Anti‐synthetase syndrome‐associated pulmonary veno‐occlusive disease

Kay, Dana; Kadri, Ferdous; Fitzpatrick, Garrett; Alnuaimat, Hassan; Reddy, Raju; Ataya, Ali

doi:10.1177/2045894020935289

Cited by 6 publications

(3 citation statements)

References 19 publications

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“…Among patients with ASS, ILD is the main clinical feature and prognostic factor with an incidence of up to 80%, while PAH secondary to ILD is also frequently reported[ 9 ]. Approximately 7.9% of patients with ASS develop PAH[ 19 ]. It has been shown that PAH is usually severe and is believed to be related to Raynaud’s phenomenon, which is commonly associated with ASS[ 19 ].…”

Section: Discussionmentioning

confidence: 99%

“…Approximately 7.9% of patients with ASS develop PAH[ 19 ]. It has been shown that PAH is usually severe and is believed to be related to Raynaud’s phenomenon, which is commonly associated with ASS[ 19 ]. A case report and literature review by García-Fernández et al [ 9 ] explored the relationship between ASS and PAH, and revealed that while their patients had stable and mild ILD, PAH at follow-up was disproportionate and unrelated to ILD severity.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary hypertension secondary to seronegative rheumatoid arthritis overlapping antisynthetase syndrome: A case report

Huang¹,

Lu²,

Tian³

et al. 2022

WJCC

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BACKGROUND Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome (ASS) forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis (RA), particularly in patients with seronegative RA (SNRA). It is unclear whether there is an overlap between ASS and RA, or if ASS sometimes mimics RA. Pulmonary hypertension (PAH) is common in connective tissue diseases (CTDs). However, published reports on CTD-PAH do not include overlapping CTDs, and its incidence and impact on patient prognosis are unclear. CASE SUMMARY We report the case of a 63-year-old woman who presented with a 3-mo history of symptom aggravation of recurrent symmetrical joint swelling and pain that had persisted for over 10 years. The patient was diagnosed with RA and interstitial lung disease. The patient repeatedly presented to the hospital’s respiratory and rheumatology departments with arthralgia, plus shortness of breath after activity. Relevant tests indicated that anti-CCP and RF remained negative, while anti-J0-1 and anti-Ro-52 were strongly positive. It was not until recently that we recognized that this could be an unusual case of SNRA with concurrent ASS. Joint pain was relieved after regular anti-rheumatic treatment. Chest computed tomography scans showed that pulmonary interstitial changes did not progress significantly over several years; however, they showed gradual widening of the pulmonary artery, and cardiac ultrasound indicated elevated pulmonary artery systolic pressure. The prescribed treatment of PAH was not effective in improving shortness of breath. CONCLUSION Overlap of RA and ASS may be missed. Further research is necessary to facilitate early diagnosis, effective evaluation, and prognosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension secondary to seronegative rheumatoid arthritis overlapping antisynthetase syndrome: A case report

Huang¹,

Lu²,

Tian³

et al. 2022

WJCC

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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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Cutaneous manifestations of anti‐synthetase syndrome: Case series and literature review

Chan,

Osman,

Yacyshyn

et al. 2024

JEADV Clinical Practice

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BackgroundAnti‐synthetase syndrome (ASyS) is a rare inflammatory myopathy associated with anti‐aminoacyl transfer RNA synthetase antibodies, and is characterized by a triad of interstitial lung disease, arthritis, and myopathy. ASyS has been considered to represent a subtype of dermatomyositis (DM), however it is not clear whether cutaneous findings overlap in both diseases.ObjectivesWe aimed to characterize mucocutaneous features which should raise suspicion of ASyS and prompt early diagnostic testing.MethodsWe reviewed 28 cases of ASyS in our institution, and retrieved data from 43 articles reporting clinical features of ASyS in the literature.ResultsDermatologic manifestations of ASyS can be categorized as: specific cutaneous signs strongly associated with ASyS (‘Mechanic's hands’, ‘Hiker's feet’); mucocutaneous signs overlapping with other collagenoses; nailfold changes (resembling those of DM and systemic sclerosis); and mucocutaneous signs of undetermined significance. Only 20% had pathognomonic DM signs.ConclusionsDermatologic manifestations of ASyS are heterogeneous and do not always reproduce the pattern seen in DM. Presence of palmar hyperkeratosis (‘Mechanic's hands’), cuticular overgrowth, skin ulcerations and nailfold capillary changes should prompt further diagnostic workup of ASyS. Limitations of our study include inconsistent reporting of dermatologic findings within our series between virtual and in‐person visits. Not all patients were seen in dermatology consultation.

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Anti‐synthetase syndrome‐associated pulmonary veno‐occlusive disease

Cited by 6 publications

References 19 publications

Pulmonary hypertension secondary to seronegative rheumatoid arthritis overlapping antisynthetase syndrome: A case report

Pulmonary hypertension secondary to seronegative rheumatoid arthritis overlapping antisynthetase syndrome: A case report

Branch Retinal Vein Occlusion as a Rare Presentation of Antisynthetase Syndrome

Cutaneous manifestations of anti‐synthetase syndrome: Case series and literature review

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