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A rare autoimmune disorder involving the fundic glands
Most patients have malabsorption secondary to chronic Helicobacter pylori infection that results in
Atrophic gastritis and achlorhydria
Small intestine bacterial overgrowth
Insufficient absorption of dietary vitamin B12
Fundic histology shows severe gland atrophy and intestinal metaplasia
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Hypergastrinemia may induce hyperplasia of gastric enterochromaffin-like cells, development of small, multicentric carcinoid tumors in 5%
Risk of adenocarcinoma is increased threefold
Prevalence is 1–3%
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Endoscopy with biopsy
Achlorhydria leads to pronounced hypergastrinemia (> 1000 pg/mL)
Anti-intrinsic factor antibodies are present in 70%
Low serum vitamin B12 level
Megaloblastic (macrocytic) anemia
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One-time endoscopic screening for adenocarcinoma or carcinoids recommended
Patients with extensive atrophy and metaplasia involving the gastric antrum and body, dysplasia, or small carcinoids require periodic endoscopic surveillance
Vitamin B12 100 mcg intramuscularly daily for first week, weekly for first month, then monthly for life if B12 deficiency documented