Department of Respiratory Medicine, Juntendo University Shizuoka Hospital, 1129 Nagaoka, Izunokuni, Shizuoka, 410-2295, Japan.
Department of Respiratory Medicine, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-8421, Japan.
J Med Case Rep. 2021 Apr 18;15(1):178. doi: 10.1186/s13256-021-02797-3.
Sarcoidosis is pathologically characterized by the formation of non-necrotizing epithelioid cell granulomas. However, pathological findings of patients with sarcoidosis have rarely revealed necrosis. We report here on a patient with sarcoidosis which needed to be distinguished from infectious disease because of marked necrosis in the lymph nodes.
A 46-year-old Japanese woman was referred to our hospital due to a dry cough and appetite loss. A chest X-ray and computed tomography revealed markedly enlarged mediastinal and hilar lymph nodes and hepatosplenomegaly. Surgical biopsy of these lymph nodes was performed in order to make a diagnosis. Pathological findings revealed epithelioid cell granuloma with marked necrosis that suggested infectious etiology such as mycobacterial and fungal infections. In addition to the pathological findings, immunoglobulin A (IgA) antibody for Mycobacterium avium complex (MAC), enlargement of lymph nodes and hepatosplenomegaly indicated disseminated MAC, while sarcoidosis was considered as another important differential diagnosis according to elevated angiotensin-converting enzyme, soluble interleukin-2 receptor and uveitis. While waiting for the results of the cultures of acid-fast bacilli, the symptoms of cough and consumption had worsened, and initiation of therapy was required before the confirmed diagnosis. The therapy for MAC was initiated because it was feared that immunosuppressive therapy containing corticosteroid for sarcoidosis could worsen the patient's condition if MAC infection was the main etiology. However, the treatment for MAC was not effective, and it was clarified that no acid-fast bacilli were cultured in the liquid culture medium, so the diagnosis was corrected to sarcoidosis after reconsideration of clinical and pathological findings. Prednisolone (30 mg/day) was administered orally, and the patient's symptoms and radiological findings improved.
Sarcoidosis must be considered even if pathological findings reveal marked necrosis, because rare cases of sarcoidosis exhibit extensive necrosis in lymph nodes. It is extremely important to carefully examine the clinical and pathological findings through discussion with the examining pathologist to reach the correct diagnosis.
结节病的病理学特征为非坏死性上皮样细胞肉芽肿的形成。然而,结节病患者的病理表现很少出现坏死。我们在此报告一例结节病患者,由于淋巴结明显坏死,需要与传染病相鉴别。
一名 46 岁的日本女性因干咳和食欲减退而被转至我院。胸部 X 线和计算机断层扫描显示纵隔和肺门淋巴结明显肿大,以及肝脾肿大。为明确诊断,对这些淋巴结进行了外科活检。病理检查发现有明显坏死的上皮样细胞肉芽肿,提示可能为分枝杆菌和真菌感染等感染性病因。除了病理学发现外,免疫球蛋白 A(IgA)抗体检测提示鸟分枝杆菌复合群(MAC)感染、淋巴结肿大和肝脾肿大,提示 MAC 播散性感染,而结节病也被认为是另一个重要的鉴别诊断,依据为血管紧张素转换酶升高、可溶性白细胞介素-2 受体升高和葡萄膜炎。在等待抗酸杆菌培养结果的同时,咳嗽和消耗症状恶化,需要在确诊前开始治疗。由于担心含有皮质类固醇的免疫抑制疗法会加重患者的病情(如果 MAC 感染是主要病因),因此开始了 MAC 的治疗。然而,MAC 的治疗无效,并且在重新考虑临床和病理发现后,明确在液体培养基中未培养出抗酸杆菌,因此修正诊断为结节病。开始口服泼尼松龙(30mg/天)治疗,患者的症状和影像学表现得到改善。
即使病理表现显示明显坏死,也必须考虑结节病,因为罕见的结节病病例会出现淋巴结广泛坏死。通过与检查病理学家讨论仔细检查临床和病理发现以做出正确诊断非常重要。