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Multisystem inflammatory syndrome in children (MIS-C) is a severe complication of SARS-CoV-2 infections in children. This syndrome manifests about a month after the initial viral infection and is characterized by fever, multiorgan dysfunction, and systemic inflammation. This chapter will review the emergence, epidemiology, clinical characteristics, diagnosis, pathophysiology, immunomodulatory treatment, prognosis, outcomes, and prevention of MIS-C. While the pathophysiology of MIS-C remains to be defined, it is a post-infection, hyperinflammatory syndrome of childhood with elevated inflammatory cytokines.

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BACKGROUND: TAFRO syndrome is a rare disorder that causes thrombocytopenia, generalized oedema, fever, organ enlargement, and renal impairment. Few reports have suggested an association with vaccines, and few cases have undergone renal biopsy. TAFRO syndrome is often severe and fatal, and its cause is unknown. We report a case of TAFRO syndrome that occurred after vaccination with the coronavirus disease 2019 (COVID-19) vaccine. CASE PRESENTATION: An 82-year-old woman received two doses of the BNT162b2 mRNA vaccine 3 weeks apart. Two weeks later, she was admitted to the hospital with oedema, accompanied with renal failure and thrombocytopenia. After close examination, she was diagnosed with TAFRO syndrome. She was treated with steroids, cyclosporine, and thrombopoietin receptor agonists. The patient was discharged after several months in remission. CONCLUSIONS: Although an incident of TAFRO syndrome after COVID-19 vaccination has been previously reported, this is a rare case in which the patient went into remission and was discharged. A renal biopsy was also performed in this case, which was consistent with previous reports. The favorable treatment course for TAFRO syndrome provides valuable insights.

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Multisystem inflammatory syndrome in adults (MIS-A) is a systemic inflammatory disease associated with COVID-19 and follows coronary artery aneurysms similar to Kawasaki disease. In many cases, it is improved by treatments such as high-dose steroids or intravenous immunoglobulin (IVIg). However, the role of untreated coronary artery aneurysms leading to future stenosis remains unknown. Untreated MIS-A may potentially lead to the formation of coronary aneurysms. In cases of COVID-19 where young adults present with angina-like symptoms, an evaluation for angina is considered. Herein, we report a case of a 27-year-old female who developed unstable angina with coronary artery aneurysms six months after COVID-19 infection. She required surgery for unstable angina, which resulted in an improvement in chest pain. Coronary artery lesions are considered to be related to MIS-A, and treatment was conducted in accordance with that for Kawasaki disease. Currently, the pathological differences and prognosis between MIS-A and Kawasaki disease remain unclear, but the elucidation of the conditions is warranted in the future.

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BACKGROUND: During the second wave of the coronavirus disease 2019 (COVID-19) pandemic, a subset of critically ill patients developed delayed respiratory deterioration in the absence of new infection, fluid overload or extra-pulmonary organ dysfunction. AIM: To describe the clinical and laboratory characteristics, outcomes, and management of these patients, and to contrast this entity with other post COVID-19 immune dysregulation related inflammatory disorders. METHODS: This was a retrospective observational study of adult patients admitted to the medical intensive care unit of a 2200-bed university affiliated teaching hospital, between May and August 2021, who fulfilled clearly defined inclusion and exclusion criteria. Outcome was assessed by a change in PaO2/FiO2 ratio and levels of inflammatory markers before and after immunomodulation, duration of mechanical ventilation after starting treatment, and survival to discharge. RESULTS: Five patients developed delayed respiratory deterioration in the absence of new infection, fluid overload or extra-pulmonary organ dysfunction at a median interquartile range (IQR) duration of 32 (23-35) d after the onset of symptoms. These patients had elevated inflammatory markers, required mechanical ventilation for 13 (IQR 10-23) d, and responded to glucocorticoids and/or intravenous immunoglobulin. One patient died (20%). CONCLUSION: This delayed respiratory worsening with elevated inflammatory markers and clinical response to immunomodulation appears to contrast the well described Multisystem Inflammatory Syndrome - Adults by the paucity of extrapulmonary organ involvement. The diagnosis can be considered in patients presenting with delayed respiratory worsening, that is not attributable to cardiac dysfunction, fluid overload or ongoing infections, and associated with an increase in systemic inflammatory markers like C-reactive protein, inteleukin-6 and ferritin. A good response to immunomodulation can be expected. This delayed inflammatory pulmonary syndrome may represent a distinct clinical entity in the spectrum of inflammatory syndromes in COVID-19 infection.

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Multisystem inflammatory syndrome in adults (MIS-A) is a life-threatening disease that can develop weeks after coronavirus disease 2019 (COVID-19). MIS-A symptoms include multiorgan involvement, especially gastrointestinal tract and heart involvement, and Kawasaki disease-like symptoms. We herein report a 44-year-old Japanese man with MIS-A who had contracted COVID-19 five weeks ago and went into shock after acute gastroenteritis, acute kidney injury, and Kawasaki disease-like symptoms. Methylprednisone pulse and high-dose intravenous immunoglobulin resulted in recovery of shock and his renal function, but diffuse ST-segment elevation on electrocardiography and pericardial effusion with a fever emerged after therapy. Additional granulocyte-monocyte adsorptive apheresis successfully ameliorated the cardiac involvement.

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BACKGROUND: The diagnosis of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated multisystem inflammatory syndrome in adults (MIS-A) requires distinguishing it from acute coronavirus disease 2019 (COVID-19) and may affect clinical management. METHODS: In this retrospective cohort study, we applied the US Centers for Disease Control and Prevention case definition to identify adults hospitalized with MIS-A at 6 academic medical centers from 1 March 2020 to 31 December 2021. Patients MIS-A were matched by age group, sex, site, and admission date at a 1:2 ratio to patients hospitalized with acute symptomatic COVID-19. Conditional logistic regression was used to compare demographic characteristics, presenting symptoms, laboratory and imaging results, treatments administered, and outcomes between cohorts. RESULTS: Through medical record review of 10 223 patients hospitalized with SARS-CoV-2-associated illness, we identified 53 MIS-A cases. Compared with 106 matched patients with COVID-19, those with MIS-A were more likely to be non-Hispanic black and less likely to be non-Hispanic white. They more likely had laboratory-confirmed COVID-19 ≥14 days before hospitalization, more likely had positive in-hospital SARS-CoV-2 serologic testing, and more often presented with gastrointestinal symptoms and chest pain. They were less likely to have underlying medical conditions and to present with cough and dyspnea. On admission, patients with MIS-A had higher neutrophil-to-lymphocyte ratio and higher levels of C-reactive protein, ferritin, procalcitonin, and D-dimer than patients with COVID-19. They also had longer hospitalization and more likely required intensive care admission, invasive mechanical ventilation, and vasopressors. The mortality rate was 6% in both cohorts. CONCLUSIONS: Compared with patients with acute symptomatic COVID-19, adults with MIS-A more often manifest certain symptoms and laboratory findings early during hospitalization. These features may facilitate diagnosis and management.

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A 21-year-old man on hemodialysis was hospitalized for coronavirus disease 2019 (COVID-19) pneumonia. After admission, he had a persistent high fever and developed erythema induratum on his extremities. Laboratory tests conducted 25 days after onset showed markedly increased procalcitonin (PCT) levels (>100 ng/mL). The patient developed impaired consciousness and hypotensive shock and required endotracheal intubation. Based on the presence of erythema induratum and multiorgan dysfunction, he was diagnosed with multisystem inflammatory syndrome (MIS). The MIS resolved after treatment with intravenous immunoglobulin and methylprednisolone. This report illustrates that MIS can occur in adults and may be accompanied by high PCT levels.

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BACKGROUND: The coronavirus disease 2019 (COVID-19) has persisted for over 2 years worldwide and has long-term effects on the health and quality of life of convalescents. Multisystem inflammatory syndrome, primary observed in children is currently increasingly recognized in adults. Immunopathology might play a crucial role in the pathogenesis of multisystem inflammatory syndrome in adults (MIS-A); therefore, the occurrence of MIS-A in non-immunocompetent patients is a significant challenge in diagnosis and treatment. CASE PRESENTATION: We described a 65-year-old patient with Waldenström's macroglobulinemia (WM) who suffered from MIS-A after COVID-19 and was successfully treated with high doses of immunoglobulins and steroids. CONCLUSION: Our study presents for the first time a case of MIS-A in a hematological patient with a broad spectrum of symptoms reflecting multiorgan damage and suggests the long-term consequences of MIS-A as persistent immune dysregulation involving T-cell response.

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Severe inflammation and one or more extrapulmonary organ dysfunctions have been observed in those who had recently developed COVID-19, except for a macrophage activation syndrome-like picture. A 50-year-old female patient was admitted to the emergency department with fever and a history of COVID-19 infection. More than one area of hemophagocytosis was found in the bone marrow aspiration. The HLH-2004 protocol was started with neurological involvement and she underwent splenectomy due to massive intra-abdominal bleeding secondary to splenic laceration on the 3rd day. Multiple microthrombosis and infarcts were observed in the splenectomy specimen. At the 4th week of the treatment, she was discharged with oral agents. Splenic microthrombosis and splenic rupture due to "multisystem inflammatory syndrome in adults" are the most important findings of this report.

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A 20-year-old woman with previous COVID-19 diagnosis presented with abdominal pain and colitis on CT scan. She was admitted in septic shock, with etiology of colitis unclear. After resuscitation, antibiotics, and steroids, she clinically deteriorated. Worsening Clostridioides difficile infection was most likely and she was taken to the operating room. Intraoperatively, only a segment of transverse colon appeared abnormal on gross and endoscopic evaluation. Total colectomy was deferred in favor of segmental resection. Given her unusual disease pattern and recent COVID-19 infection, diagnosis of MIS-C was considered. Steroids were continued and treatment broadened to include heparin and IVIG. The patient returned to the operating room for planned reexploration, endoscopy, and end colostomy. On hospital day three, the patient had an acute mental status change. Computed tomography demonstrated acute cerebral edema with brainstem herniation. The family chose comfort-care measures. Final pathology from the transverse colon demonstrated COVID-19-associated vasculitis.

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The overlap between multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) including coronary artery aneurysms (CAA) and broadly shared gastrointestinal and mucocutaneous disease is poorly defined. In this perspective, we highlight common age-related extravascular epicardial microanatomical and immunological factors that might culminate in CAA expression in both MIS-C and KD. Specifically, the coronary vasa vasorum originates outside the major coronary arteries. Widespread inflammation in the epicardial interstitial compartment in shared between KD and MIS-C. Age-related changes in the neonatal and immature coronary vasculature including the impact of coronary artery biomechanical factors including coronary vessel calibre, age-related vessel distensibility, flow, and vessel neurovascular innervation may explain the decreasing CAA frequency from neonates to older children and the virtual absence of CAA in young adults with the MIS-C phenotype. Other KD and MIS-C features including mucocutaneous disease with keratinocyte-related immunopathology corroborate that disease phenotypes are centrally influenced by inflammation originating outside vessel walls but a potential role for primary coronary artery vascular wall inflammation cannot be excluded. Hence, common extravascular originating tissue-specific responses to aetiologically diverse triggers including superantigens may lead to widespread interstitial tissue inflammation characteristically manifesting as CAA development, especially in younger subjects. Given that CAA is virtually absent in adults, further studies are needed to ascertain whether epicardial interstitial inflammation may impact on both coronary artery physiology and cardiac conduction tissue and contribute to cardiovascular disease- a hitherto unappreciated consideration.

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The clinical spectrum of Coronavirus 2019 (COVID-19) includes acute COVID-19, long covid and multisystem inflammatory syndrome in children and adults (MISC/A). The rapid roll-out of COVID-19 vaccination has the potential to affect the clinical presentation of COVID-19 and case reports document rare occurrences of MIS-A after COVID-19 infection and recent vaccination with m-RNA vaccines. We describe 2 cases of MIS-A after COVID-19 infection and recent vaccination with ChAdOx1 nCoV-19.

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Background: Following infection with severe acute respiratory syndrome coronavirus, a post-infectious multisystem inflammatory syndrome in adults (MIS-A) has been identified. It affects multiple organ systems and can lead to multi-organic failure. Case summary: This case report describes a patient with MIS-A with significant cardiac involvement including peri-myocarditis, pulmonary hypertension, right-sided heart failure, tricuspid regurgitation, and cardiogenic shock. After being diagnosed and treated correctly for MIS-A, the patient recovered completely, without any cardiac sequelae. Discussion: The hyperinflammation in MIS-A can have cardiac engagement. Although more research is required to further clarify the underlying mechanisms, prompt diagnosis and anti-inflammatory treatment are crucial for better outcomes and cardiac recovery.

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During the current pandemic, acute coronavirus disease 2019 (COVID-19) due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) provokes overwhelming inflammatory response leading to a wide range of clinical presentations including, a rare multisystem inflammatory syndrome and cardiac injury. Not only during the acute phase of the disease but a delayed immunologic response to SARS-CoV-2 infection among people with hyperinflammatory illness several weeks postacute phase of the infection is recently recognized. We report a young adult male who presented with acute myocarditis and heart failure associated with laboratory evidence of hyperinflammatory syndrome 5 weeks after a full recovery from COVID-19 infection. We believe that health-care providers need to be aware and recognize this syndrome as a rare sequela of COVID-19 infection.

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Multisystem inflammatory syndrome is a life-threatening condition associated with elevated inflammatory markers and multiple organ injury. A diagnosis of exclusion, it has been reported after severe acute respiratory syndrome coronavirus 2 infection (SARS-CoV-2) in children and adults; recently it has been described in some post-COVID-19 vaccinated individuals. The prognosis with supportive care and immunomodulatory therapy is good, although some individuals may require treatment in the intensive care unit (ICU). Here we report a case of a 58-year-old man who developed multi-organ failure after receiving the second dose of the Moderna mRNA-1273 COVID-19 vaccine. He required critical organ support in the ICU. An extensive workup was done to rule out alternative infectious and inflammatory processes. Following a period of gradual in-hospital convalescence, our patient made a full recovery. To our knowledge, this is the first comprehensively described case of multisystem inflammatory syndrome associated with Moderna mRNA-1273 COVID-19 vaccine in an adult over 50 years of age.

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We report a case of a 21-year-old previously healthy man who developed severe toxoplasmosis with chorioretinitis and myositis 2 months after receiving corticosteroids for presumed multisystem inflammatory syndrome in adults, in the setting of a recently acquired acute Toxoplasma infection, likely during a trip to Latin America.

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We herein report a case of multisystem inflammatory syndrome in adults (MIS-A) complicated with Kikuchi-Fujimoto disease (KFD). A previously healthy 41-year-old man presented with painful swelling of the cervical lymph nodes, fever, diarrhea, conjunctivitis, edema, and hypotension one month after the onset of asymptomatic coronavirus disease 2019. Laboratory investigations revealed an elevation of CRP, and echocardiography indicated diastolic dysfunction. We diagnosed the patient to have MIS-A. Histopathology of the lymph nodes showed necrotizing lymphadenitis. After the initiation of hydrocortisone and diuretics, his symptoms resolved immediately. This case suggested that post-viral immune dysregulation in MIS-A could play a role in the etiology of KFD.

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Multisystem inflammatory syndrome in an adult (MIS-A) is a rare immunological complication that gained prominence after the coronavirus disease 2019 pandemic. Patients with MIS-A often clinically present with non-specific generalized symptoms, such as fever, myalgia, or fatigue, making the diagnosis difficult. In this article, we present an unusual case of MIS-A in a 50-year-old male that raises the question of whether the immune system's dysregulation will demonstrate differing criteria of signs and symptoms for a patient on sustained immunosuppression as opposed to the non-immunosuppressed population.