Kümpers Christiane, Tharun Lars, Stellmacher Florian, Greinert Ulf, May Katharina, Perner Sven
Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, Gebäude V50, 23538, Lübeck, Deutschland.
Pathologie, Forschungszentrum Borstel, Leibniz Lungenzentrum, Borstel, Deutschland.
Pathologe. 2021 Feb;42(1):95-101. doi: 10.1007/s00292-020-00904-7. Epub 2021 Jan 25.
A 54-year-old patient with a history of pulmonary tuberculosis and occupational exposure to dust in early adulthood presented with symptoms of coughing with sputum, weight loss, occasional night sweats, and thoracic pain. Non-necrotizing granulomatosis in lung and lymph-node biopsies indicated sarcoidosis. Combined immunosuppressive therapy did not lead to an improvement. An atypical lung resectate with fibroinflammatory changes and obliterative endothelialitis may finally lead to the diagnosis of IgG4-associated lung disease with a bronchovascular pattern of involvement. The question discussed here is whether this is a coexistence of IgG4-associated lung disease with sarcoidosis or the spectrum of one disease.
一名54岁患者,有肺结核病史,成年早期有职业性粉尘接触史,出现咳嗽咳痰、体重减轻、偶尔盗汗和胸痛症状。肺和淋巴结活检显示非坏死性肉芽肿,提示结节病。联合免疫抑制治疗未见改善。具有纤维炎症改变和闭塞性内皮炎的非典型肺切除标本最终可能诊断为IgG4相关性肺病,呈支气管血管受累模式。这里讨论的问题是,这是IgG4相关性肺病与结节病并存,还是一种疾病的不同表现。