Kc Pawan, Ayele Frehiywot K, Karki Sabin, Waleed Madeeha S
Rheumatology, Emory University School of Medicine, Atlanta, USA.
Internal Medicine, Suburban Community Hospital (Lower Bucks Hospital), Bristol, USA.
Cureus. 2023 Jul 13;15(7):e41839. doi: 10.7759/cureus.41839. eCollection 2023 Jul.
Whipple's disease is a rare systemic disease caused by a infection. Although older literature reports a low rate of incidence, case reports continue to rise due to increased awareness of the disease. Classic Whipple's disease presents as weight loss, diarrhea, and arthralgia and may involve the heart, central nervous system (CNS), or any other organ system. Some patients with Whipple's disease do not have the classic signs and symptoms of the disease. We present a case of Whipple's disease in a patient with poor appetite, weight loss, and granulomatous inflammation of various organs, including the kidneys and spleen, mimicking sarcoidosis. She had presented three years earlier with acute kidney injury (AKI) and hypercalcemia. The renal biopsy revealed diffuse granulomatous interstitial nephritis. Both AKI and hypercalcemia resolved with prednisone; however, her weight loss and decreased appetite continued. The initial positron emission tomography (PET) scan showed increased fluorodeoxyglucose (FDG) avidity in the spleen and large intestine, and the splenic biopsy revealed non-caseating granulomas. A diagnosis of sarcoidosis was made, and she was started on methotrexate with prednisone. Nevertheless, the weight loss and poor appetite were relentless. A repeat PET scan showed increased FDG avidity in loops of the small and large intestines. A small intestinal biopsy revealed positive periodic acid-Schiff (PAS) and negative acid-fast bacilli (AFB) revealing the diagnosis of Whipple's disease. Whipple's disease should be considered in the differential diagnosis of sarcoidosis, especially in those patients worsening on standard immunosuppression.
惠普尔病是一种由感染引起的罕见全身性疾病。尽管早期文献报道其发病率较低，但由于对该疾病的认识提高，病例报告仍在不断增加。典型的惠普尔病表现为体重减轻、腹泻和关节痛，可能累及心脏、中枢神经系统（CNS）或任何其他器官系统。一些惠普尔病患者没有该疾病的典型体征和症状。我们报告一例惠普尔病患者，其表现为食欲不振、体重减轻以及包括肾脏和脾脏在内的多个器官出现肉芽肿性炎症，类似结节病。她三年前曾出现急性肾损伤（AKI）和高钙血症。肾活检显示弥漫性肉芽肿性间质性肾炎。使用泼尼松后，AKI和高钙血症均得到缓解；然而，她的体重减轻和食欲减退仍持续存在。最初的正电子发射断层扫描（PET）显示脾脏和大肠的氟脱氧葡萄糖（FDG）摄取增加，脾脏活检显示非干酪样肉芽肿。诊断为结节病，她开始接受甲氨蝶呤联合泼尼松治疗。尽管如此，体重减轻和食欲不佳仍持续存在。重复PET扫描显示小肠和大肠肠袢的FDG摄取增加。小肠活检显示过碘酸希夫（PAS）染色阳性，抗酸杆菌（AFB）染色阴性，从而确诊为惠普尔病。在结节病的鉴别诊断中应考虑惠普尔病，尤其是那些在标准免疫抑制治疗下病情恶化的患者。
