Antisynthetase syndrome and pulmonary hypertension: report of two cases and review of the literature

García-Fernández, Antía; Quezada-Loaiza, Carlos A.; Puente-Bujidos, Carlos de la

doi:10.1080/24725625.2020.1794521

Cited by 10 publications

(8 citation statements)

References 11 publications

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“…Screening for PH is important amongst ASS patients, due to the association with increased morbidity and mortality, especially for non‐Jo‐1 positive patients 4,14,15,20 . The importance of evaluation for PH in persisting dyspnoea and reduced exercise tolerance must be emphasized as targeted PH treatment may be of benefit 21,22 . This was relevant in our case, due to the presence of multiple comorbidities that impacted his exercise capacity.…”

Section: Discussionmentioning

confidence: 84%

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Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome

Chhor

Fogarty

et al. 2022

Respirology Case Reports

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We describe the first case of antisynthetase syndrome (ASS) with antibodies to anti‐glycyl tRNA synthetase (EJ‐1) with antiphospholipid syndrome (APLS). A 66‐year‐old man presented with progressive dyspnoea, fever, dry cough and proximal muscle weakness over several months on a background of cryptogenic organizing pneumonia. Examination revealed bibasal fine chest crackles, proximal muscle weakness of the upper and lower limbs, digital skin thickening and facial telangiectasias. Creatine kinase was elevated and autoimmune screening was positive for anti‐EJ‐1, anti‐beta‐2‐glycoprotein, anti‐Ro and anti‐La antibodies. Computed tomography of the chest revealed a usual interstitial pneumonia pattern and a ventilation–perfusion scan demonstrated scintigraphic evidence of bilateral pulmonary emboli. A diagnosis of ASS and APLS was made. Immunosuppressive therapy including pulsed methylprednisolone, rituximab and mycophenolate was commenced with improvement in symptoms. This case highlights the importance of evaluation for ASS in idiopathic interstitial pneumonia, and APLS in ASS patients.

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Section: Discussionmentioning

confidence: 84%

“…4,14,15,20 The importance of evaluation for PH in persisting dyspnoea and reduced exercise tolerance must be emphasized as targeted PH treatment may be of benefit. 21,22 This was relevant in our case, due to the presence of multiple comorbidities that impacted his exercise capacity.…”

Section: Discussionmentioning

confidence: 90%

Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome

Chhor

Fogarty

et al. 2022

Respirology Case Reports

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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 ].…”

Section: Discussionmentioning

confidence: 99%

“…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. This study suggested that direct vascular involvement may lead to PAH in patients with ASS; both anti-Jo-1 positive and anti-PL-12 positive cases were found to have intimal proliferation on histological analysis of pulmonary arterial muscles, further supporting this pathophysiological theory[ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary hypertension (PAH) is a life-threatening complication associated with connective tissue diseases (CTD) such as RA[ 8 ]. While most patients with CTD have relatively stable interstitial changes in their lungs during follow-up, a disproportionate number of patients with PAH have gradually emerged[ 9 ]. Although PAH is relatively common in most forms of CTD, there are currently no reports of PAH secondary to RA overlapping with ASS[ 10 ].…”

Section: Introductionmentioning

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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Prevalence of anti-synthetase antibodies among systemic sclerosis patients

Garra

Levy

2023

European Journal of Internal Medicine

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Antisynthetase syndrome and pulmonary hypertension: report of two cases and review of the literature

Cited by 10 publications

References 11 publications

Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome

Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome

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

Prevalence of anti-synthetase antibodies among systemic sclerosis patients

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