Autoimmune connective tissue diseases in the COVID-19 pandemic

Abstract

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.

Fig. 1

Generalized acute cutaneous lupus erythematosus. (A) Diffuse and patchy periungual erythema, maculopapules and punctiform vasculitic scars on the tips of the toes in a patient with systemic lupus erythematosus (SLE); these may closely resemble the pseudo-chilblain lesions associated with COVID-19. (B) A positive lupus band test, performed by direct immunofluorescence on nonlesional unexposed skin, is highly diagnostic of SLE.

Fig. 2

Livedo reticularis–like eruption is commonly seen in systemic lupus erythematosus, but it is a cutaneous manifestation of COVID-19 as well.

Fig. 3

Cutaneous necrosis is noted in severe systemic lupus erythematosus, dermatomyositis, and vasculitis, as well as in vasculopathies and COVID-19 infection.