Endocrine Abstracts

Introduction: Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease with heterogeneous presentation. SLE can be associated with autoimmune diseases or other entities sush as neuroendocrine tumors (NET). The occurrence of gastric NET in SLE is very rare and has been described few times in the literature. Herein we report the case of SLE woman, in whom we discovered a gastric neuroendocrine tumor.

Observation: A 68-year-old woman, with a history of treated arterial hypertension and SLE, presented a hypercalcemia (2.72 mmol/l) in a routine check-up. SLE was diagnosed, 12 years before, according to the American College of Rheumatology (ACR) SLE criteria with oral ulcers, non-scarring alopecia, discoid lupus, leukopenia, thrombocytopenia, autoimmune hemolytic anemia, positive antinuclear antibodies (ANA), and low complements. She had no kidney, heart, joint, or neuropsychiatric impairment. The investigations (thyroid test, celiac serology, and digestive endoscopies) for the search of autoimmune conditions associated with her disease were initially negative or normal. She was treated with antimalarials and corticosteroids. The evolution was good and she did not present any relapses of her SLE. Given the hypercalcemia presented by our patient, after eliminating the emergencies, we began an exhaustive etiological investigation. There were no functional complaints. Our patient denied drug or toxic intake. Laboratory tests revealed normal level of phosphatemia, parathyroid hormone and vitamin D. A body scanner didn’t reveal any abnormalities. A gastroscopy showed multiple millimetric fundal elevations with ulceration in the center. On histological examination, it was a well-differentiated grade 1 NET, with, on immunohistochemical study, intense and diffuse cytoplasmic staining of the tumor cells after the use of chromogranin-A. Furthermore, the fundic mucosa was atrophic with reduced glandular volume, suggesting Biermer’s disease. Vitamin B12 was low (115 pg/ml), antiparietal cell antibody was positive, and anti-intrinsic factor antibody was negative. Beside, blood chromogranin-A was high (681 ng/ml). After confirming the diagnosis of Biermer’s disease, NET was classified as type I. Therefore, according to The National Comprehensive Cancer Network (NCCN) guidelines, we opted for annual monitoring by fibroscopy, with monthly vitamin B12 injections in addition to endoscopic resection. The current follow-up is 2 years with a good evolution.

Conclusion: NET-SLE is a scarce association. In our observation, it occurred on a Biermer’s disease, which can be part of an autoimmune manifestation associated with SLE. NET was revealed by hypercalcemia in our case, therefore we highlight the importance of an exhaustive etiological investigation of hypercalcemia especially in SLE patients.