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Multisystem Inflammatory Syndrome in Children [Editorial]

Shust, Gail F; Soma, Vijaya L; Kahn, Philip; Ratner, Adam J
PMID: 34210761
ISSN: 1526-3347
CID: 4927192

Multisystem inflammatory syndrome in children (MIS-C) and retropharyngeal edema: A case series

Daube, Ariel; Rickert, Scott; Madan, Rebecca Pellett; Kahn, Philip; Rispoli, Joanne; Dapul, Heda
Multisystem inflammatory syndrome in children (MIS-C) is thought to follow SARS-CoV-2 infection and presents with fever and multisystem dysfunction. We report three children with suspected MIS-C found to have retropharyngeal edema without evidence of a bacterial etiology. We raise the possibility that an association between MIS-C and retropharyngeal edema exists.
PMID: 33752089
ISSN: 1872-8464
CID: 4822422

Mucocutaneous Manifestations of Multisystem Inflammatory Syndrome in Children During the COVID-19 Pandemic

Young, Trevor K; Shaw, Katharina S; Shah, Jinal K; Noor, Asif; Alperin, Risa A; Ratner, Adam J; Orlow, Seth J; Betensky, Rebecca A; Shust, Gail F; Kahn, Philip J; Oza, Vikash S
Importance/UNASSIGNED:To date, no study has characterized the mucocutaneous features seen in hospitalized children with multisystem inflammatory syndrome in children (MIS-C) or the temporal association of these findings with the onset of systemic symptoms. Objective/UNASSIGNED:To describe the mucocutaneous findings seen in children with MIS-C during the height of the coronavirus disease 2019 (COVID-19) pandemic in New York City in 2020. Design, Setting, and Participants/UNASSIGNED:A retrospective case series was conducted of 35 children admitted to 2 hospitals in New York City between April 1 and July 14, 2020, who met Centers for Disease Control and Prevention and/or epidemiologic criteria for MIS-C. Main Outcomes and Measures/UNASSIGNED:Laboratory and clinical characteristics, with emphasis on mucocutaneous findings, of children who met criteria for MIS-C. The characterization of mucocutaneous features was verified by 2 board-certified pediatric dermatologists. Results/UNASSIGNED:Twenty-five children (11 girls [44%]; median age, 3 years [range, 0.7-17 years]) were identified who met definitional criteria for MIS-C; an additional 10 children (5 girls [50%]; median age, 1.7 years [range, 0.2-15 years]) were included as probable MIS-C cases (patients met all criteria with the exception of laboratory test evidence of severe acute respiratory syndrome coronavirus 2 [SARS-CoV-2] infection or known exposure). The results of polymerase chain reaction tests for SARS-CoV-2 were positive for 10 patients (29%), and the results of SARS-CoV-2 immunoglobulin G tests were positive for 19 patients (54%). Of the 35 patients, 29 (83%) exhibited mucocutaneous changes, with conjunctival injection (n = 21), palmoplantar erythema (n = 18), lip hyperemia (n = 17), periorbital erythema and edema (n = 7), strawberry tongue (n = 8), and malar erythema (n = 6) being the most common findings. Recognition of mucocutaneous findings occurred a mean of 2.7 days (range, 1-7 days) after the onset of fever. The duration of mucocutaneous findings varied from hours to days (median duration, 5 days [range, 0-11 days]). Neither the presence nor absence of mucocutaneous findings was significantly associated with overall disease severity. Conclusions and Relevance/UNASSIGNED:In this case series of hospitalized children with suspected MIS-C during the COVID-19 pandemic, a wide spectrum of mucocutaneous findings was identified. Despite their protean and transient nature, these mucocutaneous features serve as important clues in the recognition of MIS-C.
PMID: 33295957
ISSN: 2168-6084
CID: 4708992

COVID-19 associated Kawasaki-like multisystem inflammatory disease in an adult [Case Report]

Sokolovsky, Sabrina; Soni, Parita; Hoffman, Taryn; Kahn, Philip; Scheers-Masters, Joshua
Recent reports have described a secondary Multisystem Inflammatory Syndrome in Children (MIS-C) after a prior COVID-19 infection that often has features of Kawasaki disease (KD). Here, we report the case of a 36-year-old woman who presented to the emergency department hypotensive and tachycardic after 1 week of fevers, abdominal pain, vomiting and diarrhea, and was found to have the classic phenotype of complete Kawasaki's Disease including nonexudative conjunctivitis, cracked lips, edema of the hands and feet, palmar erythema, a diffuse maculopapular rash, and cervical lymphadenopathy. Initial laboratory studies were significant for hyponatremia, elevated liver function tests including direct hyperbilirubinemia, and leukocytosis with neutrophilia. Imaging revealed mild gallbladder wall edema, a small area of colitis, and small pleural effusion. She was treated for Kawasaki Disease Shock Syndrome (KDSS) with pulse dose solumedrol, IVIG, and aspirin with near resolution of symptoms and normalization of vital signs within 1 day and subsequent improvement in her laboratory abnormalities. She was later found to be COVID-19 IgG positive, suggesting past exposure. This case represents an early report of a KD-like illness in an adult with serologic evidence of a previous COVID-19 infection, similar to MIS-C. It suggests that the virulent strain of SARS-CoV-2 appears to cause a post-infectious inflammatory syndrome similar to KD in adults, as well as children. Our understanding of the myriad of COVID-19 symptoms and sequelae is rapidly evolving. We recommend physicians remain vigilant for inflammatory syndromes that mimic KD/KDSS which may warrant prompt treatment with IVIG and steroids.
PMID: 32631771
ISSN: 1532-8171
CID: 4525062

Multisystem Inflammatory Syndrome in Children Associated with Status Epilepticus [Case Report]

Shenker, Jennifer; Trogen, Brit; Schroeder, Laura; Ratner, Adam J; Kahn, Philip
A 12-year-old boy presented to the emergency department with findings concerning for multisystem inflammatory syndrome in children. After clinical stabilization following treatment with antibiotics, remdesivir, and anakinra, the patient was noted to have episodes of altered mentation. Video electroencephalogram revealed status epilepticus, which was subsequently controlled with antiepileptic medications.
PMID: 32712284
ISSN: 1097-6833
CID: 4614242

Evaluation of the reliability and validity of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) in pediatric cutaneous lupus among pediatric dermatologists and rheumatologists

Kushner, C J; Tarazi, M; Gaffney, R G; Feng, R; Ardalan, K; Brandling-Bennett, H A; Castelo-Soccio, L; Chang, J C; Chiu, Y E; Gmuca, S; Hunt, R D; Kahn, P J; Knight, A M; Mehta, J; Pearson, D R; Treat, J R; Wan, J; Yeguez, A C; Concha, J S S; Patel, B; Okawa, J; Arkin, L M; Werth, V P
BACKGROUND:The CLASI is a reliable outcome measure for cutaneous lupus erythematosus (CLE) in adults used in clinical trials. However, it has not been validated in children, limiting clinical trials for pediatric CLE. OBJECTIVE:This study aims to validate the CLASI in pediatrics. METHODS:Eleven pediatric patients with CLE, six dermatologists and six rheumatologists, participated. Physicians were trained to use the CLASI and Physician Global Assessment (PGA). Physicians individually rated all patients using both tools. Each physician reassessed two randomly selected patients. Within each physician group, Intraclass Correlation Coefficient (ICC) was calculated to assess the reliability of each measure. RESULTS:CLASI activity scores demonstrated excellent inter- and intra-rater reliability (ICC>0.90), while the PGA activity scores had good inter-rater reliability (ICC 0.73-0.77) among both specialties. PGA activity scores showed excellent intra-rater reliability (ICC=0.89) and good intra-rater reliability (ICC=0.76) for dermatologists and rheumatologists, respectively. LIMITATIONS/CONCLUSIONS:Limitations of this study included the small sample size of patients and potential recall bias during the physician re-rating session. CONCLUSION/CONCLUSIONS:CLASI activity measurement showed excellent inter- and intra-rater reliability in pediatric CLE and superiority to findings with the PGA. These results demonstrate that the CLASI is a reliable and valid outcome instrument for pediatric CLE.
PMID: 30033560
ISSN: 1365-2133
CID: 3216292

Neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis [Case Report]

Stringer, Thomas; Gittler, Julia; Meehan, Shane; Kahn, Philip; Oza, Vikash S
This report describes a case of chronic neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis. When encountered in children, neutrophilic urticarial dermatosis should raise suspicion of autoimmune or autoinflammatory disease.
PMID: 29574969
ISSN: 1525-1470
CID: 3059922

Comparing the importance of quality measurement themes in juvenile idiopathic inflammatory myositis between patients and families and healthcare professionals

Tory, Heather O; Carrasco, Ruy; Griffin, Thomas; Huber, Adam M; Kahn, Philip; Robinson, Angela Byun; Zurakowski, David; Kim, Susan
BACKGROUND:A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures. METHODS:Surveys were developed by members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Workgroup through a consensus process and administered to patients and families through the CureJM Foundation and to healthcare professionals through CARRA. The survey asked respondents to rate the importance of 19 items related to aspects of high quality care, using a Likert scale. RESULTS:Patients and families gave generally higher scores for importance to most of the quality measurement themes compared with healthcare professionals, with ratings of 13 of the 19 measures reaching statistical significance (p < 0.05). Of particular importance, however, was consensus between the groups on the top five most important items: quality of life, timely diagnosis, access to rheumatology, normalization of functioning/strength, and ability for self care. CONCLUSIONS:Despite overall differences in the rating of importance of quality indicators between patients and families and healthcare professionals, the groups agreed on the most important aspects of care. Recognizing areas of particular importance to patients and families, and overlapping in importance with providers, will promote the development of standardized quality measures with the greatest potential for improving care and outcomes for children with JIIM.
PMID: 29673367
ISSN: 1546-0096
CID: 3042802

Evaluation of the reliability and validity of the cutaneous lupus erythematosus disease area and severity index (CLASI) in pediatrics [Meeting Abstract]

Kushner, C J; Tarazi, M; Gaffney, R; Feng, R; Ardalan, K; Brandling-Bennett, H; Castello-Soccio, L; Chang, J C; Chiu, Y E; Gmuca, S; Hunt, R D; Kahn, P; Knight, A M; Mehta, J; Pearson, D; Treat, J R; Wan, J; Yeguez, A; Concha, J S S; Patel, B; Okawa, J; Arkin, L M; Werth, V P
Cutaneous lupus erythematosus (CLE) refers to skin manifestations of the autoimmune disease lupus erythematosus (LE). Skin involvement is one of the most common presenting signs of systemic lupus erythematosus (SLE) and its evaluation can be critical in making a diagnosis and monitoring disease progression. Patients may also present with isolated CLE without systemic disease. The visible lesions of CLE can be disfiguring and distressing to patients. While CLE has been extensively researched in the adult population, few studies exist in the pediatric population. The development of a validated disease severity tool is crucial for monitoring the natural history of skin involvement in lupus registry studies. There is also a great need for new therapeutic agents, and demonstrating the efficacy of these agents will require clinical trials with reliable outcome measures. The CLASI is a reliable outcome measure for CLE in the adult population, where it is commonly used in clinical trials for SLE. However, no study has validated this assessment tool in children, potentially limiting the conduct of clinical trials in pediatric SLE. The study will include at least five pediatric dermatologists and five rheumatologists to independently evaluate patients with pediatric cutaneous lupus. The study will take place at the autoimmune disease clinic of the University of Pennsylvania, on March 3rd, 2018. The physicians will be given a training session on the assessment of cutaneous lupus using 2 measurement tools: the CLASI and the Physician Global Assessment (PGA), which allow grading of skin activity and skin damage, as well as a score for the overall findings. One cohort of physicians will apply the PGA before the CLASI and the other cohort will apply the CLASI before the PGA. Inter-rater reliability within each physician group will be determined by intraclass correlation coefficient (ICC), type ICC and its confidence interval. While many pediatric dermatologists and rheumatologists have shown strong interest in participation, there have been difficulties recruiting pediatric CLE patients with currently active disease who are willing to travel to Philadelphia. However we have reached out to over ten institutions and all physicians are actively searching for interested patients and we have identified several patients who will participate. The primary benefit of this study is the validation of a standardized instrument that can be applied to pediatric cutaneous lupus to facilitate epidemiologic studies and provide a critical tool for clinical trials. Secondary benefits include more standardized documentation of skin disease activity and damage between specialties to help facilitate clinical practice and interdisciplinary collaboration
ISSN: 1546-0096
CID: 3516822

Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease

Kim, Susan; Kahn, Philip; Robinson, Angela B; Lang, Bianca; Shulman, Andrew; Oberle, Edward J; Schikler, Kenneth; Curran, Megan Lea; Barillas-Arias, Lilliana; Spencer, Charles H; Rider, Lisa G; Huber, Adam M
BACKGROUND: Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript. METHODS: The Children's Arthritis and Rheumatology Research Alliance (CARRA) is a North American consortium of pediatric rheumatology health care providers. CARRA members collaborated to determine consensus on typical treatments for JDM patients with skin findings without significant weakness, to develop CTPs for this subgroup of patients. We used a combination of Delphi surveys and nominal group consensus meetings to develop these CTPs. RESULTS: Consensus was reached on patient characteristics and outcome assessment, and CTPs were developed and finalized for patients with skin predominant JDM. Treatment option A included hydroxychloroquine alone, Treatment option B included hydroxychloroquine and methotrexate, and Treatment option C included hydroxychloroquine, methotrexate and corticosteroids. CONCLUSIONS: Three CTPs were developed for use in children with skin predominant JDM, which reflect typical treatment approaches. These are not considered to be specific recommendations or standard of care. Using the CARRA network and prospective data collection, we will be able to apply statistical methods in the future to allow comparisons of JDM patients following these consensus treatment plans.
PMID: 28077146
ISSN: 1546-0096
CID: 2400732