Progressive interstitial lung disease in hypomyopathic dermatomyositis in the COVID-19 pandemic: A case report.

BACKGROUND: Clinically amyopathic dermatomyositis (CADM) is characterized by typical skin lesions with no (amyopathic) or subclinical (hypomyopathic) evidence of muscle involvement. Patients with CADM may also develop rapidly progressive interstitial lung disease (ILD), and have a poor prognosis. However, the diagnosis of rapidly progressive ILD faces a challenge during the severe acute respiratory syndrome coronavirus 2 pandemic. Severe acute respiratory syndrome and ground-glass attenuation on a chest computed tomography scan are the presenting features in both conditions. CASE PRESENTATION: A 45-year-old woman with amyopathic dermatomyositis had acute onset of fever and dyspnea in February 2020. She had abnormal lung findings on CT scan. Polymerase chain reaction testing for SARS-CoV-2 was not available at that time. Chest CT revealed non-specific manifestations that could be either the signs of ILD or SARS-CoV-2 infection. Antiviral therapy was initiated with oseltamivir. Three days later, she had erythema on face, palm, and back. The ratio of lactate dehydrogenase (LDH) isoenzyme 3 to total LDH was elevated. The ratio of LDH isoenzyme 1 to total LDH was declined. Therefore, she was transferred to the rheumatology ward for further treatment. However, she died from respiratory failure 2 weeks later. CONCLUSIONS: We speculate that the altered LDH isoenzyme pattern may be an early biomarker for co-occurrence of CADM and ILD.

Management of dermatomyositis patients amidst the COVID-19 pandemic: Two case reports.

RATIONALE: In December 2019, a new epidemic of coronavirus disease 2019 (COVID-19) appeared in Wuhan, Hubei Province, and spread rapidly to other parts of China and worldwide. Although established methods exist for the diagnosis and treatment of COVID-19 infection, the management of dermatomyositis (DM) patients with COVID-19 is unknown. PATIENT CONCERNS: In this article, we describe case reports of 2 patients with DM. The first case was a 67-year-old patient with DM and infected with COVID-19 who was admitted to Leishenshan Hospital for a 1-month history of fever, cough, and expectoration. The second case was a 51-year-old male patient who was admitted to Leishenshan Hospital due to fever with cough, expectoration and shortness of breath for 1 month. DIAGNOSES: The first patient was diagnosed with COVID-19 secondary to DM based on repeated SARS-CoV-2 real-time reverse-transcriptase polymerase-chain-reaction (RT-PCR) test, detailed medical history and chest computed tomography; The second patient was diagnosed with interstitial lung disease associated with anti-MDA5 DM based on the results of antirheumatic and anti-inflammatory therapy and the above 3 methods. INTERVENTIONS AND OUTCOMES: The first patient received supportive and empirical treatment, including antiviral treatment, anti-inflammatory treatment, oxygen therapy and prophylactic anticoagulation therapy. The symptoms and laboratory results got improved after the treatments. He was discharged with thrice negative PCR tests for the SARS-CoV-2 virus. The second patient received a comprehensive treatment, including glucocorticoid and plasma exchange; his symptoms were relieved and improved. LESSONS: These cases suggest that repeated new pathogenic test results for the coronavirus and a detailed diagnosis of the medical history are important means to distinguish these diseases. Increased attention to the individual characteristics of different cases may allow for more effective diagnosis and treatment.

COVID-19 outcomes in patients with Dermatomyositis: A registry-based cohort analysis.

BACKGROUND: Patients with rheumatic diseases (RDs) like DM are known to be vulnerable towards various types of infections due to aggressive disease activity mandating high dose immunosuppressive therapy. The severity of COVID-19 in RDs is limited in literature due to the heterogeneous nature of the condition. Therefore, specific details on mortality is essential to navigate any precautions required in the treatment. OBJECTIVES: To determine outcomes of COVID-19 in DM as compared to controls, and identify the risk association of gender, race, interstitial lung disease, neoplasms, and use of immunosuppressant. METHODS: Retrospective data of individuals with DM and COVID-19 and the general population with COVID-19 between January 2020 to August 2021 was retrieved from the TriNetX database. 1:1 Propensity Score matching was used to adjust for confounders. We assessed COVID-19 outcomes such as mortality, hospitalisation, ICU admission, severe COVID-19, mechanical ventilation (MV), acute kidney injury (AKI), venous thromboembolism (VTE), ischemic stroke, acute respiratory distress syndrome (ARDS), renal replacement therapy (RRT) and sepsis. Subgroup analyses included gender, race, ILD, cancer patients, disease-modifying rheumatic drugs (DMARDs) use, and glucocorticoids (GC) use. RESULTS: We identified 5,574 DM patients with COVID-19, and 5,574 general population with COVID-19 (controls). DM with COVID-19 had a lower risk of mortality in comparison to controls [RR 0.76], hospitalisation [RR 0.8], severe COVID-19 [RR 0.76], AKI [RR 0.83], and sepsis [RR 0.73]. Males and African Americans were more likely to develop AKI [RR 1.35, 1.65], while African Americans had higher odds for severe COVID-19 [RR 1.62] and VTE [RR 1.54]. DM with ILD group also experienced higher odds for severe COVID-19 infection [RR 1.64], and VTE [RR 2.06]. DM patients receiving DMARDs and glucocorticoids had higher odds for hospitalisation [RR 1.46, 2.12], and sepsis [RR 3.25, 2.4] Subgroup analysis of 5-year neoplasm history amongst DM patients with COVID-19 was inadequate for meaningful comparison. CONCLUSION: Dermatomyositis patients without comorbities have reasonable COVID-19 outcomes including mortality and hospitalisation. Black race, male gender, ILD, DMARDS and glucocorticoid users, are associated with poor outcomes.

Treatment of MDA5-positive dermatomyositis complicated by gangrenous cholecystitis with tofacitinib.

BACKGROUND: Dermatomyositis is a rare idiopathic inflammatory disease with diverse presentations that can have varying degrees of cutaneous and systemic involvement. This phenotypic heterogeneity makes DM a therapeutic challenge. Some therapeutic drugs, such as hormones and immunosuppressants, have poor therapeutic effects. In recent years, tofacitinib has been reported to be effective in the treatment of dermatomyositis. CASE PRESENTATION: We report a case of anti-MDA5 antibody-positive dermatomyositis that was relieved after treatment with tofacitinib, during which gallbladder gangrene and suppurative cholecystitis occurred. After cholecystectomy, we continued to use tofacitinib and achieved a good therapeutic effect. CONCLUSIONS: Tofacitinib is effective in the treatment of anti-MDA5 antibody-positive dermatomyositis, but the risk of infection is increased. It can still be used after infection control. Close follow-up should be performed during the use of tofacitinib.

Autoimmune connective tissue diseases in the COVID-19 pandemic.

Autoimmune connective tissue diseases are a heterogeneous group of clinical entities sharing a common feature-an impairment of structural components like collagen and elastin, arising by autoimmune mechanisms. Because most patients are on a long-term immunosuppressive therapy, which renders them vulnerable to infections, a new challenge appears in front of physicians in the coronavirus disease 2019 (COVID-19) era. Immune mechanisms are substantial for the control and ceasing of viral infections, and their impairment may cause serious complications; however, data from immunosuppressed transplant patients do not reveal a higher frequency or diseases' severity in those infected by COVID-19. Several immunotherapies used to treat autoimmune connective tissue diseases favorably modulate the immune response of severe acute respiratory syndrome coronavirus (SARS-CoV-2)-infected patients. The present review highlights the problems of susceptibility, severity, and therapeutic options in patients with autoimmune connective tissue diseases during the COVID-19 pandemic. The relationship between autoimmune connective tissue diseases and COVID-19 infection is explained with antiviral protection genes expression, hypercytokinemia, and lymphohistiocytosis/macrophage activation mechanisms. Recommendations concerning therapy for prevention during the pandemic period or in case of concomitant COVID-19 infection are also presented. Clinical trials are ongoing regarding COVID-19 therapy blocking the cytokine response. © 2021 Elsevier Inc. All rights reserved.

Fatal outcome of anti-MDA5 juvenile dermatomyositis in a paediatric COVID-19 patient: a case report.

Anti-melanoma differentiation-associated gene 5 juvenile dermatomyositis (anti-MDA5 JDM) is associated with high risk of developing rapidly progressive interstitial lung disease (RP-ILD). Here we report an 11-year-old girl with anti-MDA5 JDM and RP-ILD which led to a fatal outcome, further aggravated by SARS-CoV-2 infection. She was referred to our hospital after being diagnosed with anti-MDA5 JDM and respiratory failure due to RP-ILD. On admission, fibrobronchoscopy with bronchoalveolar lavage (BAL) revealed Pneumocystis jirovecii infection so treatment with intravenous trimethoprim-sulfamethoxazole was initiated. Due to RP-ILD worsening, immunosuppressive therapy was intensified using methylprednisolone pulses, cyclophosphamide, tofacitinib and intravenous immunoglobulin without response. She developed severe hypoxemic respiratory failure, pneumomediastinum and pneumothorax, further complicated with severe RP-ILD and cervical subcutaneous emphysema. Three real-time RT-PCR for SARS-CoV-2 were made with a negative result. In addition, she was complicated with a secondary hemophagocytic lymphohistiocytosis and a fourth real-time PCR for SARS-CoV-2 performed in BAS sample was positive. Despite aggressive treatment of RP-ILD due to anti-MDA5 JDM, there was no improvement of respiratory failure in the following days and patient developed refractory septic shock and died. Anti-MDA5 JDM patients with RP-ILD have a poor prognosis with a high mortality rate. For this reason, intensive immunosuppressive therapy is essential including the use of promising drugs such as tofacitinib. COVID-19 in children with underlying health conditions like anti-MDA5 JDM may still be at risk for disease and severe complications.

Management strategies for dermatomyositis during the coronavirus disease 2019 outbreak.

In late 2019, the coronavirus disease 2019 (COVID-19) broke out in Wuhan and then spread over China, which greatly affected the medical practices and health care systems. With most of the hospital's outpatient services closed, the routine clinical diagnosis and treatment for patients with dermatomyositis has been disturbed. We conducted telephone follow-up for 52 patients to know the changes in the condition and the continuation of drug therapy and to ensure the continuity, safety, and effectiveness of the treatment of patients with dermatomyositis during COVID-19.

COVID-19 and rheumatic autoimmune systemic diseases: report of a large Italian patients series.

INTRODUCTION: Covid-19 infection poses a serious challenge for immune-compromised patients with inflammatory autoimmune systemic diseases. We investigated the clinical-epidemiological findings of 1641 autoimmune systemic disease Italian patients during the Covid-19 pandemic. METHOD: This observational multicenter study included 1641 unselected patients with autoimmune systemic diseases from three Italian geographical areas with different prevalence of Covid-19 [high in north (Emilia Romagna), medium in central (Tuscany), and low in south (Calabria)] by means of telephone 6-week survey. Covid-19 was classified as (1) definite diagnosis of Covid-19 disease: presence of symptomatic Covid-19 infection, confirmed by positive oral/nasopharyngeal swabs; (2) highly suspected Covid-19 disease: presence of highly suggestive symptoms, in absence of a swab test. RESULTS: A significantly higher prevalence of patients with definite diagnosis of Covid-19 disease, or with highly suspected Covid-19 disease, or both the conditions together, was observed in the whole autoimmune systemic disease series, compared to "Italian general population" (p = .030, p = .001, p = .000, respectively); and for definite + highly suspected diagnosis of Covid-19 disease, in patients with autoimmune systemic diseases of the three regions (p = .000, for all comparisons with the respective regional general population). Moreover, significantly higher prevalence of definite + highly suspected diagnosis of Covid-19 disease was found either in patients with various "connective tissue diseases" compared to "inflammatory arthritis group" (p < .000), or in patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs treatments (p = .011). CONCLUSIONS: The finding of a higher prevalence of Covid-19 in patients with autoimmune systemic diseases is particularly important, suggesting the need to develop valuable prevention/management strategies, and stimulates in-depth investigations to verify the possible interactions between Covid-19 infection and impaired immune-system of autoimmune systemic diseases. Key Points • Significantly higher prevalence of Covid-19 is observed in a large series of patients with autoimmune systemic diseases compared to the Italian general population, mainly due to patients' increased susceptibility to infections and favored by the high exposure to the virus at medical facilities before the restriction measures on individual movement. • The actual prevalence of Covid-19 in autoimmune systemic diseases may be underestimated, possibly due to the wide clinical overlapping between the two conditions, the generally mild Covid-19 disease manifestations, and the limited availability of virological testing. • Patients with "connective tissue diseases" show a significantly higher prevalence of Covid-19, possibly due to deeper immune-system impairment, with respect to "inflammatory arthritis group". • Covid-19 is more frequent in the subgroup of autoimmune systemic diseases patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs, mainly hydroxyl-chloroquine and methotrexate, which might play some protective role against the most harmful manifestations of Covid-19.