Department of Allergy & Immunology, Huashan Hospital, Fudan University, Shanghai, China.
Department of Dermatology, Huashan Hospital, Shanghai Institute of Dermatology, Fudan University, Shanghai, China.
Exp Dermatol. 2024 Jul;33(7):e15136. doi: 10.1111/exd.15136.
Interstitial lung disease (ILD) has been identified as a prevalent complication and significant contributor to mortality in individuals with pemphigus. In this study, a murine model of pemphigus was developed through the subcutaneous administration of serum IgG obtained from pemphigus patients, allowing for an investigation into the association between pemphigus and ILD. Pulmonary interstitial lesions were identified in the lungs of a pemphigus mouse model through histopathology, RT-qPCR and Sircol assay analyses. The severity of these lesions was found to be positively associated with the concentration of IgG in the injected serum. Additionally, DIF staining revealed the deposition of serum IgG in the lung tissue of pemphigus mice, indicating that the subcutaneous administration of human IgG directly impacted the lung tissue of the mice, resulting in damage. This study confirms the presence of pulmonary interstitial lesions in the pemphigus mouse model and establishes a link between pemphigus and ILD.
间质性肺病 (ILD) 已被确定为天疱疮患者的常见并发症和死亡的重要原因。在这项研究中，通过皮下注射来自天疱疮患者的血清 IgG 建立了天疱疮的小鼠模型，从而研究天疱疮和间质性肺病之间的关系。通过组织病理学、RT-qPCR 和 Sircol 分析，在天疱疮小鼠模型的肺部发现了肺间质病变。这些病变的严重程度与注射血清中的 IgG 浓度呈正相关。此外，DIF 染色显示血清 IgG 在天疱疮小鼠的肺组织中沉积，表明人 IgG 的皮下注射直接影响了小鼠的肺组织，导致损伤。这项研究证实了天疱疮小鼠模型中存在肺间质病变，并建立了天疱疮和间质性肺病之间的联系。
