Idoyaga Pablo, Fielli Mariano, González Alejandra, Ferreyra Mufarregue Leila R, Fernández Casares Marcelo, Crevena Gabriela
1Servicio de Neumonología, Hospital Nacional Profesor Alejandro Posadas, El Palomar, Buenos Aires, Argentina. E-mail:
Servicio de Neumonología, Hospital Nacional Profesor Alejandro Posadas, El Palomar, Buenos Aires, Argentina.
Medicina (B Aires). 2018;78(6):449-452.
IgG4-related disease is a heterogeneous group of diseases that can affect a single organ or manifest as a systemic disease. We present the case of a 55-yearold female, admitted for dry cough, fever, progressive dyspnea and respiratory failure. Chest CT showed areas of diffuse ground glass, reticular pattern and alveolar consolidation. She received treatment for community acquired pneumonia and systemic corticosteroids with good response. One month later, she was admitted again due to subglottic stenosis, progression of pulmonary infiltrates, and increased palpebral and submaxillary glands volume. Transbronchial and salivary gland biopsies showed infiltration by IgG4. Increased levels of plasma IgG4 were also detected. Immunosuppressive therapy was given with good response.
IgG4相关疾病是一组异质性疾病，可累及单一器官或表现为全身性疾病。我们报告一例55岁女性病例，因干咳、发热、进行性呼吸困难和呼吸衰竭入院。胸部CT显示有弥漫性磨玻璃影、网状影和肺泡实变区域。她接受了社区获得性肺炎的治疗及全身糖皮质激素治疗，反应良好。一个月后，她因声门下狭窄、肺部浸润进展以及睑腺和颌下腺肿大再次入院。经支气管活检和唾液腺活检显示有IgG4浸润。还检测到血浆IgG4水平升高。给予免疫抑制治疗，反应良好。
