Objective By literature review to retrospectively study the clinical characteristics, diagnosis and treatment process, and prognosis of anti-melanoma differentiation associated gene 5 (MDA5) autoantibody-positive dermatomyositis-associated with interstitial lung disease (ILD) treated with tofacitinib (TOF), so as to improve the understanding of the disease. Methods Retrospectively analyze the clinical and laboratory characteristics, imaging findings and adverse reactions before and after TOF treatment in seven patients who admitted to the First Medical Center of Chinese PLA General Hospital from January 2018 to October 2021. Thirty-six cases were found by searching databases to summarize the efficacy and safety of TOF in treating patients with anti-MDA5 autoantibody-positive dermatomyositis-associated ILD. Results Of the 7 patients, 5 were female and 2 were male with mean age of 50(30-64) years, the mean disease duration was 3(1-36) months, and the mean follow-up time was 12(1-35) months. Of them, 4 received previously the other immunosuppressive therapy, and 3 were newly treated. One patient died, and the remaining 6 patients were in relatively stable condition and maintenance treatment. One patient experienced fungal infection in the 18th month after TOF treatment. A total of 36 cases of anti-MDA5 autoantibody-positive dermatomyositis have been reported in databases. Clinical symptoms, laboratory tests and imaging findings were all improved after TOF therapy. Opportunistic infection occurred in 9(25%) cases after application of TOF. Conclusion Application of TOF is an effective and safe regimen in treatment of anti-MDA5 autoantibody-positive dermatomyositis-associated ILD, while attention should be paid for monitoring the opportunistic infection during treatment.
| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |