Treatment of MDA5-positive dermatomyositis complicated by gangrenous cholecystitis with tofacitinib

Abstract

Background Dermatomyositis is a rare idiopathic inflammatory disease with diverse presentations that can have varying degrees of cutaneous and systemic involvement. This phenotypic heterogeneity makes DM a therapeutic challenge. Some therapeutic drugs, such as hormones and immunosuppressants, have poor therapeutic effects. In recent years, tofacitinib has been reported to be effective in the treatment of dermatomyositis. Case presentation We report a case of anti-MDA5 antibody-positive dermatomyositis that was relieved after treatment with tofacitinib, during which gallbladder gangrene and suppurative cholecystitis occurred. After cholecystectomy, we continued to use tofacitinib and achieved a good therapeutic effect. Conclusions Tofacitinib is effective in the treatment of anti-MDA5 antibody-positive dermatomyositis, but the risk of infection is increased. It can still be used after infection control. Close follow-up should be performed during the use of tofacitinib.

Update on dermatomyositis

“…Notably, ruxolitinib mainly inhibits JAK1 and JAK2 and has moderate activity against tyrosine kinase 2 (TYK2); tofacitinib mainly inhibits JAK1 and JAK3 with less activity against JAK2 and TYK2; and baricitinib has high activity against JAK1 and JAK2, moderate activity against TYK2, and less activity against JAK3 [66, 70,71 && ]. In juvenile [72][73][74][75][76][77][78] and adult [60, patients with refractory dermatomyositis, ruxolitinib, tofacitinib, and baricitinib improved skin lesions [60,[72][73][74][75][76][77][78][79][80][81][82][83][84][85][87][88][89][90]94,97,100], muscle strength [60,[72][73][74][75][76][77][78][79][80]85,94,97], joint symptoms [72,76,77,…”

“…In juvenile [72][73][74][75][76][77][78] and adult [60, patients with refractory dermatomyositis, ruxolitinib, tofacitinib, and baricitinib improved skin lesions [60,[72][73][74][75][76][77][78][79][80][81][82][83][84][85][87][88][89][90]94,97,100], muscle strength [60,[72][73][74][75][76][77][78][79][80]85,94,97], joint symptoms [72,76,77,82,92,94,96], ILD [75]…”

“…Notably, ruxolitinib mainly inhibits JAK1 and JAK2 and has moderate activity against tyrosine kinase 2 (TYK2); tofacitinib mainly inhibits JAK1 and JAK3 with less activity against JAK2 and TYK2; and baricitinib has high activity against JAK1 and JAK2, moderate activity against TYK2, and less activity against JAK3 [66,70,71 ▪▪ ]. In juvenile [72–78] and adult [60,79–100] patients with refractory dermatomyositis, ruxolitinib, tofacitinib, and baricitinib improved skin lesions [60,72–85,87–90,94,97,100], muscle strength [60,72–80,85,94,97], joint symptoms [72,76,77,82,92,94,96], ILD [75–77,81,83,86,88,90,91,93,95], and induced hair regrowth in patients with concurrent alopecia [80,82,99]. Tofacitinib reduced calcinosis in juvenile [77,101] and adult patients [81,92,102].…”

“…In juvenile [72–78] and adult [60,79–100] patients with refractory dermatomyositis, ruxolitinib, tofacitinib, and baricitinib improved skin lesions [60,72–85,87–90,94,97,100], muscle strength [60,72–80,85,94,97], joint symptoms [72,76,77,82,92,94,96], ILD [75–77,81,83,86,88,90,91,93,95], and induced hair regrowth in patients with concurrent alopecia [80,82,99]. Tofacitinib reduced calcinosis in juvenile [77,101] and adult patients [81,92,102]. Calcinosis showed variable response to ruxolitinib [72,74] but not to baricitinib [78,103]; calcinosis progression was observed in patients treated with ruxolitinib [72].…”

Clinical Image: Nasal rim sign: A clinical manifestation of dermatomyositis

“…However, on extensive literature search of clinical images of dermatomyositis, we came across some suggestions of similar involvement in other patients with anti-MDA-5 dermatomyositis. [2][3][4] Whether or not this sign is suggestive or specific for this antibody and its prognostic implication and association with airway/lung involvement remains to be established. In one review, the authors noted 4 of 10 patients who were anti-MDA-5 positive reported tender gums and/or oral erosions, significantly more than the group that was anti-MDA5 negative.…”