A case of clinically amyopathic dermatomyositis that was refractory to intensive immunosuppressive therapy including tofacitinib, but successfully treated with plasma exchange therapy.
Daisuke HiraokaJun IshizakiKenta HorieTakuya MatsumotoKoichiro SuemoriKatsuto TakenakaHitoshi HasegawaPublished in: Modern rheumatology case reports (2022)
Clinically amyopathic dermatomyositis (CADM) patients often develop rapidly progressive interstitial lung disease (RP-ILD). A high level of anti-melanoma differentiation-associated gene 5 antibodies (anti-MDA5 Ab) before treatment is associated with RP-ILD development, a poor treatment response, and poor survival. The prognosis of CADM patients remains poor due to ILD even with combined intensive immunosuppressive therapy. Recently, several additional therapies, including tofacitinib (TOF) and plasma exchange (PE) therapy, have been reported to be effective. We herein report a case of CADM-ILD with a high level of anti-MDA5 Ab that was refractory to combined intensive immunosuppressive therapy including TOF, but successfully treated with PE. The following are possible reasons why TOF was ineffective: (1) cytokines that were not suppressed by TOF played an important role in RP-ILD; (2) TOF was administered later than previously reported; and (3) TOF did not suppress pathological substances such as antibodies. On the other hand, PE removes cytokines and various pathological substances. Therefore, PE may be a more reasonable additional therapy for intractable CADM-ILD.
Keyphrases
- interstitial lung disease
- systemic sclerosis
- rheumatoid arthritis
- mass spectrometry
- idiopathic pulmonary fibrosis
- ms ms
- end stage renal disease
- newly diagnosed
- ejection fraction
- stem cells
- gene expression
- peritoneal dialysis
- prognostic factors
- multiple sclerosis
- systemic lupus erythematosus
- cell death
- cell therapy
- mesenchymal stem cells
- patient reported outcomes
- copy number
- breast cancer cells
- signaling pathway