Main Article Content
Anti-SAE1 Dermatomyositis, Clinically amyopathic dermatomyositis, dermatomyositis
To date, few patients with anti-SAE1 dermatomyositis (DM) have been described, and usually in large descriptive cohort studies including all DM subtypes. Thus, it is increasingly important to further describe this rare subtype to solidify cutaneous findings, associated symptoms, and potential therapeutic options. Herein, this case series describes three patients with anti-SAE1 DM with respect to their clinical and laboratory findings, and also their response to treatment. All three patients eventually presented with classic cutaneous manifestations of dermatomyositis, however, Case 2 presented initially with cutaneous manifestations not described in the literature. Furthermore, two patients presented with dysphagia, with Case 2 experiencing a life-threatening dysphagia not typically described in the literature. Two of three patients also presented with early signs of interstitial lung disease. Lastly, cutaneous disease was only responsive to immunosuppressants in one patient. Unfortunately, this observational case-series consisted of only three patients, limiting the generalizability of our results. However, this study provides further support of the recalcitrant nature of this DM subtype, and commonly affected extracutaneous systems including the gastrointestinal and pulmonary systems. Larger studies of this patient population are needed to further confirm our associated findings.
2. Bodoki L, Nagy-Vincze M, Griger Z, Betteridge Z, Szollosi L , Danko K. Four dermatomyositis-specific autoantibodies-anti-TIF1gamma, anti-NXP2, anti-SAE and anti-MDA5-in adult and juvenile patients with idiopathic inflammatory myopathies in a Hungarian cohort. Autoimmun Rev 2014;13:1211-9.
3. Ge Y, Lu X, Shu X, Peng Q , Wang G. Clinical characteristics of anti-SAE antibodies in Chinese patients with dermatomyositis in comparison with different patient cohorts. Sci Rep 2017;7:188.
4. Muro Y, Sugiura K , Akiyama M. Low prevalence of anti-small ubiquitin-like modifier activating enzyme antibodies in dermatomyositis patients. Autoimmunity 2013;46:279-84.
5. Peterson LK, Jaskowski TD, La'ulu SL , Tebo AE. Antibodies to small ubiquitin-like modifier activating enzyme are associated with a diagnosis of dermatomyositis: results from an unselected cohort. Immunol Res 2018;66:431-6.
6. Borges IBP, Silva MG, Misse RG , Shinjo SK. Lipid-lowering agent-triggered dermatomyositis and polymyositis: a case series and literature review. Rheumatol Int 2018;38:293-301.