Patients with idiopathic inflammatory myopathies suffer from poor self-reported PROMIS physical function after COVID-19 infection: an interview-based study from the MyoCite cohort

Background/Aims Patients with rheumatic diseases are known to exhibit worse outcomes with COVID-19. However, the effect on individual diseases such as idiopathic inflammatory myopathies (IIM) remain unexplored. Methods A self-report e-survey with 36 questions was pilot tested and validated to cover demographics, history of COVID-19 contact, course and severity of COVID-19 infection, influence of COVID-19 infection on physical state one-month after the infection and vaccination status. Baseline characteristics were retrieved from the MyoCite registry and biobank archive. PROMIS-10a, a validated self-report tool was used to quantify physical function. Age and gender similar healthy controls (HC) were included for comparison. Results 10 patients of IIM were compared with 40 controls. Fever was the most common symptom reported by both the groups however, fatigue, chest pain, and breathlessness were experienced more by patients of IIM compared to HC. However, severity of COVID-19 infection as substantiated by pneumonia, hospitalization, and oxygen requirement was comparable in IIM and HC, possibly due to small numbers PROMIS physical function was comparable before COVID-19 in both groups, and in HC physical function remained the same a month before and after COVID-19. On the other hand, the physical function deteriorated at 30-days after COVID-19 in the IIM group as compared with their baseline function as well as HC. Specific tasks were more challenging for IIM, including climbing stairs, bending or kneeling and shampooing hair, although the limitation was mild (Table 1). IIM patients were also less likely to be vaccinated, suggesting prevalent hesitancy in this group (50% vs 90%, p: 0.007) (Table 1). P070 Table 1: Comparison of physical health one-month after Covid-19 in IIM patients and HC and vaccination statusVariables1 month after covid-19 IIM (n-10)1 month after covid-19 HC (n-40)p-valueOverall physical health n (%) Excellent Very good Good Fair Poor1 (10%) 4 (40%) 0 3 (30%) 2 (20%)28 (70%) 5 (12.5%) 5 (12.5%) 2 (5%) 00.006 0.05 0.43 0.03 0.04Fatigue (median±IQR)4 (2-5.75)0 (0-1)Pain (median±IQR)1 (0-6.75)00.67Did your health limit you in doing vigorous activities n (%) Not at all Very little Somewhat Quite a lot Cannot do5 (50%) 1 (10%) 3 (30%) 1 (10%) 025 (62.5%) 8 (20%) 5 (12.5%) 2 (5%) 00.47 0.47 0.19 0.55 -Did your health limit you in walking more than a mile (1.6 km) n (%) Not at all Very little Somewhat Quite a lot Cannot do6 (60%) 0 1 (10%) 3 (30%) 027 (67.5%) 9 (22.5%) 2 (5%) 2 (5%) 00.65 0.21 0.55 0.03 -Did your limit you in climbing a flight of stairs n (%) Not at all Very little Somewhat Quite a lot Cannot do6 (60%) 0 3 (30%) 1 (10%) 027 (67.5%) 10 (25%) 2 (5%) 1 (2.5%) 00.65 0.18 0.03 0.31 -Did your health limit you in lifting or carrying groceries n (%) Not at all Very little Somewhat Quite a lot Cannot do7 (70%) 1 (10%) 2 (20%) 0 030 (75%) 7 (17.5%) 2 (5%) 1 (2.5%) 00.7 0.56 0.14 0.89 -Did your health limit you in bending, kneeling or stooping (%) Not at all Very little Somewhat Quite a lot Cannot do7 (70%) 0 3 (30%) 0 036 (90%) 3 (7.5%) 0 1 (2.5%) 00.12 0.004 0.02 0.89 -Were you able to do chores such as cleaning the floor or yard work n (%) Without any difficulty With a little difficulty With some difficulty With much difficulty Cannot do7 (70%) 0 1 (10%) 0 2 (20%)29 (72.5%) 6 (15%) 0 2 (5%) 3 (7.5%)0.87 0.36 0.12 0.84 0.64Were you able to dress yourself, including tying shoelaces and buttoning your clothes n (%) Without any difficulty With a little difficulty With some difficulty With much difficulty Cannot do8 (80%) 0 1 (10%) 1 (10%) 038 (95%) 2 (5%) 0 0 00.14 0.84 0.12 0.12 -Were you able to shampoo your hair n (%) Without any difficulty With a little difficulty With some difficulty With much difficulty Cannot do7 (70%) 1 (10%) 2 (20%) 0 040 (100%) 0 0 0 00.02 0.12 0.04 - -Were you able to wash and dry your body n (%) Without any difficulty With a little difficulty With some difficulty With much difficulty Cannot do9 (90%) 1 (10%) 0 0 040 (100%) 0 0 0 00 12 0.12 - - -Were you able to sit and get up from the toilet n (%) Without any difficulty With a little difficulty With some difficulty With much difficulty Cannot do8 (80%) 1 (10%) 1 (10%) 0 037 (92.5%) 1 (2.5%) 1 (2.5%) 1 (2.5%) 00.25 0.55 1 0.89 -Did you have any symptoms in the month before and after you got COVID19 n (%) None Rashes Generalized fatigue Muscle weakness Fingertip ulcers Oral ulcers Joint pain or swelling in hands Joint pain or swelling in other joints Chest pain Shortness of breath Raynaud’s Elevated muscle enzyme in blood (high creatine kinase level) Elevated inflammatory markers in blood (high ESR or CRP)4 (40%) 2 (20%) 3 (30%) 3 (30%) 1 (10%) 0 1 (10%) 1 (10%) 0 4 (40%) 0 4 (40%) 5 (50%)26 (65%) 0 7 (17.5%) 0 0 0 0 2 (5%) 0 0 0 - -0.15 0.04 0.38 0.02 0.12 - 0.12 0.55 - 0.009 – -Vaccinated n (%) Yes No5 (50%) 5 (50%)36 (90%) 4 (10%)0.007Type of vaccine n (%) Covishield Covaxin5/5 (100%) 027/36 (75%) 9/36 (25%)0.56Number of doses n (%) 1 23 (30%) 2 (20%)21 (52.5%) 19 (47.5%)0.2 0.1Received at least 1 dose before covid positive report n (%) Yes No0 10 (100%)21(52.5%) 19 (47.5%)0.03 Conclusion Patients with IIM exhibit increase in fatigue, and dyspnoea during COVID-19 although there is no increase in hospitalisation and had similar disease severity compared to HC. Short term physical function is impacted at 30-days post COVID-19, suggesting the need for closer long-term follow-up. Vaccine hesitancy is prevalent and merits addressing in patients with IIM. Disclosure R. Kharbanda: None. K. Ganatra: None. V. Agarwal: None. L. Gupta: None.

Systemic flare and cutaneous ulceration following cytomegalovirus infection in a patient with anti-melanoma differentiation-associated protein 5 (MDA5) associated myositis: Diagnostic challenge during the time of coronavirus disease (COVID-19) pandemic.

Background: Anti-melanoma differentiation-associated protein 5 (MDA5) associated idiopathic inflammatory myopathy (IIM) often manifests with minimal muscle weakness and rapidly progressive interstitial lung disease (RP-ILD) with a poor prognosis. The clinical presentation may be varied in different ethnic groups. The ongoing coronavirus disease (COVID-19) pandemic has made management even more challenging as certain manifestations may be difficult to diagnose remotely. Aim of the work: To throw light on the rare association of CMV infection in established anti-MDA5 myositis with severe consequences. Similar cases were presented and compared. Case report: A 42-year-old lady presented with heliotrope rash, periorbital edema, ulcerated Gottron's papules, proximal muscle weakness and intermittent fever of six-month duration. Anti-MDA5 antibodies were positive. Active disease, including myocarditis and RP-ILD, were challenging to diagnose on teleconsultation. Upon initiating tofacitinib, cytomegalovirus (CMV) polymerized chain reaction (PCR) came positive. Ganciclovir was started with the possibility of viral activation being the potential driving force for interferon pathway activation and dermatomyositis (DM) flare, but the patient succumbed to the illness. Conclusion: Viral triggers are known to induce autoimmune disease in the genetically predisposed. However, CMV infection in established anti-MDA5 myositis is uncommon and further association with myocarditis is a rare occurrence. Ulcerated Gottron's and periorbital oedema may carry a sinister connotation in Indians with anti-MDA5 DM, with worse manifestations such as myocarditis- which albeit rare, can be fatal.

Patient Perspectives on the Effect of the SARS-CoV-2 Pandemic on Patients With Systemic Sclerosis: An International Patient Survey.

BACKGROUND: The coronavirus disease (COVID-19) pandemic and its subsequent effects on health care systems have significantly impacted the management of chronic rheumatic diseases, including systemic sclerosis (SSc). METHODS: In this context, a 25-item anonymized e-survey was posted on the Twitter and Facebook e-groups and pages of various scleroderma organizations and patient communities to assess the problems faced by patients with SSc during the pandemic, with a focus on effects on the disease, drug procurance, continuity of medical care, and prevalent fears among patients. RESULTS: Of the 291 participants (median age of 55 [43.5-63] years, 93.8% females), limited systemic sclerosis was the most common diagnosis (42.3%). Many patients experienced problems attributable to the COVID-19 pandemic (119, 40.9%), of which 46 (38.7%) required an increase in medicines, and 12 (10.1%) of these needed hospitalizations for disease-related complications. More than one-third (36.4%) were on glucocorticoids or had underlying cardiovascular risks (39%) that would predispose them to severe COVID-19.A significant proportion (38.1%) faced hurdles in procuring medicines or experienced disruption in physiotherapy sessions (24.7%). One-quarter (24.1%) felt it was difficult to contact their specialist, whereas another 7.2% were unable to do so. Contracting COVID-19 was the most prevalent fear (71.5%), followed by infection in the family (61.9%), and a flare of the disease (45.4%). Most respondents preferred teleconsultations (55.7%) over hospital visits in the pandemic period. CONCLUSION: The results of the patient survey suggest that the COVID-19 pandemic has affected many patients with SSc and may translate to poorer outcomes in this population in the postpandemic period.