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Article in Japanese
A case of protein-losing gastroenteropathy associated with Sjögren's syndrome-related interstitial pneumonia
Satoshi Araia Naoko Matoa Takao Nagashimab Naoto Araia Masashi Bandoa Koichi Hagiwaraa
aDivision of Pulmonary Medicine, Department of Medicine, Jichi Medical University
bDepartment of Rheumatology and Clinical Immunology, Jichi Medical University
We herein report a case of protein-losing gastroenteropathy in a 77-year-old female patient. She had been diagnosed with Sjögren's syndrome-associated interstitial pneumonia 12 years earlier. Treatment with a low-dose oral glucocorticoid had stabilized the pulmonary lesions. However, dyspnea on exercise progressively increased and was accompanied by the development of leg edema and thoracoabdominal effusion. Consequently, the patient developed hypoproteinemia and hypocomplementemia. Multimodal examination, including thoracoscopy, did not yield any specific findings. Nevertheless, despite the lack of gastrointestinal symptoms, the α1-antitrypsin clearance rate was elevated, and intestinal protein loss was evident on scintigraphy. Finally, the patient was diagnosed with protein-losing gastroenteropathy. This is a rare complication of Sjögren's syndrome and is occasionally refractory to treatment with glucocorticoids. In the current case, administration of a high-dose glucocorticoid was required to achieve remission. The present case was characterized by protein leakage that developed during the stable stage of Sjögren's syndrome in the absence of typical gastrointestinal symptoms.
Sjögren's syndrome Hypocomplementemia Protein-losing gastroenteropathy Interstitial pneumonia
Received 14 Nov 2022 / Accepted 13 Dec 2022
AJRS, 12(2): 79-83, 2023