Severe respiratory failure as a presenting feature of an interstitial lung disease associated with anti-synthetase syndrome (ASS)

Piroddi, Ines Maria Grazia; Ferraioli, Gianluca; Barlascini, Cornelius; Castagneto, Corrado; Nicolini, Antonello

doi:10.1016/j.resinv.2016.01.005

Cited by 14 publications

(9 citation statements)

References 18 publications

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“…AS and aMDA-5 dermato-pulmonary syndromes may be clinically indistinguishable one from another, with almost three-quarter of patients with aMDA-5 dermato-pulmonary syndrome exhibiting the clinical attributes of the AS syndrome [ 8 ]. When ARF is the initial presentation of AS or aMDA-5 syndromes [ 9 – 11 , 13 – 17 ] or when extra-respiratory manifestations, such as muscular, cutaneous or articular signs are lacking [ 9 , 18 – 22 ], the diagnosis is challenging, especially in the intensive care unit (ICU) setting, where many other reasons of acute respiratory failure (ARF) can be discussed. To the best of knowledge, a number of case reports of ARF revealing autoimmune inflammatory myopathies have been previously reported, but an extended case series has not been published as yet.…”

Section: Introductionmentioning

confidence: 99%

Clinical features and outcome of patients with acute respiratory failure revealing anti-synthetase or anti-MDA-5 dermato-pulmonary syndrome: a French multicenter retrospective study

Vuillard

Chambrun

Prost

et al. 2018

Ann. Intensive Care

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BackgroundAnti-synthetase (AS) and dermato-pulmonary associated with anti-MDA-5 antibodies (aMDA-5) syndromes are near one of the other autoimmune inflammatory myopathies potentially responsible for severe acute interstitial lung disease. We undertook a 13-year retrospective multicenter study in 35 French ICUs in order to describe the clinical presentation and the outcome of patients admitted to the ICU for acute respiratory failure (ARF) revealing AS or aMDA-5 syndromes.ResultsFrom 2005 to 2017, 47 patients (23 males; median age 60 [1st–3rd quartiles 52–69] years, no comorbidity 85%) were admitted to the ICU for ARF revealing AS (n = 28, 60%) or aMDA-5 (n = 19, 40%) syndromes. Muscular, articular and cutaneous manifestations occurred in 11 patients (23%), 14 (30%) and 20 (43%) patients, respectively. Seventeen of them (36%) had no extra-pulmonary manifestations. C-reactive protein was increased (139 [40–208] mg/L), whereas procalcitonine was not (0.30 [0.12–0.56] ng/mL). Proportion of patients with creatine kinase ≥ 2N was 20% (n = 9/47). Forty-two patients (89%) had ARDS, which was severe in 86%, with a rate of 17% (n = 8/47) of extra-corporeal membrane oxygenation requirement. Proportion of patients who received corticosteroids, cyclophosphamide, rituximab, intravenous immunoglobulins and plasma exchange were 100%, 72%, 15%, 21% and 17%, respectively. ICU and hospital mortality rates were 45% (n = 21/47) and 51% (n = 24/47), respectively. Patients with aMDA-5 dermato-pulmonary syndrome had a higher hospital mortality than those with AS syndrome (n = 16/19, 84% vs. n = 8/28, 29%; p = 0.001).ConclusionsIntensivists should consider inflammatory myopathies as a cause of ARF of unknown origin. Extra-pulmonary manifestations are commonly lacking. Mortality is high, especially in aMDA-5 dermato-pulmonary syndrome.

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Section: Introductionmentioning

confidence: 99%

Clinical features and outcome of patients with acute respiratory failure revealing anti-synthetase or anti-MDA-5 dermato-pulmonary syndrome: a French multicenter retrospective study

Vuillard

Chambrun

Prost

et al. 2018

Ann. Intensive Care

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“…3 Acute respiratory failure as the first presentation in ASS is rare, but reports within the literature are increasing. 3,4 The use of ECMO in these patients is rarer still but there are published case series from Germany 5 and England 6 describing the use of ECMO in ILD with at least one patient from each series having a confirmed diagnosis of the ASS. 5,6 Once the diagnosis has been confirmed, the mainstay of treatment is immunosuppression.…”

Section: Discussionmentioning

confidence: 99%

Life-threatening respiratory failure requiring extra-corporeal membrane oxygenation secondary to the anti-synthetase syndrome

Sampson

Taylor

Dyson

et al. 2019

Journal of the Intensive Care Society

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Veno-venous extra-corporeal membrane oxygenation (VV ECMO) provides support in severe acute respiratory failure (SARF) refractory to maximal conventional ventilatory support. ECMO does not treat the lungs per se, but allows time for the underlying condition to reverse or resolve. Common indications include acute respiratory distress syndrome (ARDS) and life-threatening asthma. On occasion, rarer conditions causing respiratory failure are diagnosed during ECMO support. The anti-synthetase syndrome (ASS) comprises of a group of conditions characterised by the presence of anti-aminoacyl-tRNA-synthetase antibodies with one or more of interstitial lung disease, inflammatory myositis and/or arthritis. Mainstay of management is immunosuppression. Here, we present two patients requiring ECMO support for SARF, whose respiratory function failed to respond to usual treatment for their assumed pneumonia. Both showed a rapid improvement in respiratory function and oxygenation once immunosuppressive therapy was instigated. Further testing revealed anti-synthetase antibodies, therefore both went on to receive further immunosuppression and an ultimately good outcome. Despite life-threatening respiratory failure, VV ECMO support allowed time for stabilisation, diagnosis and treatment. Outcomes in acute inflammatory interstitial pneumonitis are improved if immunosuppressive treatment is initiated as soon as possible. Our experience with these two cases have led to an institutional change in practice to send an urgent auto-antibody screen (including extractable nuclear antigen panel) on admission for all our SARF patients.

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“…AS is an autoimmune condition, characterized by antibodies directed against an aminoacyl-tRNA synthetase, along with clinical features that can also include ILD, which is more prevalent and severe in patients with AS, compared to other inflammatory myopathies 9,10 . IgG4-RD is a chronic fibroinflammatory condition characterized by infiltrative fibrosis and lymphoplasmacytic infiltrates, involving 1 or several anatomic sites and often, but not always, with an elevated IgG4 serum level 8 .…”

Section: Discussionmentioning

confidence: 99%

“…A previous consensus statement suggested using the following major histopathologic features to diagnose IgG4-RD: (1) dense lymphoplasmacytic infiltrate; (2) fibrosis, arranged at least focally in a storiform pattern; and (3) obliterative phlebitis 5 . Compared with other sites, pulmonary involvement of IgG4-RD is often difficult to recognize because of a relative paucity of pathognomonic features and a plethora of overlapping findings with other fibroinflammatory diseases 9,10 . Moreover, storiform fibrosis and obliterative phlebitis can be minimal or absent in the lung 8 .…”

Section: Discussionmentioning

confidence: 99%

Rheumatological Assessment Is Important for Interstitial Lung Disease Diagnosis

et al. 2018

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Severe respiratory failure as a presenting feature of an interstitial lung disease associated with anti-synthetase syndrome (ASS)

Cited by 14 publications

References 18 publications

Clinical features and outcome of patients with acute respiratory failure revealing anti-synthetase or anti-MDA-5 dermato-pulmonary syndrome: a French multicenter retrospective study

Clinical features and outcome of patients with acute respiratory failure revealing anti-synthetase or anti-MDA-5 dermato-pulmonary syndrome: a French multicenter retrospective study

Life-threatening respiratory failure requiring extra-corporeal membrane oxygenation secondary to the anti-synthetase syndrome

Rheumatological Assessment Is Important for Interstitial Lung Disease Diagnosis

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