Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
Annals of Intensive Care
Published in: Annals of Intensive Care 1/2018

Open Access 01-12-2018 | Research

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

Authors: Constance Vuillard, Marc Pineton de Chambrun, Nicolas de Prost, Claude Guérin, Matthieu Schmidt, Auguste Dargent, Jean-Pierre Quenot, Sébastien Préau, Geoffrey Ledoux, Mathilde Neuville, Guillaume Voiriot, Muriel Fartoukh, Rémi Coudroy, Guillaume Dumas, Eric Maury, Nicolas Terzi, Yacine Tandjaoui-Lambiotte, Francis Schneider, Maximilien Grall, Emmanuel Guérot, Romaric Larcher, Sylvie Ricome, Raphaël Le Mao, Gwenhaël Colin, Christophe Guitton, Lara Zafrani, Elise Morawiec, Marie Dubert, Olivier Pajot, Hervé Mentec, Gaëtan Plantefève, Damien Contou

Published in: Annals of Intensive Care | Issue 1/2018

Login to get access

Abstract

Background

Anti-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.

Results

From 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).

Conclusions

Intensivists 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.
Literature
1.
Papazian L, Doddoli C, Chetaille B, Gernez Y, Thirion X, Roch A, et al. A contributive result of open-lung biopsy improves survival in acute respiratory distress syndrome patients. Crit Care Med. 2007;35:755–62.CrossRefPubMed
2.
Vincent JL, Santacruz C. Do we need ARDS? Intensive Care Med. 2012;42:282–3.CrossRef
3.
Gibelin A, Parrot A, Maitre B, Brun-Buisson C, Mekontso Dessap A, Fartoukh M, et al. Acute respiratory distress syndrome mimickers lacking common risk factors of the Berlin definition. Intensive Care Med. 2015;42:164–72.CrossRefPubMed
4.
de Prost N, Pham T, Carteaux G, Mekontso Dessap A, Brun-Buisson C, Fan E, et al. Etiologies, diagnostic work-up and outcomes of acute respiratory distress syndrome with no common risk factor: a prospective multicenter study. Ann Intensive Care. 2017;7:69.CrossRefPubMedPubMedCentral
5.
Ranieri VM, Rubenfeld GD, Thompson BT, Ferguson ND, Caldwell E, Fan E, et al. Acute respiratory distress syndrome: the Berlin definition. JAMA. 2012;307:2526–33.PubMed
6.
Papazian L, Calfee CS, Chiumello D, Luyt CE, Meyer NJ, Sekiguchi H, et al. Diagnostic workup for ARDS patients. Intensive Care Med. 2016;42:674–85.CrossRefPubMed
7.
Bellani G, Laffey JG, Pham T, Fan E, Brochard L, Esteban A, et al. Epidemiology, patterns of care, and mortality for patients with acute respiratory distress syndrome in intensive care units in 50 countries. JAMA. 2016;315:788–800.CrossRefPubMed
8.
Hall JC, Casciola-Rosen L, Samedy LA, Werner J, Owoyemi K, Danoff SK, et al. Anti-melanoma differentiation-associated protein 5-associated dermatomyositis: expanding the clinical spectrum. Arthritis Care Res (Hoboken). 2013;65:1307–15.CrossRef
9.
Tillie-Leblond I, Wislez M, Valeyre D, Crestani B, Rabbat A, Israel-Biet D, et al. Interstitial lung disease and anti-Jo-1 antibodies: difference between acute and gradual onset. Thorax. 2008;63:53–9.CrossRefPubMed
10.
Uzunhan Y, Nunes H, Leroux G, Miyara M, Benveniste O, Allenbach Y. Dermato-pulmonary syndrome associated with MDA-5 antibodies. Eur Respir J. 2016;48(suppl 60):2138.
11.
Chaisson NF, Paik J, Orbai AM, Casciola-Rosen L, Fiorentino D, Danoff S, et al. A novel dermato-pulmonary syndrome associated with MDA-5 antibodies: report of 2 cases and review of the literature. Medicine (Baltimore). 2012;91:220–8.CrossRef
12.
Grasselli G, Vergnano B, Pozzi MR, Sala V, D’Andrea G, Scaravilli V, et al. Interstitial pneumonia with autoimmune features: an additional risk factor for ARDS? Ann Intensive Care. 2017;7:98.CrossRefPubMedPubMedCentral
13.
Bizien N, Renault A, Boles JM, Delluc A. Acute interstitial lung disease revealing antisynthetase syndrome. Rev Pneumol Clin. 2011;67:367–70.CrossRefPubMed
14.
Guglielmi S, Merz TM, Gugger M, Suter C, Nicod LP. Acute respiratory distress syndrome secondary to antisynthetase syndrome is reversible with tacrolimus. Eur Respir J. 2008;31:213–7.CrossRefPubMed
15.
Kim SH, Park IN. Acute respiratory distress syndrome as the initial clinical manifestation of an antisynthetase syndrome. Tuberc Respir Dis (Seoul). 2016;79:188–92.CrossRef
16.
Clawson K, Oddis CV. Adult respiratory distress syndrome in polymyositis patients with the anti-Jo-1 antibody. Arthritis Rheum. 1995;38:1519–23.CrossRefPubMed
17.
Piroddi IM, Ferraioli G, Barlascini C, Castagneto C, Nicolini A. Severe respiratory failure as a presenting feature of an interstitial lung disease associated with anti-synthetase syndrome (ASS). Respir Investig. 2016;54:284–8.CrossRefPubMed
18.
Friedman AW, Targoff IN, Arnett FC. Interstitial lung disease with autoantibodies against aminoacyl-tRNA synthetases in the absence of clinically apparent myositis. Semin Arthritis Rheum. 1996;26:459–67.CrossRefPubMed
19.
Shi J, Li S, Yang H, Zhang Y, Peng Q, Lu X, et al. Clinical profiles and prognosis of patients with distinct antisynthetase autoantibodies. J Rheumatol. 2017;44:1051–7.CrossRefPubMed
20.
Kinebuchi S, Mizuno K, Moriyama H, Ooi H, Hasegawa T, Yoshizawa H, et al. Two cases of interstitial pneumonia with anti-Jo-1 antibodies in the absence of myositis. Nihon Kokyuki Gakkai Zasshi. 2003;41:739–45.PubMed
21.
Sauty A, Rochat T, Schoch OD, Hamacher J, Kurt AM, Dayer JM, et al. Pulmonary fibrosis with predominant CD8 lymphocytic alveolitis and anti-Jo-1 antibodies. Eur Respir J. 1997;10:2907–12.CrossRefPubMed
22.
Hervier B, Wallaert B, Hachulla E, Adoue D, Lauque D, Audrain M, et al. Clinical manifestations of anti-synthetase syndrome positive for anti-alanyl-tRNA synthetase (anti-PL12) antibodies: a retrospective study of 17 cases. Rheumatology (Oxford). 2010;49:972–6.CrossRef
23.
Vincent JL, Moreno R, Takala J, Willatts S, De Mendonca A, Bruining H, et al. The SOFA (Sepsis-related Organ Failure Assessment) score to describe organ dysfunction/failure. On behalf of the Working Group on Sepsis-Related Problems of the European Society of Intensive Care Medicine. Intensive Care Med. 1996;22:707–10.CrossRefPubMed
24.
Le Gall JR, Lemeshow S, Saulnier F. A new simplified acute physiology score (SAPS II) based on a European/North American multicenter study. JAMA. 1993;270:2957–63.CrossRefPubMed
25.
Wettstein RB, Shelledy DC, Peters JI. Delivered oxygen concentrations using low-flow and high-flow nasal cannulas. Respir Care. 2005;50:604–9.PubMed
26.
Coudroy R, Frat JP, Boissier F, Contou D, Robert R, Thille AW. Early identification of acute respiratory distress disorder in the absence of positive pressure ventilation: implications for revision of the berlin criteria for acute respiratory distress syndrome. Crit Care Med. 2017;46:540–6.CrossRef
27.
Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest. 2010;138:1464–74.CrossRefPubMed
28.
Imbert-Masseau A, Hamidou M, Agard C, Grolleau JY, Cherin P. Antisynthetase syndrome. Joint Bone Spine. 2003;70:161–8.CrossRefPubMed
29.
Schmidt WA, Wetzel W, Friedlander R, Lange R, Sorensen HF, Lichey HJ, et al. Clinical and serological aspects of patients with anti-Jo-1 antibodies—an evolving spectrum of disease manifestations. Clin Rheumatol. 2000;19:371–7.CrossRefPubMed
30.
Hengstman GJ, van Engelen BG, van Venrooij WJ. Myositis specific autoantibodies: changing insights in pathophysiology and clinical associations. Curr Opin Rheumatol. 2004;16:692–9.PubMed
31.
Marguerie C, Bunn CC, Beynon HL, Bernstein RM, Hughes JM, So AK, et al. Polymyositis, pulmonary fibrosis and autoantibodies to aminoacyl-tRNA synthetase enzymes. Q J Med. 1990;77:1019–38.CrossRefPubMed
32.
Marie I, Josse S, Decaux O, Diot E, Landron C, Roblot P, et al. Clinical manifestations and outcome of anti-PL7 positive patients with antisynthetase syndrome. Eur J Intern Med. 2013;24:474–9.CrossRefPubMed
33.
Vazquez-Abad D, Rothfield NF. Sensitivity and specificity of anti-Jo-1 antibodies in autoimmune diseases with myositis. Arthritis Rheum. 1996;39:292–6.CrossRefPubMed
34.
Marie I, Josse S, Decaux O, Dominique S, Diot E, Landron C, et al. Comparison of long-term outcome between anti-Jo1- and anti-PL7/PL12 positive patients with antisynthetase syndrome. Autoimmun Rev. 2012;11:739–45.CrossRefPubMed
35.
Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol. 2010;65:25–34.CrossRef
36.
Zamora AC, Hoskote SS, Abascal-Bolado B, White D, Cox CW, Ryu JH, et al. Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome. Respir Med. 2016;118:39–45.CrossRefPubMed
37.
Zafrani L, Lemiale V, Lapidus N, Lorillon G, Schlemmer B, Azoulay E. Acute respiratory failure in critically ill patients with interstitial lung disease. PLoS ONE. 2014;9:e104897.CrossRefPubMedPubMedCentral
38.
Gay SE, Kazerooni EA, Toews GB, Lynch JP 3rd, Gross BH, Cascade PN, et al. Idiopathic pulmonary fibrosis: predicting response to therapy and survival. Am J Respir Crit Care Med. 1998;157:1063–72.CrossRefPubMed
39.
Wells AU, Desai SR, Rubens MB, Goh NS, Cramer D, Nicholson AG, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med. 2003;167:962–9.CrossRefPubMed
40.
Thille AW, Esteban A, Fernandez-Segoviano P, Rodriguez JM, Aramburu JA, Penuelas O, et al. Comparison of the Berlin definition for acute respiratory distress syndrome with autopsy. Am J Respir Crit Care Med. 2013;187:761–7.CrossRefPubMed
41.
Patel SR, Karmpaliotis D, Ayas NT, Mark EJ, Wain J, Thompson BT, et al. The role of open-lung biopsy in ARDS. Chest. 2004;125:197–202.CrossRefPubMed
42.
Gerard L, Bidoul T, Castanares-Zapatero D, Wittebole X, Lacroix V, Froidure A, et al. Open lung biopsy in nonresolving acute respiratory distress syndrome commonly identifies corticosteroid-sensitive pathologies, associated with better outcome. Crit Care Med. 2018;46:907–14.CrossRefPubMedPubMedCentral
43.
Yousem SA, Gibson K, Kaminski N, Oddis CV, Ascherman DP. The pulmonary histopathologic manifestations of the anti-Jo-1 tRNA synthetase syndrome. Mod Pathol. 2010;23:874–80.CrossRefPubMed
44.
Marie I, Hachulla E, Cherin P, Dominique S, Hatron PY, Hellot MF, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002;47:614–22.CrossRefPubMed
45.
46.
Zou J, Li T, Huang X, Chen S, Guo Q, Bao C. Basiliximab may improve the survival rate of rapidly progressive interstitial pneumonia in patients with clinically amyopathic dermatomyositis with anti-MDA5 antibody. Ann Rheum Dis. 2014;73:1591–3.CrossRefPubMed
47.
Castaneda-Pomeda M, Prieto-Gonzalez S, Grau JM. Antisynthetase syndrome and malignancy: our experience. J Clin Rheumatol. 2011;17:458.CrossRefPubMed
48.
Sato S, Hirakata M, Kuwana M, Suwa A, Inada S, Mimori T, et al. Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum. 2005;52:1571–6.CrossRefPubMed
49.
Amato MB, Meade MO, Slutsky AS, Brochard L, Costa EL, Schoenfeld DA, et al. Driving pressure and survival in the acute respiratory distress syndrome. N Engl J Med. 2015;372:747–55.CrossRefPubMed
Metadata
Title
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
Authors
Constance Vuillard
Marc Pineton de Chambrun
Nicolas de Prost
Claude Guérin
Matthieu Schmidt
Auguste Dargent
Jean-Pierre Quenot
Sébastien Préau
Geoffrey Ledoux
Mathilde Neuville
Guillaume Voiriot
Muriel Fartoukh
Rémi Coudroy
Guillaume Dumas
Eric Maury
Nicolas Terzi
Yacine Tandjaoui-Lambiotte
Francis Schneider
Maximilien Grall
Emmanuel Guérot
Romaric Larcher
Sylvie Ricome
Raphaël Le Mao
Gwenhaël Colin
Christophe Guitton
Lara Zafrani
Elise Morawiec
Marie Dubert
Olivier Pajot
Hervé Mentec
Gaëtan Plantefève
Damien Contou
Publication date
01-12-2018
Publisher
Springer International Publishing
Published in
Annals of Intensive Care / Issue 1/2018
Electronic ISSN: 2110-5820
DOI
https://doi.org/10.1186/s13613-018-0433-3

Other articles of this Issue 1/2018

Annals of Intensive Care 1/2018 Go to the issue

Research

High-flow nasal oxygen versus noninvasive ventilation in adult patients with cystic fibrosis: a randomized crossover physiological study

Review

New perspectives to improve critical care benchmarking

Research

Hyperoxia toxicity in septic shock patients according to the Sepsis-3 criteria: a post hoc analysis of the HYPER2S trial

Research

Estimation of sodium and chloride storage in critically ill patients: a balance study

Review

Effectiveness of antimicrobial-coated central venous catheters for preventing catheter-related blood-stream infections with the implementation of bundles: a systematic review and network meta-analysis

Research

Mean arterial pressure and mortality in patients with distributive shock: a retrospective analysis of the MIMIC-III database