retroflexed view shows several subepithelial tumors with diffuse atrophy in the upper body. (B) Antegrade view shows diffuse atrophy with several subepithelial tumors in the lower body. Transparent vessels are observed on the surface of the subepithelial lesions. The lesions were hard in consistency without the cushion or rolling sign. (C) No atrophy is observed in the antrum. (D) Neuroendocrine tumors arising from the atrophic gastric mucosa (H&E stain, ×40). (E) Tumor cells positive for synaptophysin, which is specific for neuroendocrine tumors (synaptophysin immunohistochemical stain, ×40). (F) Proliferation of enterochromaffin-like cells observed with atrophy in the biopsied specimen taken from the lower body (H&E stain, ×200). (G) In the biopsied specimen taken from the upper body, atrophic mucosa without fundic glands are observed. Pseudo-pyloric metaplasia is also observed (left part of this image) with intestinal metaplasia and lymphoplasma cell infiltration (right part) in the atrophic mucosa (H&E stain, ×200).
<Case 2, F/58>
Figure 2. Multiple gastric neuroendocrine tumors in an autoimmune gastritis patient with newly-diagnosed thyroid cancer. (A) Diffuse atrophy is observed with multiple subepithelial lesions in the fundus. (B) Retroflex view of the upper body reveals multiple subepithelial lesions with yellowish sticky mucus. A grade 1 neuroendocrine tumor with positive staining for chromogranin A and synaptophysin is observed in the biopsied specimen of the largest subepithelial lesion in the greater curvature side of the upper body. (C) Linear hyperemic streaks are evident in the antrum without atrophy.
<Case 3, F/62>
Figure3. Endoscopic findings of an autoimmune gastritis patient with a history of breast cancer resection. (A) Diffuse atrophy is observed in the fundus. (B) Retroflex view of the body shows diffuse atrophy. (C) Diffuse irregular nodularity is observed in the antrum. Mild intestinal metaplasia and erosion are evident in the biopsied specimen from the elevated lesion in the posterior wall of the antrum. (D) In the lamina propria taken from the upper body, lymphoplasma cell infiltration can be observed in the atrophic mucosa (H&E stain, ×200). On the right side of this image, the pink-colored cells indicate parietal cells in remnant fundic glands. Intestinal metaplasia and pseudo-pyloric metaplasia are observed in other parts of the image. The findings are consistent with autoimmune gastritis. (E) No abnormal findings are observed in the biopsied specimen taken from the background mucosa of the antrum (H&E stain, ×200).
<Case 4, F/61>
Figure 4. Endoscopic findings in an autoimmune gastritis patient with autoimmune thyroiditis. (A) Retroflexed view shows diffuse atrophy in the upper body. (B) Diffuse atrophy extending down to the lower body. (C) No atrophy is observed in the antrum. (D) An ulcer is observed in the lesser curvature side of the distal antrum. A mild degree of intestinal metaplasia and erosion are observed in the biopsied specimen from the margin of the ulcer. Helicobacter pylori was found to be absent.
<Case 5, F/60>
Figure 5. Endoscopic findings in an autoimmune gastritis patient with a history of Helicobacter pylori eradication and gastric hyperplastic polyp resection. (A) Diffuse mucosal nodularity and atrophy are observed in the fundus with whitish nodular lesions. (B) Retroflex view of the body with diffuse mucosal nodularity and atrophy. There was no evidence of recurrence in the lesser curvature side of the lower body after gastric hyperplastic polyp resection (2 years prior). (C) Diffuse mucosal nodularity and atrophy extending down to the antrum. A 1.5 cm-sized elevated lesion is observed in the greater curvature side of the antrum. A marked degree of intestinal metaplasia was found in the biopsied specimen from the elevated lesion.
*Discussion
- 체부 우세형 위축성 위염이 관찰된 한국인 4명과 백인 1명에서 혈청 항벽세포 항체를 검사한 결과, 양성으로 보고되어 자가면역성 위염으로 진단하였다.
- 폐경 후의 여성, 평균 나이는 59.0±3.2세 (3명은 자가 면역성 갑상선염으로 치료 중)
- H. pylori 감염력의 증거가 없는 미감염자 2명에서는 위에서 다발성 신경내분비종양이 함께 진단 됨
- 자가면역성 위염은 H. pylori 감염 상태와 무관하게 발생할 수 있는 질환으로 의사가 내시경 소견을 보고 체부 우세형 위염을 진단하여 혈청 항벽세포나 항내인성인자 항체를 검사하지 않는 이상 진단할 수 없는 질환이다. (2023년 6월, ultra-early AIG 증례가 발표되었습니다. 내시경 진단률은 30% 정도로 낮기 때문에 정확한 진단을 위한 보다 많은 논의가 필요할 것으로 보입니다)
- 자가면역성 위염은 H. pylori 감염력이 있는 한국인에서도 발생하므로 체부 우세형 위축이 저명하거나 자가면역성 갑상선염이 있는 환자가 낮은 혈청 PG I 수치를 보이면 혈청 항체 검사를 해서 진단할 것을 권한다. (자가면역질환(특히, 갑상선염이나 갑상선 항진증)이 있는 경우, 내시경에서 저명한 체부 우세형 위축, 낮은 혈청 PG I, PG I/II ratio, Hypergastrinemia의 경우 Anti-parietal cell ab. 검사를 시행하고, 가급적 조직검사도 함께 시행하여 진단해야 하겠습니다.)
<Abstract>
Autoimmune gastritis is a corpus-dominant type of gastritis with positive serum anti-parietal cell antibodies (APCA) and/or anti-intrinsic factor antibodies. Serum APCA and pepsinogen (PG) assays were performed in subjects with corpus-dominant gastritis detected by endoscopy. Serum APCA was positive in five patients. All these patients were postmenopausal women (four Koreans and one Caucasian from the Russian Federation) with a mean age of 59.0±3.2 years. They displayed low PG I levels ranging from 8.1 to 18.8 ng/mL (mean, 11.4±4.8 ng/mL) and low PG I/II ratios ranging from 0.7 to 2.4 (mean, 1.2±0.7). Three of the patients were being treated for autoimmune thyroiditis. Multiple gastric neuroendocrine tumors were observed in two Helicobacter pylori (H. pylori)-naive patients with high serum gastrin levels exceeding 700 pg/mL and serum chromogranin A levels exceeding 1,000 ng/mL. In the remaining three patients, intestinal metaplasia was observed in the biopsied specimens from the antrum, suggesting a history of H. pylori infection. Our findings indicate the value of positive serum APCA findings, low serum PG I levels, and low serum PG I/II ratios in confirming autoimmune gastritis in patients showing corpus-dominant atrophy, regardless of their H. pylori infection status.
The Korean Journal of Helicobacter and Upper Gastrointestinal Research 2021;21(3):226-234.
Published online: August 2, 2021 // DOI: https://doi.org/10.7704/kjhugr.2021.0016
<Case Report> 우리나라에서 발표된 첫 cases report
<Case 1, F/54>
Figure 1. Gastric neuroendocrine tumors in an autoimmune gastritis patient with general weakness. (A) Retroflexed view shows several subepithelial tumors with diffuse atrophy in the upper body. (B) Antegrade view shows diffuse atrophy with several subepithelial tumors in the lower body. Transparent vessels are observed on the surface of the subepithelial lesions. The lesions were hard in consistency without the cushion or rolling sign. (C) No atrophy is observed in the antrum. (D) Neuroendocrine tumors arising from the atrophic gastric mucosa (H&E stain, ×40). (E) Tumor cells positive for synaptophysin, which is specific for neuroendocrine tumors (synaptophysin immunohistochemical stain, ×40). (F) Proliferation of enterochromaffin-like cells observed with atrophy in the biopsied specimen taken from the lower body (H&E stain, ×200). (G) In the biopsied specimen taken from the upper body, atrophic mucosa without fundic glands are observed. Pseudo-pyloric metaplasia is also observed (left part of this image) with intestinal metaplasia and lymphoplasma cell infiltration (right part) in the atrophic mucosa (H&E stain, ×200).
<Case 2, F/58>
Figure 2. Multiple gastric neuroendocrine tumors in an autoimmune gastritis patient with newly-diagnosed thyroid cancer. (A) Diffuse atrophy is observed with multiple subepithelial lesions in the fundus. (B) Retroflex view of the upper body reveals multiple subepithelial lesions with yellowish sticky mucus. A grade 1 neuroendocrine tumor with positive staining for chromogranin A and synaptophysin is observed in the biopsied specimen of the largest subepithelial lesion in the greater curvature side of the upper body. (C) Linear hyperemic streaks are evident in the antrum without atrophy.
<Case 3, F/62>
Figure3. Endoscopic findings of an autoimmune gastritis patient with a history of breast cancer resection. (A) Diffuse atrophy is observed in the fundus. (B) Retroflex view of the body shows diffuse atrophy. (C) Diffuse irregular nodularity is observed in the antrum. Mild intestinal metaplasia and erosion are evident in the biopsied specimen from the elevated lesion in the posterior wall of the antrum. (D) In the lamina propria taken from the upper body, lymphoplasma cell infiltration can be observed in the atrophic mucosa (H&E stain, ×200). On the right side of this image, the pink-colored cells indicate parietal cells in remnant fundic glands. Intestinal metaplasia and pseudo-pyloric metaplasia are observed in other parts of the image. The findings are consistent with autoimmune gastritis. (E) No abnormal findings are observed in the biopsied specimen taken from the background mucosa of the antrum (H&E stain, ×200).
<Case 4, F/61>
Figure 4. Endoscopic findings in an autoimmune gastritis patient with autoimmune thyroiditis. (A) Retroflexed view shows diffuse atrophy in the upper body. (B) Diffuse atrophy extending down to the lower body. (C) No atrophy is observed in the antrum. (D) An ulcer is observed in the lesser curvature side of the distal antrum. A mild degree of intestinal metaplasia and erosion are observed in the biopsied specimen from the margin of the ulcer. Helicobacter pylori was found to be absent.
<Case 5, F/60>
Figure 5. Endoscopic findings in an autoimmune gastritis patient with a history of Helicobacter pylori eradication and gastric hyperplastic polyp resection. (A) Diffuse mucosal nodularity and atrophy are observed in the fundus with whitish nodular lesions. (B) Retroflex view of the body with diffuse mucosal nodularity and atrophy. There was no evidence of recurrence in the lesser curvature side of the lower body after gastric hyperplastic polyp resection (2 years prior). (C) Diffuse mucosal nodularity and atrophy extending down to the antrum. A 1.5 cm-sized elevated lesion is observed in the greater curvature side of the antrum. A marked degree of intestinal metaplasia was found in the biopsied specimen from the elevated lesion.
*Discussion
- 체부 우세형 위축성 위염이 관찰된 한국인 4명과 백인 1명에서 혈청 항벽세포 항체를 검사한 결과, 양성으로 보고되어 자가면역성 위염으로 진단하였다.
- 폐경 후의 여성, 평균 나이는 59.0±3.2세 (3명은 자가 면역성 갑상선염으로 치료 중)
- H. pylori 감염력의 증거가 없는 미감염자 2명에서는 위에서 다발성 신경내분비종양이 함께 진단 됨
- 자가면역성 위염은 H. pylori 감염 상태와 무관하게 발생할 수 있는 질환으로 의사가 내시경 소견을 보고 체부 우세형 위염을 진단하여 혈청 항벽세포나 항내인성인자 항체를 검사하지 않는 이상 진단할 수 없는 질환이다. (2023년 6월, ultra-early AIG 증례가 발표되었습니다. 내시경 진단률은 30% 정도로 낮기 때문에 정확한 진단을 위한 보다 많은 논의가 필요할 것으로 보입니다)
- 자가면역성 위염은 H. pylori 감염력이 있는 한국인에서도 발생하므로 체부 우세형 위축이 저명하거나 자가면역성 갑상선염이 있는 환자가 낮은 혈청 PG I 수치를 보이면 혈청 항체 검사를 해서 진단할 것을 권한다. (자가면역질환(특히, 갑상선염이나 갑상선 항진증)이 있는 경우, 내시경에서 저명한 체부 우세형 위축, 낮은 혈청 PG I, PG I/II ratio, Hypergastrinemia의 경우 Anti-parietal cell ab. 검사를 시행하고, 가급적 조직검사도 함께 시행하여 진단해야 하겠습니다.)
<Abstract>
Autoimmune gastritis is a corpus-dominant type of gastritis with positive serum anti-parietal cell antibodies (APCA) and/or anti-intrinsic factor antibodies. Serum APCA and pepsinogen (PG) assays were performed in subjects with corpus-dominant gastritis detected by endoscopy. Serum APCA was positive in five patients. All these patients were postmenopausal women (four Koreans and one Caucasian from the Russian Federation) with a mean age of 59.0±3.2 years. They displayed low PG I levels ranging from 8.1 to 18.8 ng/mL (mean, 11.4±4.8 ng/mL) and low PG I/II ratios ranging from 0.7 to 2.4 (mean, 1.2±0.7). Three of the patients were being treated for autoimmune thyroiditis. Multiple gastric neuroendocrine tumors were observed in two Helicobacter pylori (H. pylori)-naive patients with high serum gastrin levels exceeding 700 pg/mL and serum chromogranin A levels exceeding 1,000 ng/mL. In the remaining three patients, intestinal metaplasia was observed in the biopsied specimens from the antrum, suggesting a history of H. pylori infection. Our findings indicate the value of positive serum APCA findings, low serum PG I levels, and low serum PG I/II ratios in confirming autoimmune gastritis in patients showing corpus-dominant atrophy, regardless of their H. pylori infection status.