ABSTRACT
The clinical presentation of dermatomyositis (DM) is diverse, with varied phenotypes that may be correlated with specific autoantibodies. The anti-melanoma differentiation-associated gene 5 (MDA5) antibody in DM is associated with an amyopathic phenotype of DM, with several unusual cutaneous manifestation and increased risk for rapidly progressive interstitial lung disease. Initial presentation may be subtle, but early diagnosis is key to initiation of proper immunosuppressive therapy. In this report, we describe perinasal edema and erythema as a presenting complaint of anti-MDA5 DM in an otherwise healthy 40-year-old woman. The edema began shortly after heavy sun exposure and was followed by painful papules in her hands and arthritis within a few weeks. She was found to have high titer of anti-CCP and anti-MDA5, and thus was diagnosed with DM and rheumatoid arthritis overlap. A CT chest, abdomen, and pelvis showed patchy ground-glass and interstitial opacities in bilateral lower lobes consistent with mild interstitial lung disease without evidence of malignancy. Perinasal cutaneous findings and arthralgias improved with initiation of prednisone. To our knowledge, this is the first report of perinasal edema as a presenting symptom for DM and should raise suspicion for MDA-5 disease.
ABSTRACT
BACKGROUND: People with autoimmune or inflammatory conditions taking immunomodulatory/suppressive medications may have higher risk of novel coronavirus disease 2019 (COVID-19). Chronic disease care has also changed for many patients, with uncertain downstream consequences. METHODS: We included participants with autoimmune or inflammatory conditions followed by specialists at Johns Hopkins. Participants completed periodic surveys querying comorbidities, disease-modifying medications, exposures, COVID-19 testing and outcomes, social behaviors, and disruptions to healthcare. We assessed whether COVID-19 risk is higher among those on immunomodulating or suppressive agents and characterized pandemic-associated changes to care and mental health. RESULTS: In total, 265 (5.6%) developed COVID-19 over 9 months of follow-up (April-December 2020). Patient characteristics (age, race, comorbidity, medications) were associated with differences in social distancing behaviors during the pandemic. Glucocorticoid exposure was associated with higher odds of COVID-19 in models incorporating behavior and other potential confounders (odds ratio [OR]: 1.43; 95% confidence interval [CI]: 1.08, 1.89). Other medication classes were not associated with COVID-19 risk. Diabetes (OR: 1.72; 95% CI: 1.08, 2.73), cardiovascular disease (OR: 1.68; 95% CI: 1.24, 2.28), and kidney disease (OR: 1.76; 95% CI: 1.04, 2.97) were associated with higher odds of COVID-19. Of the 2156 reporting pre-pandemic utilization of infusion, mental health or rehabilitative services, 975 (45.2%) reported disruptions therein, which disproportionately affected individuals experiencing changes to employment or income. CONCLUSIONS: Glucocorticoid exposure may increase risk of COVID-19 in people with autoimmune or inflammatory conditions. Disruption to healthcare and related services was common. Those with pandemic-related reduced income may be most vulnerable to care disruptions.