The clinical characteristics and predictors of severe digital ischemia in patients with anti‐aminoacyl transfer RNA synthetase antibodies

Abstract

Severe digital ischemia (SDI), which presents with digital ulcers, necrosis, or gangrene, has been reported to be a rare manifestation of anti‐aminoacyl transfer RNA synthetase (ARS) antibody‐positive polymyositis/dermatomyositis or anti‐synthetase syndrome. A retrospective study was conducted between 2009 and 2020 at our department to investigate the clinical features of anti‐ARS antibody‐positive patients with SDI and identify their predictors. A total of 46 patients who were positive for anti‐ARS antibody were included, four of whom (8.7%) presented with SDI. The characteristics of the patients with SDI were as follows: the median age was 74 years, with 75% being female; anti‐Jo–1 antibody, Raynaud's phenomenon, interstitial lung disease, and myositis were observed in two (50%), four (100%), four (100%), and three patients (75%), respectively. Next, we reviewed the literature of anti‐ARS antibody‐positive patients with SDI and investigated the predictors of SDI by analyzing a total of 51 patients, including the previously reported five patients with SDI. Multivariable analyses revealed that Raynaud's phenomenon and myositis independently predicted the development of SDI in patients with anti‐ARS antibody. In conclusion, digital ulcers, necrosis, or gangrene seem to be more common presentations in our study, and Raynaud's phenomenon and myositis can predict the complications of SDI in anti‐ARS antibody‐positive patients.

Severe finger necrosis in antisynthetase syndrome with positive anti‐OJ antibodies

“…Linear hemorrhage of fingernails accompanied by digital ischemia or digital ulcer in anti‐ARS syndrome has also been reported. 15 , 16 , 17 In our case, no obvious stenosis or occlusion was observed on contrast‐enhanced CT angiography, and there was no linear hemorrhage of the nails. However, we observed extreme necrosis, which was unprecedented in our experience.…”

“…Suma et al. 16 reported Jo‐1(+) anti‐ARS antibody (+) mechanic's hand, with peripheral angiography revealing occlusion of both the ulnar and radial arteries. Linear hemorrhage of fingernails accompanied by digital ischemia or digital ulcer in anti‐ARS syndrome has also been reported.…”

Severe digital ischemia as an unrecognized manifestation in patients with antisynthetase autoantibodies: Case series and systematic literature review

“…The underlying mechanisms of digital vasculopathy in antisynthetase-positive patients are largely unknown, and the contribution of SSc-related vascular remodeling, vasculitis, thromboembolisms, and comorbidities such as low cardiac output or severe hypoxia has been suggested in previous reports. 13,[36][37][38][39][40][41][42][43][44] This study suggests some similarities between severe digital ischemia observed in antisynthetasepositive patients and SSc patients. In antisynthetase-positive patients, severe digital ischemia was associated with SSc features and therapeutic response to vasodilators rather than immunosuppressive treatment.…”

“…In a recent case series from another hospital in Japan, severe digital ischemia was observed in 8.7% of 46 patients with antisynthetase antibodies. 43 Routine measurement of antisynthetase antibodies using a convenient enzyme-linked immunosorbent assay is available in clinical practice in Japan. 46 These results suggest that antisynthetase antibody-positive patients with severe digital ischemia might be overlooked in routine clinical practice.…”

“…Three additional case reports were identified from the reference lists. Finally, 12 cases with antisynthetase antibodies and severe digital ischemia were identified from one case series and eight case reports, and were included in the analysis [36][37][38][39][40][41][42][43][44] . The risk of bias by the Newcastle-Ottawa Scale was assessed as intermediate (total score: 4/9) for an article of case series 43 (Supplemental Table S2).…”