Rapidly Progressive Multiple Digital Gangrene and Diffuse Alveolar Damage in a Patient With Antisynthetase Antibody and Gastric Cancer

Fukue, Ryosuke; Gono, Takahisa; Hayashi, Hideharu; Terasaki, Yasuhiro; Kuwana, Masataka

doi:10.1097/rhu.0000000000000974

Cited by 2 publications

(4 citation statements)

References 14 publications

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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: 80%

“…This case has been reported previously. 13 A 60-year-old male patient was admitted to an emergency room because of rapidly progressive ILD and multiple digital gangrenes. The possibility that low cardiac output or severe hypoxia played as provoking factors for severe digital ischemia could not be excluded.…”

Section: Resultsmentioning

confidence: 99%

“…Among those, one case series including 4 patients, and 6 case reports fulfilled the inclusion criteria. One case was previously reported by our institution, 13 thus was excluded from SLR cases and included in the cohort cases. Three additional case reports were identified from the reference lists.…”

Section: Resultsmentioning

confidence: 99%

“…4 Nevertheless, we have recently observed several patients with antisynthetase antibodies who developed severe digital ischemia, including a case already reported in the literature. 13 The prevalence, associated clinical features, and outcomes of severe digital ischemia in antisynthetase-positive patients remain unclear. Therefore, we investigated clinical features of patients with antisynthetase antibodies accompanied by severe digital ischemia through a comprehensive literature review and case series in our single-center cohort.…”

Section: Introductionmentioning

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: 80%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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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Anti‐PL‐12 anti‐synthetase syndrome manifesting with multiple digital ischemia: Case report & review of the literature

Doumeth,

Petrinec,

Chaudhary

et al. 2024

Clinical Case Reports

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Key Clinical MessageAcute digital ischemia is a rare manifestation of anti‐synthetase syndrome in the absence of Raynaud's phenomenon. A high index of suspicion may result in early diagnosis and better clinical outcomes.AbstractA 61‐year‐old male patient was admitted to the hospital for worsening arthralgias with morning stiffness lasting hours, as well as left sided headaches, and jaw pain while eating. He had significant weight loss and subjective fever at home. Multiple fingers and toes were noted to be ischemic. His laboratory workup was pertinent for significantly elevated inflammatory markers, and mild Creatinine kinase elevation. Chest imaging and later lung biopsy were notable for organizing pneumonia. Conventional angiogram showed evidence of significant digital disease without collaterals. Subsequent autoimmune screening tests with extended myositis‐specific and myositis‐associated panels revealed a strongly positive anti‐PL‐12 antibody and moderately positive anti‐ SSA‐52KD IgG ab. After ruling out infectious etiologies and malignancy, anti‐synthetase syndrome (ASyS) diagnosis was considered in the presence of ischemic digits, organizing pneumonia, polyarthralgia, constitutional symptoms, increased inflammatory markers and positive antibodies. The patient was treated with high dose prednisone and mycophenolate mofetil along with amlodipine and sildenafil for digital vasodilation. Acute digital ischemia may be the first manifestation of ASyS with ILD. A high index of suspicion is warranted for early diagnosis and better outcomes.

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Rapidly Progressive Multiple Digital Gangrene and Diffuse Alveolar Damage in a Patient With Antisynthetase Antibody and Gastric Cancer

Cited by 2 publications

References 14 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

Anti‐PL‐12 anti‐synthetase syndrome manifesting with multiple digital ischemia: Case report & review of the literature

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