Objective: Interstitial lung disease（ILD）is a serious complication that can affect the prognosis for with rheumatoid arthritis（RA）patients, but appropriate treatment has not been established to ...address this issue. This study aimed to clarify the efficacy and safety of Janus kinase inhibitors （JAKi）in RA patients with ILD.Methods: This non-interventional observational study included 26 RA patients with ILD complications who were treated with JAKi. ILD was evaluated by changes in computed tomography（CT）scores（fibrosis and ground-glass opacity GGO）12 months after JAKi initiation. The safety evaluation items were acute exacerbation of ILD and severe infections requiring hospitalization.Results: In total, 17 patients were able to continue JAKi for over 12 months, while 9 discontinued. f these 17 patients, 12 patients were analyzed using CT scores. The CT-GGO scores were significantly decreased, but the CT-fibrosis scores remained steady throughout the 12 months. （p=0.037 and p=0.813, respectively）. The DAS28-CRP and prednisolone dosage were significantly decreased from the initiation to 12 months later（p<0.001 and p=0.031, respectively）. Among the 26 patients, no exacerbation of existing ILD and two infections（same patient）requiring hospitalization were observed（6.4/100 person-year）.Conclusion: Although this study has several limitations, such as a small sample size and limited observation period, the results support that JAKi may be a therapeutic option for RA-ILD patients due to its efficacy for arthritis and safety.
A 44-year-old Japanese woman was diagnosed with type 1 hereditary angioedema (HAE) at the age of 30. In March 2007, she began suffering from severe abdominal pain due to intestinal edema. After ...treatment with C1-INH concentrate, her symptoms disappeared. However, during the subsequent three years, the frequency of the attacks increased continuously, and C1-INH concentrate was necessary for treatment of every attack. The increase in the number of attacks might have been due to the frequent injection of C1-INH concentrate or the deterioration of her disease course. In a genetic investigation, the patient was found to have a novel mutation in the C1-INH gene.
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