Increased Prevalence of Depression in Juvenile Dermatomyositis during the Covid-19 Pandemic

Background. Juvenile Dermatomyositis (JDM) is a chronic systemic vasculopathy of unknown etiology characterized by symmetrical proximal muscle weakness, raised serum concentration of muscle enzymes and pathognomonic skin rashes. Although JDM is the most common pediatric idiopathic inflammatory myopathy, it is still quite rare with an annual incidence of 3.2 cases per million children in the US. Youth with chronic disease are reported to have a higher prevalence of mental health disorders compared to healthy peers, with some research reporting up to a fourfold increase in risk. The COVID-19 pandemic has raised psychological distress among youth;data from the first year of the pandemic suggests that 1 in 4 youth globally are experiencing clinically elevated depression symptoms. The primary aim of this study is to describe the prevalence of depression in a cohort of patients with JDM to help providers better understand the mental health issues that arise in this population. This is critically important as early intervention for depression in youth with JDM has the potential to improve both medical and psychosocial outcomes. Methods. This was a cohort study measuring depression in youth with JDM at Children's Healthcare of Atlanta (CHOA). Subjects were recruited during routine outpatient visits to CHOA rheumatology clinics from August to December 2020. Subjects had a diagnosis of JDM for at least 3 months, were between 5-20 years old, and had no cognitive deficit precluding questionnaire completion. Parent completed a proxy questionnaire if the child was 5-7 years old. Depression was assessed using the Patient Questionnaire-9 (PHQ-9). Of 15 eligible subjects, all consented to the study. Informed consent/assent was obtained. CHOA Institutional Review Board approved the study. Upon identification of depression, an educational handout was offered, which also included mental health care providers. Identified suicide risk was addressed with immediate direct questioning of suicidal intent, plan or attempt within the prior week;endorsement of any of these prompted enactment of a safety plan and urgent psychiatric evaluation. Statistical comparisons were performed using SAS. Medians and interquartile ranges (IQR), mean and standard deviation and frequencies were calculated for demographic and disease related variables. The presence of depression symptoms were analyzed as binary covariates for positive screens on the PHQ-9. Results. Demographics of the 15 participants included 53% female, median age of 12 years (IQR 10.0, 19.0;range 5-20) with a range of 5-20 years. The sample was heterogeneous with respect to race/ethnicity, with 8 (53.3%) Black, 6 (40%) White and 1 (6.7%) Asian participant. Median disease duration was 4.1 years (IQR 2.2, 6.9). Calcinosis was present in 10 (67%) of patients. Five (33%) participants had active disease at the time of completing PHQ-9, all of whom had mild disease with median Physician Global Score of 0.6 (IQR 0, 0.9). Depression was identified in 6 subjects (40%): 5 subjects (33%) were classified as having mild depression and 1 subject (7%) was classified as having moderate depression. No subjects had severe depression nor endorsed suicidal ideation. There was no significant difference in depression prevalence in patients with active disease versus inactive disease. The prevalence of depression in this small cohort is similar to previously reported rates of depression in patients with JIA and SLE;notably, it is higher than rates of depression in healthy children in the US. Conclusions. This pilot study adds to our understanding of the relationship between JDM diagnosis and psychosocial functioning in children and youth. The COVID-19 pandemic has been associated with a rise in depression in all children. Our findings suggest that regardless of disease status, there is a higher prevalence of depression in JDM patients compared to their healthy peers. Given the small sample size, further studies are needed to assess depression in paediatric rheumatology clinics.

Calcinosis cutis in a patient with severe COVID-19 infection

Background: Atypical presentation of calcinosis cutis in the context of COVID-19 infection complicated by acute renal failure has not been described in literature. We report a case of severe COVID-19 infection and its associated uncommon skin manifestation. It is a rare condition and its association with different diseases has been established in the past. However, to the authors' knowledge, calcinosis cutis has not yet been described in relation to COVID-19 infection complicated by acute renal failure. Case Presentation: Here we describe a case of a 55-year-old gentleman admitted to the intensive care unit with severe COVID-19 infection whose hospital stay was complicated by acute renal failure and development of hypocalcemia which was treated with oral and intravenous calcium. Subsequently, he developed an atypical fleshy lesion on his left ankle during his in-patient stay which was histologically proven calcinosis cutis. It was successfully treated with topical medications. Conclusion: This case highlights the importance of considering a wide differential of skin lesions including calcinosis cutis in patients who are critically unwell with COVID-19 or any other severe infections and develop isolated skin lesions in the setting of impaired renal functions and abnormal calcium phosphate metabolism with calcium administration. [ FROM AUTHOR] Copyright of European Journal of Medical Case Reports is the property of Discover STM Publishing Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full . (Copyright applies to all s.)

A rare occurrence of tumoural calcinosis of long head biceps tendon post-COVID-19 infection

Tumoural calcinosis is a rare entity commonly caused by hyperphosphatemia due to bone mineral disease, hyperparathyroidism of chronic renal failure. However, our case demonstrated a normo-phosphatemic tumoural calcinosis post-COVID-19. This is a 36-year-old with a multiple history of soft-tissue calcification presented with acute onset severe right shoulder pain associated with anterior shoulder swelling at day 20 post-COVID-19. The clinical examination reveals anterior shoulder swelling at bicipital groove with severe restriction of range of motion due to pain. Ultrasound revealed an initial solid mass arising from the sheath of long head of biceps tendon which turns into cystic mass at week 4 of the disease. Computed tomography scan demonstrate sedimentation sign. His blood parameters revealed normo-calcemic, normo-phosphatemic bone profile, normal renal function and no sign suggestive of rheumatological disease. He was started on short course on non-steroidal anti-inflammatory drugs (NSAIDs) for 3 week and does not require surgical intervention. His symptoms completely resolved after 4 weeks with persistent shoulder swelling. He was started with prophylaxis low phosphate diet to prevent future recurrence. Our case demonstrates that conservative management using the short course of NSAIDs can be beneficial in treating primary normophosphatemic tumoural calcinosis.

Adult onset refractory nxp2 dermatomyositis following covid 19 infection

Background: Dermatomyositis is a type of systemic inflammatory autoimmune disorder characterised by muscle inflammation and skin rashes. We present a rare adult onset refractory Nxp2 dermatomyositis following COVID 19 infection Methods: 36-year- old male came with the complaints of: Redness of right eye, Easy fatgiuability ,dysphagia of 3 months duration * Patient had uncomplicated COVID-19 1 month prior to onset of present complaints * On examination he had anasarca proximal muscle weakness and muscle tenderness and had neck and pharyngeal muscle weakness dysphagia and nasal regurgitation.He also had malar rash and periribital rash and swelling (Figure 1) * Investigations revealed biochemical radiological and Electrophysiological evidence of myositis (Table 1) * He was managed with pulse sterids ivig rituximab and tacrolimus with gradual but definite resolution Conclusion(s): Auto-antibodies against NXP2 are detected in 15% to 25% cases of Juvenile dermatomyositis and in only 1% of adult cases. This form of DM is characterized by accompanying calcinosis and severe and chronic disease course and is often carcinoma-associated (breast, uterine or pancreatic carcinoma). Post COVID NXP2 DM has not yet been reported. (Figure Presented).

An Unusual Case of Fetal Myocardial Injury Leading to Intrauterine Fetal Demise: A Possible Sequela of Maternal COVID Infection?

Background. Intrauterine myocardial injury/infarction (MI) is exceedingly uncommon and the few reported cases occur most commonly with congenital cardiac anomalies, particularly anomalous coronary arteries. Here we present a case of MI in a fetus resulting in intrauterine fetal demise (IUFD). A 36-year-old G3 P1011 mother with a history of methylenetetrahydrofolate reductase (MTHFR) mutation (double heterozygote;may lead to a procoagulable state) on prophylactic aspirin and recent COVID-19 infection during pregnancy (5 weeks prior, recovered) presented at 28 weeks gestational age with one day of decreased fetal movement, at which time IUFD was diagnosed. Method(s): Induction of labor with misoprostol was performed, and the fetus was delivered stillborn vaginally without complications. A full autopsy including histologic examination (including placental histopathology and immunohistochemistry) was performed. Result(s): Autopsy examination of the fetus revealed normal development, including a structurally normal heart. However, histologic examination of the heart demonstrated a discrete region of intramyocardial fibrosis, dystrophic calcification, and giant cell reaction involving a large portion of one ventricle, consistent with MI (Fig 1). Immunostains for infectious causes of myocarditis (including COVID, cytomegalovirus, herpes simplex virus, parvovirus, adenovirus, and toxoplasmosis) were negative. Histologic examination of all other organs was unremarkable. Gross examination of the placenta was remarkable for a hypercoiled umbilical cord, and histologic examination revealed a hypoplastic umbilical artery with partial smooth muscle necrosis as well as a single, nonoccluding stem villus thrombus, without histologic evidence of downstream fetal vascular malperfusion. Conclusion(s): The cause of demise was concluded to most likely be due to fetal myocardial injury, but the etiology is unclear. The differential diagnosis includes a thromboembolic phenomenon possibly leading to infarction (given maternal history of MTHFR mutation and recent COVID infection), myocarditis (possibly burned- out phase), coronary artery abnormalities that were not appreciated on fresh dissection of the heart, and ventricular dysplasia/aneurysm (least likely given histologically normal surrounding myocardium). This rare case is an example of a lethal myocardial injury in an otherwise structurally normal fetal heart.

Proton pump inhibitors in systemic sclerosis: a reappraisal to optimise treatment of gastro-oesophageal reflux disease

Gastro-oesophageal reflux disease (GERD) is associated with substantial morbidity in patients with systemic sclerosis. Although the introduction of proton pump inhibitors (PPIs) into clinical care represents a major achievement in the management of gastro-oesophageal problems in systemic sclerosis, PPIs are seldom fully effective in patients with systemic sclerosis, and the use of maximum PPI doses is a very frequent clinical practice. However, there is little evidence to support the empirical use of PPIs in systemic sclerosis. This scarcity of evidence is especially relevant with regards to the safety concerns of long-term exposure, which have been raised in the general population. The purpose of this Viewpoint is to highlight the substantial beneficial impact of PPIs on GERD in patients with systemic sclerosis, while considering the potential adverse effects in this patient population. Furthermore, we highlight the unmet needs of people with systemic sclerosis and GERD and propose an agenda for future research to optimise the safe and effective use of PPIs in systemic sclerosis. Copyright © 2022 Elsevier Ltd

NON-UREMIC CALCIPHYLAXIS SECONDARY TO ACUTE COVID-19

CASE: A 40-year-old white female with medical history significant for COVID-19 infection three months prior to presentation and previous spontaneous miscarriage presented with bilateral lower extremity lesions present for several weeks and described as “sunburn-like” with blistering. The lesions were initially located on her anterior thighs and spread to the lateral thighs and lower back. On presentation, she was found to have several distinct lower extremity lesions, with evidence of necrosis and eschar formation, along with blackened mottled skin. The lesions were extremely painful to the patient. Laboratory evaluation demonstrated hyponatremia, elevated ESR and CRP, with normal serum creatine and calcium. Calciphylaxis was confirmed with biopsy. The patient was treated with strict wound care to prevent infection and received sodium thiosulfate three times weekly and anticoagulation with apixaban due to concern for underlying hypercoagulability. An extensive work-up for underlying autoimmunity and hypercoagulability demonstrated presence of antiphospholipid antibodies with positive Cardiolipin IgM, lupus anticoagulant, and a homogenous ANA patern that showed a titer of 1:160. Her clinical status improved on extensive pain regimen and on follow-up one week later, the lesions were unchanged. IMPACT/DISCUSSION: Calciphylaxis is a known dermatologic finding that is typically diagnosed in the setting of End-stage Renal disease (ESRD). It presents with non-healing, painful skin ulcers that are at a high risk for infection and have poor healing. In the absence of ESRD, calciphylaxis is rare but has been reported in certain settings including hypercoagulable states and/ or autoimmune conditions. We present a case that has an absence of known etiologies for calciphylaxis and hypothesize that this is due to a hypercoagulable state caused by recent COVID-19 infection, or COVID-19 aggravating an underlying hypercoagulable state. This case offers an uncommon diagnosis with an even rarer presentation. Calciphylaxis must be confirmed with biopsy and is extremely debilitating and painful. In the setting of non-uremic calciphylaxis, prevention of infection and management of pain should be prioritized. Additionally, this case offers a platform to identifying COVID-19 as a risk factor for development of calciphylaxis in previously healthy individuals. CONCLUSION: The general internist should be aware of non-uremic calciphylaxis and also be concerned for hypercoagulable state induced from COVID-19. It is important to have accurate history-taking and consider delayed reactions, as in this case.

Is this scleroderma lung or COVID-19 lung?

Case report-IntroductionThis is a case of Pakistani female with limited systemic sclerosis and associated mild interstitial lung disease. The lung disease was complicated by SARS-COV-2 related pneumonitis in April 2020 and that led to treatment challenges.She was previously seen in multiple private hospitals and labelled as Rheumatoid arthritis. She was being treated with long term steroids and Methotrexate. After her initial presentation to our Rheumatology services, her diagnosis was correctly revised to Systemic Sclerosis with phenotype of CREST. Her treatment was adjusted to Vasodilators and Mycophenolate due to skin and Lung involvement.Case report-Case descriptionThis is a case of 40-year-old Pakistani female who had been having multiple joint pains since 2010. She also experienced severe Raynaud's.She presented to our Rheumatology clinic in December 2018. Her symptoms included recurrent digital ulcers, tight and tough skin at fingers and Raynaud's worse during winter months. Her examination confirmed peripheral cyanosis with multiple digital ulcers with superimposed infection, marked sclerodactyly and calcinosis. She was started on Vasodilator therapy including calcium channel blocker and PDE5 inhibitor due to severity of ulceration. Infection was managed with prolonged course of antimicrobial therapy. Her immunology showed positive anti nRNP/Sm. Anti-centromere and anti Scl 70 were negative. Her condition fit description of CREST (Calcinosis, RP, Oesophageal dysmotility, telangiectasia). Her management included weaning off Methotrexate and reduction in the dose of corticosteroids.In February 2019, Respiratory work up showed normal Chest radiograph, High resolution CT chest showing no significant abnormality and FEV1 82%, FVC 86%, and DLCO 77%. Her PASP was 25mmHg. Overall, her condition remained stable over the course of next year. Her medication included Cellcept, low dose prednisolone, hydroxychloroquine, and Sildenafil. More importantly, Digital ulcers have been well controlled with combined vasodilator therapy.In April 2020, she developed SARS-CoV-2 with mild respiratory symptoms and was admitted to a different hospital. Fortunately, she responded well to ward based supportive and symptomatic treatment with no need for respiratory support. Subsequently, she has seen a different respiratory physician and had repeat imaging of chest which has led to dilemma whether the ground glass opacities in both lungs is due to scleroderma lung or COVID-19 related lung disease. She was given high dose prednisolone by the respiratory physician which has been reduced in rheumatology clinic. The new findings on chest imaging are sequelae of SARS-COV-2.Case report-DiscussionThis case highlights few important points as below:Systemic sclerosis diagnosis was not made for many years even though she has had severe digital ulcers for a long time. She was being managed as Rheumatoid arthritis. Systemic sclerosis remains a difficult disease to diagnose and is still under recognised.SARS-COV-2 related illness has not affected this patient adversely despite the fact of being on long term maintenance prednisolone of 7.5mg daily dose and Cellcept 2gm. Her cellcept was temporarily stopped during acute illness.We know that viral pneumonitis can present with typical ground glass opacities in bilateral areas of lungs and differential diagnosis does include connective tissue related lung disease but this lady had no significant respiratory involvement prior to COVID-19 illness and follow up scan will help to decide if this is disease progression or related to viral cause.Case report-Key learning pointsThere are multiple learning points in this case:Continuity of care under same primary team can avoid confusion related to diagnosis and diagnosis related complications. This lady had none, or mild subclinical lung involvement related to systemic sclerosis prior to contracting COVID-19 illness. Her CT chest findings after the episode of SARS-COV-2 were attributed to systemic sclerosis as she was seen by different respiratory team. This conti uity is not always possible, but MDT collaboration needs to be improved across hospitals and across various departments.Systemic sclerosis remains an under diagnosed and under recognized complex rheumatic disorder and more primary care physicians need to be educated so they can appropriately refer these cases to Rheumatology services.Multi-disciplinary collaboration between Rheumatology, Respiratory and other specialties is the key point to manage these complex cases.This case also highlights an interesting observation that presence of significant immune disorder and immunosuppressant medication does not always equate to worse outcome if patient contracts SARS-COV-2. Supportive care, appropriate observation, and temporary suspension of DMARD in such cases can avoid any further complications.