Lipatov Kirill, Kubbara Aahd F, Weerheim Laura E, Schiavo Dante N, Specks Ulrich, Ryu Jay H, Baqir Misbah
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
Respir Med Case Rep. 2021 May 19;33:101432. doi: 10.1016/j.rmcr.2021.101432. eCollection 2021.
Constrictive bronchiolitis is one of the manifestations of small-airway involvement in primary Sjögren syndrome (SS) and is associated with fixed airflow obstruction despite treatment with bronchodilators, macrolides, corticosteroids, and corticosteroid-sparing agents. Reports have shown a beneficial effect of rituximab on interstitial lung disease associated with SS, but the effect of rituximab on constrictive bronchiolitis is unknown. Herein, we present 2 cases of patients with constrictive bronchiolitis associated with SS who experienced symptomatic improvement and stabilization of pulmonary function testing (PFT) after rituximab therapy. Lung function declined in one of the patients when B cells reconstituted, with improved PFT results on re-administration of rituximab. Our case reports suggest that B cells may be involved in the pathogenesis of SS-associated constrictive bronchiolitis. Therapy targeting B cells may therefore be helpful in treating this debilitating and refractory condition. Further research is warranted.
缩窄性细支气管炎是原发性干燥综合征（SS）中小气道受累的表现之一，尽管使用支气管扩张剂、大环内酯类药物、皮质类固醇和皮质类固醇节约剂进行治疗，仍与固定性气流受限相关。报告显示利妥昔单抗对与SS相关的间质性肺病有有益作用，但利妥昔单抗对缩窄性细支气管炎的作用尚不清楚。在此，我们报告2例与SS相关的缩窄性细支气管炎患者，在接受利妥昔单抗治疗后症状改善，肺功能测试（PFT）稳定。其中1例患者在B细胞重建时肺功能下降，再次使用利妥昔单抗后PFT结果改善。我们的病例报告表明，B细胞可能参与了SS相关缩窄性细支气管炎的发病机制。因此，针对B细胞的治疗可能有助于治疗这种使人衰弱且难治的疾病。有必要进行进一步研究。
