结节病和自身免疫性肝炎和原发性胆汁性胆管炎(PBC)的重叠综合征具有共同的临床,生物,和组织学特征。在少数病例中报道了这些疾病的同时发生,这表明在两种情况下都有可能导致肉芽肿形成的共同途径。我们报告了两名女性患者与结节病和炎症性肝病有关的病例。第一例是一名61岁的女性,接受了PBC和自身免疫性肝炎(AIH)重叠综合征的监测。因此,已开始与熊去氧胆酸(UDCA)相关的硫唑嘌呤治疗。硫唑嘌呤因消化不耐受而不得不停用,特别是慢性腹泻和腹痛。患者在UDCA上保持临床稳定,她的肝功能测试稳定多年,直到她出现进行性呼吸困难的症状,没有任何其他相关症状。胸部计算机断层扫描(CT)显示纵隔增大,双侧肺结节,和对称的腺样体在纵隔。支气管肺泡灌洗(BAL)显示细胞增多,淋巴细胞计数显着升高(48%),CD4/CD8比率为4。患者接受了纵隔镜检查;对右后气管(4R)腺样体进行了活检。淋巴结组织学检查显示上皮样和巨细胞结核样淋巴结炎,无坏死,与结节病相容。眼科和心脏评估正常。体积描记术测试正常,不需要皮质类固醇治疗;计划进行监测。继续用UDCA治疗。第二例是一名无病史的50岁女性,其症状包括眼睛和口腔干涩,炎症型多关节痛影响膝盖和手腕,双边雷诺现象,右侧软骨病疼痛,6个月以上呼吸困难恶化.肝脏分析显示碱性磷酸酶(ALP)升高至正常上限(ULN)的三倍,γ-谷氨酰转移酶(GGT)升高至ULN的5倍。这种胆汁淤积与转氨酶活性增加至ULN的5倍超过六个月有关。免疫测试显示抗核抗体(ANA)阳性,抗Ro52,抗M23E,和抗着丝粒抗体.胸部CT显示双侧细支气管实质微结节,主要在上部和后部区域,无任何纵隔腺肿大。支气管内镜检查正常,活检显示慢性炎症。BAL显示细胞数量增加,淋巴细胞计数高(51.7%),CD4/CD8比率为2.8。小唾液腺活检显示4级淋巴细胞性唾液腺炎。皮肤活检显示上皮样肉芽肿,无干酪样坏死。在存在细胞溶解和中度肝功能不全的情况下进行肝活检,发现肉芽肿性肝炎和胆管炎病变以及间隔纤维化提示PBC。保留了PBC和Sjögren综合征的皮肤和肺结节病的诊断。肺活量测定和一氧化碳值的扩散能力正常。治疗涉及UDCA,皮质类固醇,还有硫唑嘌呤,导致临床和生物学改善。结节病与自身免疫性肝病有一些临床表现,主要是PBC。具有不同外观和位置的肝肉芽肿可以指导诊断。早期诊断和适当处理可避免严重并发症,改善预后。
Sarcoidosis and the overlap syndrome of autoimmune hepatitis and primary biliary cholangitis (PBC) share common clinical, biological, and histological features. The simultaneous occurrence of these diseases have been reported in few cases and suggests that a common pathway which may contribute to granuloma formation in both conditions. We report the cases of two female patients having an association of sarcoidosis and inflammatory liver diseases. The first
case is of a 61-year-old woman had been monitored for an overlap syndrome of PBC and autoimmune hepatitis (AIH). Therefore, treatment with azathiprine has been initiated associated with ursodeoxycholic acid (UDCA). Azathioprine had to be discontinued due to digestive intolerance, specifically chronic diarrhea and abdominal pain. The patient remained clinically stable on UDCA and her liver function tests were stable for years, until she developed symptoms of progressive dyspnea without any other associated signs. Chest computed tomography (CT) revealed mediastinal enlargement, bilateral pulmonary nodules, and symmetrical adenomegalies in the mediastinum. The bronchoalveolar lavage (BAL) revealed increased cellularity, with a notable elevation in lymphocyte count (48 %) and a CD4/CD8 ratio of 4. The patient underwent mediastinoscopy; a biopsy of the right laterotracheal (4R) adenomegaly was performed. Histological examination of the lymph node showed epithelioid and giant-cell tuberculoid lymphadenitis without necrosis, compatible with sarcoidosis. Ophthalmological and cardiac assessments were normal. Plethysmography test was normal and there was no need for corticosteroid treatment; a surveillance was planned. Treatment with UDCA was pursued. The second
case is of a 50-year-old woman with no medical history presented symptoms including dry eyes and mouth, inflammatory-type polyarthralgia affecting knees and wrists, bilateral Raynaud\'s phenomenon, right hypochondrium pain, and worsening dyspnea over six months. Liver analysis showed elevation of alkaline phosphatase (ALP) to three times upper limit of normal (ULN) and gamma-glutamyltransferase (GGT) to 5 times ULN. This cholestasis was associated with an increase in transaminase activity to 5 times ULN for over six months. Immunological tests revealed positive anti-nuclear antibodies (ANA), anti-Ro52, anti-M23E, and anti-centromere antibodies. Chest-CT showed multiple bilateral bronchiolar parenchymal micronodules mostly in the upper and posterior regions without any mediastinal adenomegaly. Bronchial endoscopy was normal, and biopsies indicated chronic inflammation. The BAL revealed increased cellularity, characterized by a high lymphocyte count (51.7 %) and a CD4/CD8 ratio of 2.8. Biopsy of minor salivary gland revealed grade 4 lymphocytic sialadenitis. Skin biopsy revealed an epithelioid granuloma without caseous necrosis. Liver biopsy performed in the presence of cytolysis and moderate hepatic insufficiency, revealed granulomatous hepatitis and cholangitis lesions along with septal fibrosis suggestive of PBC. The diagnosis of cutaneous and pulmonary sarcoidosis with PBC and Sjögren\'s syndrome was retained. The spirometry and diffusing capacity for carbon monoxide value were normal. Treatment involved UDCA, corticosteroids, and azathioprine, leading to clinical and biological improvement. Sarcoidosis shares some clinical manifestations with autoimmune liver diseases, primarily PBC. A hepatic granuloma with a different appearance and location can guide the diagnosis. Early diagnosis and appropriate management can avoid serious complications and improve prognosis.