Successful Therapy in Cases Series of Systemic Autoinflammatory Disease and Literature Review

Objectives: Systemic autoinflammatory disease (SAID) is a rare systemic auto-inflammatory and progressive disorders. There have been some reports with various therapies in SAID patients. The objective of this study is to describe the chareatercis of four cases of NAIDs benefiting from JAK 1/2 inhibitor baricitinib.Methods: We reported the four cases with SAID including two cases of Blau syndrome, one case of FMF and one case of FCAS3 syndrome. These four different patients were either resistant to currently available therapies or biologics were unaccessible during COVID-19 pandemic. We also conducted a systematic literature review about the current therapies of SAID.Results: Although genetically and phenotypically different, four cases of SAID that were treated with single use baricitinib 4 mg per day achieved improvement over eight weeks. We further identified 132 manuscripts providing more than 100 cases of SAID. Among these patients, 24 underwent biological treatments and 22 of them recovered. In these 132 manuscripts, 2 underwent JAK 1/3 inhibitor tofacitinib treatment and recovered fully.Conclusions: Case series study on the use of Jak inhibitor agents have yielded positive results in our study. For SAID patients baricitinib may be a better choice compared to injection biological treatments.