Department of Internal Medicine and Rheumatology, Juntendo University Urayasu Hospital, Chiba, Japan.
Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan.
Mod Rheumatol Case Rep. 2021 Jul;5(2):310-316. doi: 10.1080/24725625.2021.1886666. Epub 2021 Feb 25.
Anti-MDA5 antibody-positive clinically amyopathic dermatomyositis (CADM) is often complicated by rapidly progressive interstitial lung disease and is associated with poor prognosis. However, even though recurrence is reported to be infrequent if successful medical treatment is administered, the long-term prognosis remains unclear. In this case report, we examined the clinical features and treatment details of three patients with anti-MDA5 antibody-positive CADM with multiple recurrences during long-term survival at Juntendo University Urayasu Hospital. Of the three patients, two failed to convert to an anti-MDA5 antibody-negative status, and one patient died. One of the remaining patients experienced two relapses but eventually tested negative for anti-MDA5 antibodies and showed a relatively stable clinical course. Although cases of recurring anti-MDA5 antibody-positive CADM rarely occur, they may occasionally be fatal. The prognosis for anti-MDA5 antibody-positive CADM has improved over time owing to its establishment as a disease. However, further information and research is necessary to ascertain its long-term prognosis.
抗 MDA5 抗体阳性的无肌病性皮肌炎(CADM)常伴有快速进展性间质性肺病,预后不良。然而,尽管报道称如果给予成功的药物治疗,复发频率较低,但长期预后仍不清楚。在本病例报告中,我们研究了在顺天堂大学浦安医院长期生存的 3 例抗 MDA5 抗体阳性 CADM 伴多次复发患者的临床特征和治疗细节。在这 3 例患者中,有 2 例未能转为抗 MDA5 抗体阴性,1 例患者死亡。其中一名存活的患者经历了两次复发,但最终抗 MDA5 抗体转为阴性,且临床过程相对稳定。虽然复发的抗 MDA5 抗体阳性 CADM 病例很少见,但偶尔可能致命。由于该病的确立,抗 MDA5 抗体阳性 CADM 的预后已得到改善。然而,仍需要进一步的信息和研究来确定其长期预后。