Describing and expanding the clinical phenotype of anti-MDA5-associated rapidly progressive interstitial lung disease: case series of nine Canadian patients and literature review

Abstract

In an inpatient setting, RPILD associated with characteristic skin rashes, profound weight loss, articular symptoms, normal or low CK with elevated ferritin, and absent fluorescence on ANA testing should alert the clinician to the possibility of MDA5-RPILD. T-cell-mediated therapies may play a role in this highly lethal condition.

239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14–16 December 2018

“…ILD is frequently rapidly progressive [46] explaining the poor survival rate of the disease (59-75%) [40 , 41] . Moreover, up to two thirds of the patients have polyarthritis [47] . Of note, even though anti-MDA5-positive DM is not associated with an increased risk of malignancy, the disease remains the most severe, in term of mortality, compared to all other forms of myositis.…”

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

“…Although our research showed that relatively lower WBC counts and CRP levels are risk factors, the magnitude of its impact on prognosis is relatively limited. Numerous studies have con rmed that elevated serum ferritin is an independent risk factor for RP-ILD and poor prognosis in IIM patients [16,17,26,[29][30][31][32]. Our results showed that the elevation of serum ferritin still brings a trend of poor prognosis in IIM patients admitted to the ICU because of acute respiratory failure, although the statistical difference is not signi cant enough.…”

Interstitial Lung Disease-Complicated Anti-MDA5 Antibody in Clinically Amyopathic Dermatomyositis Patients: Report of Two Cases With Distinct Clinical Features

“…On the other hand, plasma exchanges represent a reasonable alternative for refractory cases, though adverse events such as allergy cannot be predicted (25). Altogether, combined immunosuppressive therapies, possibly by adjunction of mycophenolate and/or tacrolimus, give promising results (26). Large prospective studies are still lacking to identify the optimal treatment in anti-MDA5 positive patients with RP-ILD, but the scarcity of such patients will most likely require the set-up of multicenter clinical trials.…”