Acute digital ischemia: A rare presentation of antisynthetase syndrome

Chan, Jesse C.; Palakodeti, Sandeep; Koster, Matthew J.

doi:10.5152/eurjrheum.2017.16077

Cited by 10 publications

(20 citation statements)

References 12 publications

(6 reference statements)

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“…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.…”

Section: Discussionmentioning

confidence: 83%

“…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).…”

Section: Cases Identified By Slrmentioning

confidence: 99%

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

Yoshida

Gono

Okazaki

et al. 2022

Journal of Scleroderma and Related Disorders

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Objective: Severe digital ischemia, including digital ulcers and gangrene, is considered rare in patients with antisynthetase antibodies. This study aimed to elucidate the clinical features of antisynthetase-positive patients complicated with digital ulcers and/or gangrene using a systematic literature review and case series in a single-center cohort. Methods: A systematic literature review was conducted to identify reports describing antisynthetase-positive cases with digital ulcers and/or gangrene. Our cohort of consecutive patients with antisynthetase antibodies was stratified by the history of severe digital ischemia. Demographic and clinical features and outcomes in patients with severe digital ischemia identified in the systematic literature review and our cohort were compared with those in patients without severe digital ischemia in our cohort. Results: The systematic literature review revealed 12 antisynthetase-positive patients with severe digital ischemia from one case series and eight case reports. Seven (7%) of 100 patients with antisynthetase antibodies in our cohort had a record of severe digital ischemia. Severe digital ischemia was often found at presentation and was associated with the classification of systemic sclerosis with or without myositis overlap. Clinical features associated with severe digital ischemia in antisynthetase-positive patients included Raynaud’s phenomenon ( p < 0.001), digital pitting scars ( p = 0.001), and nailfold capillary abnormality ( p = 0.02). Outcomes of severe digital ischemia were generally favorable with vasodilators. Conclusion: Severe digital ischemia is an overlooked complication in antisynthetase-positive patients. Antisynthetase antibodies should be measured in patients presenting with digital ulcers or gangrene, especially in those with systemic sclerosis phenotype and features associated with antisynthetase antibodies in the absence of systemic sclerosis-specific autoantibodies.

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Section: Discussionmentioning

confidence: 83%

Section: Cases Identified By Slrmentioning

confidence: 99%

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

Yoshida

Gono

Okazaki

et al. 2022

Journal of Scleroderma and Related Disorders

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“…Zöller et al have described autoimmune diseases as hypercoagulable disorders, which is attributed to their inflammatory role in cytokine cascade-mediated upregulation of pro-coagulants, inhibition of the protein C system and inhibition of fibrinolysis and the development of autoantibodies [ 12 ]. Three cases of the extra-ocular vaso-occlusive disease in ASS have been reported in the literature: a case of vaso-occlusive digital ischaemia in a 61-year-old Caucasian lady with anti-PL7 antibodies [ 13 ]; a case of pulmonary vaso-occlusive disease in a 55-year-old male with anti-Jo-1 antibodies [ 14 ]; and a case of a concurrent stroke at the time of ASS diagnosis in a 52-year-old Southeast Asian lady with anti-Jo-1 antibodies [ 15 ]. Interestingly, the patient in the first case developed vaso-occlusive disease prior to ILD symptoms, similar to our case, indicating that vaso-occlusive disease may be a precursor to severe ILD in patients with anti-PL7 antibodies.…”

Section: Discussionmentioning

confidence: 99%

Branch Retinal Vein Occlusion as a Rare Presentation of Antisynthetase Syndrome

Parida

Tsaousis

Deol

et al. 2021

Cureus

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A 52-year-old woman developed branch retinal vein occlusion (BRVO) in her right eye, resulting in blurred vision with visual acuities of 6/9 and 6/6-2 in the affected and unaffected eye respectively (Snellen). The patient was successfully treated with a course of eight intravitreal aflibercept injections, improving binocular visual acuity to 6/6. During the course of her ocular management, she was admitted for acute dyspnoea secondary to interstitial lung disease (ILD). The patient was diagnosed with the antisynthetase syndrome (ASS), testing positive for PL-7 auto-antibodies. ASS may have a systemic association with BRVO; although ASS is a rare condition, it should be suspected and investigated in patients with risk factors, particularly if they present with symptoms of ILD. Early ocular intervention is associated with excellent visual outcomes, and prompt diagnosis and treatment of ASS may potentially reduce risks of further retinal vaso-occlusive episodes.

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“…This conclusion arose because the condition of the impaired regions successfully improved after immunosuppressive treatments. As for now, five patients with digital ulcers, necrosis, or gangrene who were positive for anti‐ARS have been reported 5–9 . These are some of the most serious complications of impaired peripheral circulation because they can be irreversible and may require surgical intervention such as amputation if proper early treatment were not performed.…”

Section: Introductionmentioning

confidence: 99%

“…As for now, five patients with digital ulcers, necrosis, or gangrene who were positive for anti-ARS have been reported. [5][6][7][8][9] These are some of the…”

mentioning

confidence: 99%

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

Suma

Yoshida

Sugimoto

et al. 2021

The Journal of Dermatology

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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.

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Acute digital ischemia: A rare presentation of antisynthetase syndrome

Cited by 10 publications

References 12 publications

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

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

Branch Retinal Vein Occlusion as a Rare Presentation of Antisynthetase Syndrome

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

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