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

Abstract

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.

Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment

“…In these patients, the severity is evaluated clinically or with the CT-scan (showing extended OP or acute interstitial pneumonia lesions), but rarely with the pulmonary function tests, often impossible to perform. In intensive care unit, the mortality ratio reaches 50% (56). Most of the severe patients presented a RP-ILD, which is itself associated with a high mortality risk ratio, as compared to patients with chronic onset of ILD, both during aSyS and anti-MDA-5 dermatomyositis.…”

“…Patients with IM-related ILD may present with clinical symptoms, including fever (1/4), cough (1/3) or dyspnea (>1/2), which could be either related to ILD or not, especially when gastro-esophageal reflux or respiratory muscle involvement also occurs as part of the aSyS (18,19). Regarding the shortness of breath, it is immediately important to evaluate (i) the rapidity of onset, as the (sub)acute forms settling within 3 months -defining rapidly-progressive (RP)-ILD are of worse prognosis (54, 55) and (ii) the severity, as some patients require intensive care support (56). In contrast, the patients with mild ILD or in which ILD will develop later in the follow-up (1/5) can be asymptomatic, justifying careful explorations.…”

Outcomes and prognostic factors of patients with anti-melanoma differentiation-associated protein 5 and anti-aminoacyl-RNA synthetase antibodies positive idiopathic inflammatory myopathies in intensive care unit

“…Data from the largest Chinese single-center PM/DM-ILD cohort also showed that the 5-year survival rate of patients with anti-MDA5 antibody positive is also signi cantly lower than patients with anti-ARS antibodies positive [5]. For IIM patients who admitted to the ICU due to acute respiratory failure, patients with anti-MDA5 antibody positive also exhibited signi cantly higher mortality than patients with anti-ARS antibodies positive [15]. Our study consistently revealed that anti-MDA5 antibody positive is the strongest independent risk factor for poor prognosis in IIM patients admitted to the ICU because of acute respiratory failure.…”

“…Moreover, patients with DM or CADM have a higher risk of ICU mortality than those with PM [19]. In another retrospective study of IIM patients admitted to the ICU for acute respiratory failure, Gottron papules and heliotrope rash were more common in patients with anti-MDA5 antibody positive [15]. This may be the reason for higher mortality in patients with these cutaneous manifestations.…”

Die Diagnose liegt auf der Hand

“…Trotz Einsatz einer breiten und differenzierten immunsupprimierenden Therapie wird jedoch über eine hohe Letalität bis zu 84 % berichtet [18].…”