key: cord-0694900-smqli7wl authors: Kogami, Masahiro; Suzuki, Satoshi; Nanjo, Yuta; Ikeda, Keigo; Tamura, Naoto; Sasaki, Shinichi; Morimoto, Shinji title: Complication of COVID-19 during remission induction therapy against anti-MDA5 antibody positive dermatomyositis date: 2020-11-17 journal: Rheumatol Adv Pract DOI: 10.1093/rap/rkaa068 sha: f2d58a110b7c9d48517a5413e63648118fbe0fd8 doc_id: 694900 cord_uid: smqli7wl nan SARS-CoV-2 infection might not be exacerbated during intensive immunosuppression, but viral clearance is delayed. Dear Editor, The coronavirus disease 2019 (COVID-19), a respiratory infection caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), originated in and spread from Wuhan city, Hubei Province, China, in December 2019. In cases with severe COVID-19, conditions similar to those of macrophage activation syndrome (MAS) and increased blood levels of inflammatory cytokines such as interleukin-6 and tumour necrosis factor alpha have been reported (1, 2) . Anti-MDA5 antibody-positive dermatomyositis is a refractory rheumatic disease, and 50%-80% of the cases develop severe rapid progressive interstitial pneumonia as a complication (3). Hyperferritinemia, a marker of MAS, is a primary pathological feature of the disease (4). In addition to high-dose steroid therapy, calcineurin inhibitor and cyclophosphamide triple immunosuppression therapy was recommended in a previous report (5) . Although this treatment has been established, the 6-month survival after diagnosis is only 75% (3). A 46-year-old woman was diagnosed with interstitial pneumonia developed as a complication of anti-MDA5 antibodypositive dermatomyositis in February 2020 ( Fig 1A) . Remission induction therapy with 3 days of steroid pulse therapy (methylprednisolone 500 mg/day) and post-therapy prednisolone (PSL) 50 mg/day + intravenous cyclophosphamide pulse therapy (IVCY) + tacrolimus 4 mg/day triple therapy was administered. Improvement of interstitial pneumonia ( Fig 1B) and decreased anti-MDA5 antibody titre was confirmed (950 → 65 INDEX; cut off 32 INDEX), and the patient was discharged after four cycles of IVCY (CY 750 mg/dose). The patient was scheduled to undergo further IVCY. Non-productive cough was noted since April 23, and the patient visited our hospital on May 6. Plain chest CT showed bilateral non-segmental diffuse ground-glass opacity ( Fig 1C) . Oxygen saturation using a 2 l nasal cannula was 96%. Blood tests at admission revealed low lymphocyte count (299.2/µl), inflammatory reaction (C-reactive protein 6.3 mg/dl), and hypercoagulability (D-dimer, 2.63 µg/ml). The ferritin level was within the reference range (160.8 ng/ml), but fluctuated at approximately 40 ng/ml before admission, suggesting a mild increase. Anti-MDA5 antibody titres were 37 INDEX and continued to show an improving trend. On day 2 of hospitalization, the patient was confirmed to be SARS-CoV-2-positive following a polymerase chain reaction https://mc.manuscriptcentral.com/rheumap (PCR) test (nasopharyngeal swab sample) and was diagnosed with COVID-19. Treatment was initiated with favipiravir, nafamostat, and inhaled ciclesonide, and continued for 14 days. On day 5 of hospitalization, oxygen therapy was discontinued. On day 16 of hospitalization, plain chest CT showed reduced findings of pneumonia (Fig 1D) . On the same day, PCR tests were initiated and performed every alternate day. According to the hospital protocol at that time, two consecutive negative nasopharyngeal swab PCR test results were necessary for discharge, because there had been two instances of the PCR test showing a positive result after a negative result, twice during the hospital stay (on hospital days 24 and 29). On day 29 of hospitalization, erythematous papules appeared on the trunk and limbs (Fig 1E, 1F) . Various virus antibody tests for cytomegalovirus, EB virus, herpes simplex virus, and herpes zoster virus showed negative results, suggesting that the eruptions were caused by COVID-19. Subsequently, the eruptions improved spontaneously. On day 43 of hospitalization, the second consecutive PCR test was negative, and the patient was discharged on day 46 (Fig 1G) . In this patient, after the onset of dermatomyositis in 2019, remission induction therapy was initiated in the early phase (the only symptom was skin lesion and slightly arthralgia); hence, the symptoms of respiratory failure, CT findings of pneumonia, and serum ferritin levels were more prominent at the time of onset of COVID-19 in 2020 (Fig 1A, 1C) . Anti- showed a relationship with delayed clearance of the viral RNA from airway secretions (8) . In our patient as well, there was a delay of 42 days for the PCR test result to turn negative. https://mc.manuscriptcentral.com/rheumap We treated a patient with anti-MDA5 antibody-positive dermatomyositis complicated with COVID-19 pneumonia during remission therapy. The patient followed a good course without signs of exacerbation of the rheumatic disease during intense immunosuppression therapy; however, the PCR results turned negative after a prolonged duration. Clinical predictors of mortality due to COVID-19 based on an analysis of data of 150 patients from Wuhan, China Clinical features of patients infected with 2019 novel coronavirus in Clinical significance and new detection system of autoantibodies in myositis with interstitial lung disease Macrophage activation syndrome in adult dermatomyositis: A case-based review Combination Therapy with Corticosteroids, Cyclosporin A, and Intravenous Pulse Cyclophosphamide for Acute/Subacute Interstitial Pneumonia in Patients with Dermatomyositis Dexamethasone in Hospitalized Patients with Covid-19 -Preliminary Report Antibody to severe acute respiratory syndrome (SARS)-associated coronavirus spike protein domain 2 cross-reacts with lung epithelial cells and causes cytotoxicity Corticosteroid Therapy for Critically Ill Patients with Middle East Respiratory Syndrome We are grateful to Ms. Kazumi Goto for secretarial assistance.Funding: No specific funding was received from any funding agency in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript. The authors have declared no conflicts of interest.https://mc.manuscriptcentral.com/rheumap