Table 1.
Comparison between COVID-19, anti-MDA5 dermatomyositis and classic dermatomyositis.
COVID-19 | Anti-MDA5 dermatomyositis with ILD | Classic dermatomyositis with ILD | ||
---|---|---|---|---|
Epidemiology | Prevalence | More than two million cases globally | Rare | Rare |
Geographic clusters | First reports in China, (now in all continents) | Mainly reported in east Asia | None | |
Sex predominance | None | None | Female predominance | |
Natural history | Severe and rapidly progressive disease in about 20% of cases | Rapidly progressive | Slowly progressive | |
Recurrence | Unknown | Rare | Relapsing-remitting | |
Mortality rate | High | Very high | High | |
Pathogenesis | Association with viral infection | Proven association with SARS-CoV-2 infection | Possible trigger of picoRNA- or other viruses | Debated triggering role of viruses |
Inflammatory state | High grade systemic inflammation | High grade systemic inflammation | Low-moderate grade systemic inflammation | |
Prothrombotic state and endothelial dysfunction | Hallmark of the disease | Hallmark of the disease | Hallmark of the disease | |
Autoantibody mediated injury | Possible cross-reactivity of induced antibodies | Postulated direct role of anti-MDA5 | Debated direct pathogenetic role | |
Lung histopathology | DAD and microangiopathy | DAD and microangiopathy | NSIP and OP | |
Clinical manifestations | Lung disease | Almost always present | Almost always present | Common |
Myositis | Mild-absent | Mild-absent | Almost always present | |
Skin and peripheral vascular involvement | Common | Almost always present | Almost always present | |
Fever | Almost always present | Very common | Uncommon | |
Association with cancer | Absent | Rare | Possible | |
Diagnosis and monitoring | CK | Mild-moderate high | Mild-moderate high | Very high |
Ferritin | High | High | Normal or slightly increased | |
Lymphocytes | Commonly low | Occasionally low | Occasionally low | |
CRP | Very high | Very high | Usually normal | |
ESR | High | High | High | |
Antinuclear Antibodies | Unknown | Negative | Usually positive | |
Antiphospholipid antibodies | Possibly positive | Possibly positive | Possibly positive | |
CT scan of the chest | Bilateral GGO or consolidation in posterior and peripheral lungs | Bilateral GGO or consolidation in posterior and peripheral lungs | Bilateral peribronchovascular GGO or consolidation | |
Nailfold capillaroscopy | Unknown | Enlarged capillaries, hemorragias, neovascularization | Enlarged capillaries, hemorragias, neovascularization | |
Treatment | Corticosteroids | Under investigation | Commonly used | Commonly used |
Anti-IL6 | Under investigation | Unknown efficacy | Unknown efficacy | |
Anti-IL1 | Under investigation | Unknown efficacy | Unknown efficacy | |
JAK-inhibitors | Under investigation | Under investigation | Under investigation | |
Anti-CD20 | Not suitable | Rescue therapy | Rescue therapy |
ILD interstitial lung disease, DAD diffuse alveolar damage, NSIP nonspecific interstitial pneumonia, OP organizing pneumonia, CK creatine kinase, CRP C reactive protein, ESR erythrocyte sedimentation rate, CT computed tomography, GGO ground glass opacities, IL interleukin, JAK Janus kinase.