New-onset dermatomyositis following SARS-CoV-2 infection and vaccination: a case-based review

Abstract

Dermatomyositis is a rare, type I interferon-driven autoimmune disease, which can affect muscle, skin and internal organs (especially the pulmonary system). In 2021, we have noted an increase in new-onset dermatomyositis compared to the years before the SARS-CoV-2 pandemic in our center. We present four cases of new-onset NXP2 and/or MDA5 positive dermatomyositis shortly after SARS-CoV-2 infection or vaccination. Three cases occurred within days after vaccination with Comirnaty and one case after SARS-CoV-2 infection. All patients required intensive immunosuppressive treatment. MDA5 antibodies could be detected in three patients and NXP2 antibodies were found in two patients (one patient was positive for both antibodies). In this case-based systematic review, we further analyze and discuss the literature on SARS-CoV-2 and associated dermatomyositis. In the literature, sixteen reports (with a total of seventeen patients) of new-onset dermatomyositis in association with a SARS-CoV-2 infection or vaccination were identified. Ten cases occurred after infection and seven after vaccination. All vaccination-associated cases were seen in mRNA vaccines. The reported antibodies included for instance MDA5, NXP2, Mi-2 and TIF1γ. The reviewed literature and our cases suggest that SARS-CoV-2 infection and vaccination may be considered as a potential trigger of interferon-pathway. Consequently, this might serve as a stimulus for the production of dermatomyositis-specific autoantibodies like MDA5 and NXP2 which are closely related to viral defense or viral RNA interaction supporting the concept of infection and vaccination associated dermatomyositis.

A systematic review of the incidence, management and prognosis of new-onset autoimmune connective tissue diseases after COVID-19

“…Mild thoracic, abdominal, and pelvic lymphadenopathy, non-specific. Mild mesenteric stranding may be secondary to mesenteric panniculitis, with possible pancreatitis Proteinuria Holzer et al 2022 [ 19 ] 14 – < 5 – – CK 19,647 Positive ANA: 1:640 Positive anti-NXP2 MRI muscle: Bilateral myositis of muscles of the pelvic hip girdle and thighs Giuggioli et al 2022 [ 41 ] 28 – 65 – – Positive ANA, anticentromere pattern Nailfold capillaroscopy: “early scleroderma pattern” Chandra and Kahaleh 2022 [ 42 ] – – – – Positive ANA > 1:1280 HRCT chest: interstitial lung disease, findings suggestive of non-specific interstitial pneumonia Bouchard Marmen et al 2022 [ 43 ] 28 – 61 – – CK 7696, Positive ANA and anti-Jo-1 CT thorax: missed opacities with sub segmental consolidation MRI: oedema of the gluteal and thigh muscles consistent with myositis Blum et al 2022 [ 44 ] 91 – – – – Positive ANA Positive anti-RNP and ani-SSA antibodies CT chest: ground-glass opacities possibility indicating interstitial lung disease Assar et al 2022 [ 32 ] 18 53 …”

“…CTD symptoms onset time also varied markedly, ranging from 14 to 112 days since COVID-19 diagnosis. Autoantibody serology also varied, with just three cases reporting positive ANA [ 19 – 21 ] and six reporting positivity for other autoantibodies, including NXP2, Mi2, Ku, and Ro [ 19 – 24 ]. In three cases with negative autoantibody serology, a diagnosis of myositis was made based on MRI muscle imaging, skin biopsy histology (consistent with dermatomyositis) and electromyography findings [ 23 , 25 – 27 ].…”

“…Subsequent hydroxychloroquine 400 mg/day and prednisolone 1 mg/kg/day (dose not specified) Yes Yes Survived Kazzi et al 2022 [ 31 ] Low None SLE No Antibiotics, corticosteroids and MMF 1500 mg twice daily with resolution. Subsequent hydroxychloroquine (dose unspecified) No Yes Survived Holzer et al 2022 [ 19 ] Unknown Unknown Dermatomyositis Unknown Corticosteroids, IVIG, MMF, ciclosporin A, tofacitinib, rituximab No Yes Survived Giuggioli et al 2022 [ 41 ] Unknown Azithromycin for 5 days; hydroxychloroquine 400 mg twice a day for 1 day and then 200 mg every 12 h for 5 days Raynaud's and systemic sclerosis No Nifedipine for Raynaud’s No Yes Survived Chandra & Kahaleh 2022 [ 42 ] Unknown Unknown Systemic Sclerosis No MMF 1500 mg twice daily, amlodipine 5 mg daily, methotrexate 12.5 mg once weekly, prednisone 5 mg twice daily No No Survived Bouchard Marmen et al 2022 [ 43 ] Unknown Unknown Anti-synthetase syndrome Unknown Pulsed IV methylprednisolone, then oral prednisolone, cyclophosphamide, rituximab, IVIG Yes Yes Survived Blum et al 2022 [ 44 …”

Increased rates of idiopathic inflammatory myopathies during the COVID-19 pandemic: a single-centre experience

“…Studies regarding SARS-CoV-2-induced IIM are lacking. Review of the literature yields mostly single case reports (9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20) , with larger case series referring to SARS-CoV-2 vaccines (21,22). Certainly, higher-level evidence is still required to discern whether the incidence of IIM has increased during the pandemic and whether the disease pattern and course have changed.…”

Current concepts and advances in dermatomyositis: a dermatological perspective

“…A recent review reported 21 cases of DM occurring after either COVID-19 infection or vaccination (43). Given COVID-19 infection-associated risks, vaccination should not be avoided in this population as it is generally safe in IIMs, but clinicians must be aware of this potential sequela (44).…”