Hyepereosiniphilic syndrome and COVID-19: 2 case reports.

BACKGROUND: Nearly half of the patients with hypereosinophilic syndrome (HES) have cardiovascular involvement, a major cause of mortality. COVID-19 infection can lead to cardiac involvement, negatively impacting the clinical course and prognosis. We reported two patients with HES complicated by COVID-19, with cardiac involvement and valve replacement. CASE PRESENTATION: Our first patient was a 27-year-old woman admitted due to dyspnea and signs of heart failure. She had severe mitral stenosis and mitral regurgitation on the echocardiogram. Corticosteroid therapy improved her symptoms initially, but she deteriorated following a positive COVID-19 test. A repeated echocardiogram showed right ventricular failure, severe mitral regurgitation, and torrential tricuspid regurgitation and, she underwent mitral and tricuspid valve replacement. Our second patient was a 43-year-old man with HES resulted in severe tricuspid stenosis, which was improved with corticosteroid treatment. He underwent tricuspid valve replacement due to severe valvular regurgitation. He was admitted again following tricuspid prosthetic mechanical valve thrombosis. Initial workups revealed lung involvement in favor of COVID-19 infection, and his PCR test was positive. CONCLUSION: COVID-19 infection can change the clinical course of HES. It may result in a heart failure exacerbation due to myocardial injury and an increased risk of thrombosis in prosthetic valves or native vessels due to hypercoagulability.

Concurrent hypereosinophilic syndrome and deep vein thrombosis after Pfizer-BioNTech COVID-19 vaccination: A case report.

Herein, we report a case of eosinophilia syndrome and deep vein thrombosis presenting concurrently after the administration of the BNT162b2 mRNA-based coronavirus disease 2019 (COVID-19) vaccine. It is extremely rare to have both hypereosinophilic syndrome and deep vein thrombosis simultaneously. Both are serious diseases and should be treated with caution.

Hypereosinophilic Syndrome Following the BNT162b2 (BioNTech/Pfizer) Vaccine Successfully Treated with Mepolizumab: A Case Report and Review of the Literature.

Although an increasing number of real-life data confirm large-scale clinical trial findings on the efficacy and safety of SARS-CoV-2 vaccines, rare but severe adverse reactions have begun to emerge. Here, we report a full-blown hypereosinophilic syndrome (HES) following a BNT162b2 (BioNTech/Pfizer) vaccine. A 48-year-old man developed, 5 days after the first shot of the SARS-CoV-2 vaccine, erythematous and painful nodular lesions in the lower and upper limbs accompanied by widespread itching, acrocyanosis with gangrenous lesions at the tips of the first and fourth fingers of the right hand, as well as paresthesia in the right hand and foot. Investigations revealed isolated eosinophilia, occlusion of the right ulnar artery, and electromyography alteration compatible with multifocal sensory neuropathy, as well as minimal accentuation of the interstitial texture with some ground glass appearance. Despite treatment with prednisone in combination with warfarin, he developed thrombosis of the left ulnar artery. Therefore, therapy with an IL-5 inhibitor and acetylsalicylic was successfully added. Given the time interval between the onset of clinical manifestations and the vaccine shot, we believe that the mRNA vaccine triggered the eosinophilic response. This case evidences a possible link between HES and the SARS-CoV-2 vaccination. Mepolizumab, an IL-5 inhibitor, might be considered in steroid refractory cases.

Acute exacerbation of idiopathic hypereosinophilic syndrome following asymptomatic coronavirus disease 2019: a case report.

BACKGROUND: Previous research has suggested that some autoimmune diseases develop after the occurrence of coronavirus disease 2019. Hypereosinophilic syndrome is a rare disease presenting with idiopathic eosinophilia and multiple organ involvement, including the skin, lungs, gastrointestinal tract, heart, and nervous system. The diagnosis of idiopathic hypereosinophilic syndrome poses a dilemma because clinical manifestation and serum biomarkers are similar to those of eosinophilic granulomatosis with polyangiitis. Only a few cases have been reported where coronavirus disease 2019 may have caused the new onset or exacerbation of eosinophilic granulomatosis with polyangiitis or idiopathic hypereosinophilic syndrome. CASE PRESENTATION: We present the case of a 48-year-old Japanese woman with history of asthma who developed deteriorating symptoms of insidiously developed idiopathic hypereosinophilic syndrome following asymptomatic coronavirus disease 2019. She developed acute-onset back pain, tachycardia, abdominal discomfort, loss of appetite, weight loss, skin rash on the back, and numbness of the extremities 3 days after the quarantine period. Extreme hypereosinophilia with multiple abnormal findings including pulmonary ground-glass opacity lesions and mononeuritis multiplex was consistent with hypereosinophilic syndrome. Normal cellularity with eosinophilic proliferation in the bone marrow and negative FIP1L1-PDGFRA raised the diagnosis of idiopathic hypereosinophilic syndrome. Although the patient tested negative for anti-neutrophilic cytoplasmic antibodies and skin biopsy was negative for vasculitis, eosinophilic granulomatosis with polyangiitis could not be excluded. Since glucocorticoids are a standard therapy for both idiopathic hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis, we initiated glucocorticoids following a multidisciplinary discussion. CONCLUSION: Although the relationship between asymptomatic coronavirus disease 2019 and acute idiopathic hypereosinophilic syndrome exacerbation was uncertain, the chronological order of the symptomatic development suggested a possible link. More clinical cases and population-based studies are needed to determine the potential effect of coronavirus disease 2019 on autoimmune diseases.

HYPEREOSINOPHILIC SYNDROME FOLLOWING the BNT162B2 (BIONTECH/PFIZER) VACCINATION

Background: Some reports of small vessel vasculitis following nSARS-CoV2 vaccination are reported in the literature (1, 2). Objectives: We purpose to report the case of small-medium vessel vasculitis after BNT162b2 (BioNTech/Pfzer) vaccination. Methods: We present the case of a 48 years old man with an unremarkable history who underwent BNT162b2 vaccination. Results: Five days after the frst shot of BNT162b2 vaccine, the patient refer the onset of left inguinal adenopathy, and erythematous dermatitis of the trunk. Ultrasound of the groin found increase bilateral inguinal lymph nodes with reactive characters. Contextually, erythematous, itchy and painful nodular lesions appear in the lower and upper limbs as well as acrocyanosis and paresthesia in the right hand and foot. The tests performed showed thrombocytopenia and eosinophilia. While, CRP, search for fecal parasites, pANCA, cANCA, ANA, RAST test, serum tryptase were all absent. Haematological evaluation, bone marrow biopsy, karyotype and molecular biology (FIP1L1/PDGFRa), were performed, all results negative. The patient was admitted in Internal Medicine ward for worsening of skin lesions and of acrocyanosis with gangrenous lesions at the tips of the fourth fnger of the right hand. An angio-CT showed an occlusion of the right ulnar artery. At electromyography an axonal sensory neuropathy was found. The skin biopsy showed fbrinoid necrosis of venules of the superfcial vascular plexus associated with numerous eosinophils, lymphocytes and karyorrhetic debris (Figure 1). High-resolution CT scan described diffuse minimal accentuation of the interstitial texture with micronodular aspects and some ground glass appearance. The diagnosis of hypereosinophilic syndrome was made. Therapy with Methylprednisolone 500 mg/daily for 3 days then Prednisone 1 mg/kg daily in association with IL-5 inhibitor (mepolizumab) with good clinical response, in addition to anticoagulation with warfarin was started. Conclusion: To our knowledge this might be the frst case of (HES) following COVID vaccine. As our experience, due to the short commercialization of anti-nSARs-CoV2 vaccines, is limited further studies are needed to explore the possible effect on small-medium vessels.

Pancarditis as the Clinical Presentation of Eosinophilic Granulomatosis with Polyangiitis: A Multimodality Approach to Diagnosis

Eosinophilic pancarditis (EP) is a rare, often unrecognized condition caused by endomyocardial infiltration of eosinophil granulocytes (referred as eosinophilic myocarditis, EM) associated with pericardial involvement. EM has a variable clinical presentation, ranging from asymptomatic cases to acute cardiogenic shock requiring mechanical circulatory support (MCS) or chronic restrictive cardiomyopathy at high risk of progression to dilated cardiomyopathy (DCM). EP is associated with high in‐hospital mortality, particularly when associated to endomyocardial thrombosis, coronary arteries vasculitis or severe left ventricular systolic dysfunction. To date, there is a lack of consensus about the optimal diagnostic algorithm and clinical management of patients with biopsy‐proven EP. The differential diagnosis includes hypersensitivity myocarditis, eosinophil granulomatosis with polyangiitis (EGPA), hypereosinophilic syndrome, parasitic infections, pregnancy‐related hypereosinophilia, malignancies, drug overdose (particularly clozapine) and Omenn syndrome (OMIM 603554). To our knowledge, we report the first case of pancarditis associated to eosinophilic granulomatosis with polyangiitis (EGPA) with negative anti‐neutrophil cytoplasmic antibodies (ANCA). Treatment with steroids and azathioprine was promptly started. Six months later, the patient developed a relapse: treatment with subcutaneous mepolizumab was added on the top of standard therapy, with prompt disease activity remission. This case highlights the role of a multimodality approach for the diagnosis of cardiac involvement associated to systemic immune disorders.

Eosinophilic granulomatosis with polyangiitis: Diagnostic and therapeutic challenges during COVID-19 pandemic

Case report-IntroductionCOVID-19 pandemic affected medical practise significantly and caused difficulties in accessing necessary investigations at the appropriate time. As of March 2020, NHS England issued measures to redirect staffs and resources in preparation for the rising cases of coronavirus. As a result of this, non-urgent tests/treatments were put on hold. We present a new case of EGPA admitted to our district general hospital during the COVID-19 pandemic to highlight the challenges faced. The diagnosis was reached based on clinical judgment in the absence of some confirmatory tests as well as the decision of starting immunosuppressant treatment during the pandemic.Case report-Case descriptionA 41-years-old lady with a background of well-controlled asthma, presented with five days history of paraesthesia and swelling in both legs. She also reported mild pleuritic chest pain, which radiated to her left arm. Physical examination revealed left foot drop. She had reduced sensation on the L5-S1 dermatomal distribution with absent ankle reflex and reduced knee reflex of her left leg. Her left calf was swollen and tender. The rest of her examination was unremarkable.Baseline blood revealed raised WCC of 19.3 with significant eosinophilia (10). CRP and ESR were 135 mg/L and 48mm/hr, respectively. Electrocardiogram showed new T-wave inversion in the anterolateral leads with significantly raised troponin levels. There was ground glass appearance in both lungs, keeping with suspected COVID-19 and no evidence of pulmonary embolus was found on CTPA. MRI spine confirmed no evidence of cauda equina compression. Deep vein thrombosis was also excluded with US doppler.She was treated as myocarditis and pneumonia secondary to probable COVID-19 infection. Echocardiogram revealed severe LVSD (EF < 35%) with no LV hypertrophy. Three days later, she became acutely breathless and required high flow oxygen. New bilateral basal crackles were found on auscultation. Her antibiotic regimes were escalated to intravenous infusion.A revised CT report suggested the findings may correlate with eosinophilic pneumonia or EGPA. MRI of lower legs proved muscular oedema in bilaterally, which was suggestive of myositis with fasciitis. There was no significant change on the thigh musculature. CK level was slightly elevated (403 IU/L). Urinalysis was positive for blood (3+). Given the strong clinical suspicion of EPGA, a decision to start high dose steroid therapy was made, despite the pending immunology results. After the third dose of the methylprednisolone, pulsed cyclophosphamide was started along with high dose oral prednisolone. The patient was discharged home following significant clinical improvement.Case report-DiscussionThis patient has fulfilled 4 out of 6 criteria of ACR 1990 classification for EGPA, which are eosinophilia, bronchial asthma, mononeuritis multiplex and pulmonary infiltrates on radiological images. However, in the context of current pandemic, these changes on chest CT findings could also be suggestive of COVID-19 pneumonitis.At present, there is no reliable test for COVID-19. Even though RT-PCR testing has been the gold standard for diagnosing suspected cases, the clinical sensitivity and specificity of these tests are variable. A negative test may not rule out infection. In our case, the patient was tested twice at separate times to rule out the possibility of COVID-19 infection.During the pandemic, there is extremely limited access to some confirmatory tests. We were not able to perform nerve conduction studies on our patient as the service was suspended, instead, we sought neurologist's review to confirm the mononeuritis multiplex. We also sought advice from haematologist to rule out the possibility of hyper-eosinophilic syndrome as bone marrow biopsy was unavailable. The screen for atypical pneumonia, aspergillosis, viruses, and tuberculosis were negative. By excluding the alternative diagnoses related to eosinophilia, we concluded that this was likely to be a case of first presentation EGPA.Our next obstacle was intr ducing remission-induction regimens during COVID-19 pandemic. BSR does not recommend starting new treatment due to the increased risk of infection. We had to weigh out the benefits and risks of initiating immunosuppression. Our patient was made aware of the potential risks involved which include severe infection with COVID-19. She was also shifted to a side room with strict infection control precautions and PCP prophylaxis prescribed before starting pulsed methylprednisolone and cyclophosphamide. Fortunately, her neurological symptoms resolved after three days of steroid therapy. Eosinophils count dropped within 1 day to zero, after the first dose of IV methylprednisolone.Case report-Key learning pointsDespite the rising cases of COVID-19 infection, it is essential to keep an open mind and consider alternative diagnosis if a patient did not respond to conventional treatment. As EGPA and COVID-19 pneumonia share similar clinical and radiological presentation, clinical judgement is essential when making the diagnosis as the treatments for both conditions are vastly different. When EGPA is suspected, a multidisciplinary team should be involved in the evaluation of different organ involvements as well as ruling out other causes of eosinophilia. The role of specialists' inputs is extremely important in reaching the diagnosis, especially with limited access to the usual confirmatory tests due to reduced services during the pandemic.In addition, when there is an increased risk of infection such as during the COVID-19 pandemic, it is essential to weigh up the benefits and risks of commencing immunosuppressant treatment carefully. Patients need to be involved in the decision-making process as well as take precautions to minimise the risk of infection. The decision to start remission induction regimes should not be delayed if there is a presence of life or organ threatening disease manifestations in EGPA patients. Our patient has had a life-threatening disease because of multi-organ involvements (cardiac, pulmonary, and neurological systems).

Transient left ventricular clot in COVID-19-related myocarditis is associated with hypereosinophilic syndrome: a case report.

Frequent clinical presentations have been reported in patients with Coronavirus disease 2019 (COVID-19). It may be associated with multi-organ and cardiovascular involvements such as myocarditis and clot formation. Hypereosinophilic syndrome (HES) is a rare disease diagnosed with idiopathic eosinophilia and organ involvement. Here, we report a patient with COVID-19 who presented with clot formation and myocarditis. One month after discharge, regarding persistent peripheral/bone marrow hypereosinophilia and clot in echocardiography, fluorescent in situ hybridization (FISH) analysis was done that showed FIP1L1-CHIC2 fusion (PDGFRɑ rearrangement) in 18% of scored cells and PDGFRß rearrangement in 12% of scored cells, which confirmed HES diagnosis. Clot formation may be a late manifestation of COVID-19 or myocarditis due to COVID-19, or the first manifestation of HES that COVID-19 might provoke in this rare syndrome.

COVID-19 Pneumonia or Hypereosinophilic Syndrome?

Hypereosinophilic syndromes (HESs) are a group of disorders characterized by pathological proliferation of eosinophils. Diagnostic criteria include eosinophil count of 1,500/mm3 or higher, presence of organ involvement and exclusion of other causes of eosinophilia for at least 6 months. A 69-year-old male patient was referred to the pandemic clinic with a preliminary diagnosis of coronavirus disease 2019 (COVID-19) with fever and dyspnea. Computed tomography (CT) was compatible with COVID-19, nasopharyngeal swab polymerase chain reaction (PCR) was negative for two times. He had 20,000/mm3 eosinophilia and retrospective examinations showed that he have had eosinophilia for more than 1 year. Platelet-derived growth factor receptor alpha (PDGFRα) resulted positively. After starting parenteral methylprednisolone with a dose of 1 mg/kg, he was discharged with oral maintenance therapy. In outpatient follow-up, it was observed that eosinophilic pneumonia completely regressed. HES may occur with multiple system and organ involvement and findings. In the differential diagnosis of patients presenting with heart failure, pulmonary involvement and eosinophilia, HES must definitely be considered.