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The problem of prevention of coronavirus disease 2019 (COVID-19) in patients with immune-mediated inflammatory rheumatic diseases (IMRD) remains highly relevant. The presence of IRD is associated with a high risk of disease and severe course of COVID-19 during immunosuppressive treatment, primarily anti-B cell therapy with rituximab (RTX), and a low level of post-vaccination response in such patients. A new strategy for the prevention and treatment of COVID-19 are virus-neutralizing monoclonal antibodies to coronavirus;currently, combined long-acting monoclonal antibodies tixagevimab and cilgavimab (Evusheld) are registered for prevention in the world and the Russian Federation. . Tixagevimab and cilgavimab (TC) show neutralizing activity against SARS-CoV-2, including the Omicron strain, primarily its variants BA.4, BA.5, BA.2.75 ("Centaur"). Objective - to evaluate the efficacy and safety of TC for pre-exposure prophylaxis of COVID-19 in rheumatic patients receiving RTX, based on a prospective observational study. Materials and methods. The main group included 86 patients with various IMRD receiving RTX: 50 of them had ANCA-associated systemic vasculitis (AAV), 15 - rheumatoid arthritis, 9 - Sjogren's syndrome (SS), 4 - IgG4-related disease, 3 - systemic lupus erythematosus (SLE), 3 - dermatomyositis (DM), 2 - systemic scleroderma (SSD). Median age was 59 (19-82) years;male: female ratio - 1:1,8. From March 26 to August 30 2022, patients received a single intramuscular injection of TC in a total dose of 300 mg, mainly after RTX (in 52% of cases, in 28% on the next day after RTX). The control group included 42 patients with AAV (median age - 45 (35-71) years;male: female ratio - 1:1), also treated with RTX, who did not receive pre-exposure prophylaxis of TC. The duration of observation was 7 months, until November 1 2022. At this time, 98% of confirmed cases of coronavirus in the Russian Federation were Omicron. A telephone and/or online survey of patient has been conducted to detect cases of COVID-19 and adverse reactions. Results. In the TC group, confirmed coronavirus infection have been detected in 17 (20%) patients (AAV - 10, SS - 3, SSD - 2, SLE - 1, DM - 1), with fever in 7 (8%), only in one case hospitalization was required (lung damage was not detected in computed tomography), in two cases, according to CT mild lung damage (CT 1-2), there were no deaths. Good TC's tolerability was noted, signs not associated with COVID-19 or progression of IMRD after administration of TC were observed in 8 (9%) patients (GPA - 3 MPA - 1, RA - 2, SLE - 1, IgG4-related disease - 1), adverse reactions definitely associated with the use of TC were not found. The most serious event not associated with coronavirus infection was the progression of polyneuropathy in a patient with RA. In the control group, 3 (7%) patients were diagnosed with COVID-19, one with severe lung injury (CT 3, pulmonary embolism) and death. Conclusions. The data of clinical studies and our own clinical experience evidence the effectiveness of the use of a combination of long-acting monoclonal antibodies TC (Evusheld), registered for indications for pre-exposure prophylaxis and treatment of COVID-19. Patients with IMRD treated with RTX have a favorable safety profile of TC. The introduction of virus-neutralizing monoclonal antibodies, a new drug class for the prevention and treatment of infectious diseases, opens significant prospects for improving the prognosis of patients with IRD.Copyright © 2023 Ima-Press Publishing House. All rights reserved.
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Coronavirus disease 2019 (COVID-19) possesses a substantial challenge for rheumatologists and rheumatologic patients. They are concerned about the reciprocal interaction between connective tissue diseases, such as systemic lupus erythematosus (SLE), and the virus. Here, we report a 21-year-old female SLE patient presented to the emergency department with gastrointestinal symptoms and kidney involvement evidence. Based on the pathology and laboratory assessments, she was suspected of C-anti-neutrophil cytoplasmic antibody (ANCA) positive SLE and ANCA-associated vasculitis overlap syndrome (SLE/AAV OS), and plasmapheresis every other day was performed due to this diagnosis alongside the high titer of C-ANCA. We also administered methylprednisolone (1 g/day, IV) for three days, followed by dexamethasone with the maintenance dosage (1mg/kg/day, IV). Although the patient's general condition improved the next days, her condition deteriorated suddenly on the 7th day of hospitalization. She got intubated and went to the intensive care unit. Despite taking possible measures to manage the patient's condition, she eventually passed away due to severe acute respiratory distress syndrome (ARDS), triggered by COVID-19. The distinct role of C-ANCA in SLE/AAV vascular damage and activating neutrophil cytokine release accompanied by the impaired immune system while facing COVID-19 seems to lead to increased morbidity and mortality in such patients. This report was presented to bring into consideration the possible role of C-ANCA in the burden and prognosis of COVID-19 in SLE/AAV OS patients.
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Objectives: To describe the clinical characteristics and outcomes of SARSCoV-2 infection in patients with systemic vasculitis. Method(s): Observational, multicenter, cross-sectional analytical study in patients 18 or older diagnosed with systemic vasculitis with confirmed SARSCoV-2 infection (RT-PCR or serology) included in the SAR-COVID registry. Patients were evaluated from July 2020 to February 2022. Patients diagnosed with ANCA-associated vasculitis (AAV), other systemic vasculitides (Giant cell arteritis, Takayasu), and a control group of patients with other rheumatological diseases matched by age, sex, comorbidities, and date of SARS-CoV-2 infection. The survival curve of the groups was studied by Kaplan-Meier and compared through the Log-Rank Test. A Cox regression model will be performed to adjust survival for different variables (sex, age, treatments for underlying disease, treatments for viral infection, smoking, obesity, d-dimer level, and disease activity). Result(s): A total of 282 out of 2694 patients in the SAR-COVID registry were included, 57.4%women with a mean age of 55.7 years (SD 14.1). Fifty-four patients in the AAV group, 32 in the other vasculitis group, and 196 controls were studied. Hospitalization was required in 53.7% of the AAV group, 37.5% in other vasculitides, and 26.2% in the control group. 5.6% of patients in the control group presented acute respiratory distress syndrome (ARDS), 15.6% in the other vasculitis group, and 22.2% in the AAV group (p alpha 0.001). Complete recovery was observed in 82.3% of patients in the control group, 75%in the other vasculitis group, and 63%in the AAV group.We observed that 5.7% of the patients in the control group died from COVID-19, 9.4%from other vasculitides, and 27.8% in the AAV group (p alpha 0.001). We found a lower survival in the AAV group compared to the control group (p alpha 0.005). In the multivariate Cox regression model, older age (HR:1.05 IC95%1.01-1.09 p = 0.01), BMI > 40 (HR:13.2 IC95% 2.1-83.2 p = 0.01), and high activity of the underlying disease (HR:16 95% CI 3.7-69.4 p alpha 0.005) were associated with lower survival. Conclusion(s): In conclusion, patients diagnosed with AAV presented a worse disease course during SARS-CoV-2 infection with a more frequent requirement for invasive mechanical ventilation. Likewise, these patients showed lower survival compared to patients with other autoimmune diseases.
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Background/Aims Giant cell arteritis (GCA) is the most common vasculitis in adults aged over 50 years old with the highest incidence among persons aged 70- 79. It is more commonly seen in female patients. Most cases have been reported in whites of Northern European descent. A broad range of symptoms can be reported including headache, jaw or tongue claudication, visual disturbances, PMR and other systemic features including weight loss, fever and sweats. In recent years new evidence has emerged regarding the investigation and treatment of GCA. This audit is to review the demographics, symptoms and investigations of patients who presented to the Rheumatology Department in SEHSCT with features concerning for possible GCA. Methods Retrospective collection of data from January 2020 to July 2021 using the regional Electronic Care Record NI with reference to presentations, investigation results, clinic records and follow-up letters. Results 70 patients were included (24 males and 46 females). Mean age was 72 years old. Table 1 shows the percentages of clinical symptoms reported. All patients investigated had an ESR (mean 57.8) and CRP (mean 54.1) checked. 43 patients had ANCA checked with 3 positive results. 40 patients underwent CT brain with 2 abnormalities reported unrelated to GCA. TA ultrasound was performed on one occasion with a positive result demonstrating ''halo'' sign recorded. 6 patients underwent CTPET with 3 diagnoses of LVV and 1 of PMR. 70 TAB performed with 12 positive results and 4 'suggestive' of GCA. Conclusion Our cohort of patients demonstrated demographics similar to the current global geographic trends in GCA. There are a broad range of clinical symptoms that can present in GCA, none of which are entirely specific or pathognomonic. Clinical diagnosis is based on clinical symptoms, signs and laboratory tests, each of which are imperfect markers for GCA. Our audit demonstrated that the use of additional confirmatory diagnostic tests including temporal artery ultrasound and CTPET was being under-utilized in the SEHSCT. Use of these tests may improve the diagnostic yield in this challenging condition. As a result of this audit, a quality improvement project to provide a rapid access GCA pathway is being designed. (Table Presented).
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Case report: A 63 y.o. man with known allergic rhinoconjunctivitis and mild asthma, sensitized to house dust mites, cat dander and grass and pellitory pollens, presented at the Emergency Department (ED) of our Hospital for diplopia, left temporo-parietal headache, fleeting knurled scotoma, and impaired color vision, associated with dry cough, occurring the day after the booster dose of the anti-SARS- CoV- 2 vaccine (mRNA-1273). Collecting clinical history, it emerged that 6 months before, just after the first vaccine dose (BNT162b2), he developed a progressive worsening of asthma, becoming severe and uncontrolled despite high dose inhaled corticosteroids plus long-acting beta2-agonists, montelukast and tiotropium bromide, and requiring maintenance oral corticosteroid treatment: any attempt to withdraw systemic corticosteroid was associated with exacerbations of asthma. During the access to the emergency room and the subsequent hospitalization, the following emerged: severe hypereosinophilia (12400 cells/mcl), left third cranial nerve palsy, elevated serum troponin, echocardiographic signs of acute myopericarditis with interventricular septal thickening, MRI signs of cardiac interventricular septal hypokinesia, and multiple pulmonary consolidations on CT scan. Serum ANCA was negative. The clinical presentation (asthma, third cranial nerve mononeuropathy, myopericarditis with signs of interventricular septal distress, typical lung involvement) associated with the finding of severe hypereosinophilia was suggestive of eosinophilic granulomatosis with polyagioitis (EGPA). Already in the emergency room, the patient was promptly treated with 3 boluses of methylprednisolone 1 g i.v. (1 bolus per dey). During the hospitalization he underwent the first cycle (out of 6 planned) of i.v. cyclophosphamide 1000 mg and initiated therapy with oral prednisone 75 mg/day (1 mg/kg/day). After the initiation of systemic corticosteroid therapy there was depletion of blood eosinophils, progressive reduction of serum troponin, resolution of cough and almost complete regression of 3rd cranial nerve palsy. At the time of submission, the patient was discharged and taken on an outpatient basis to continue therapy with cyclophosphamide and possibly associate mepolizumab as a steroid-sparing strategy. In conclusion, this is a case of EGPA arising after SARS-CoV- 2 vaccination. It has been described that vasculitis can be triggered by infectious episodes or vaccinations: to date only two other EGPA cases likely induced by anti-SARS- CoV- 2 mRNA vaccines have been described in the literature.
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Background: Although rarely, vaccines can stimulate the immunological mechanisms underlying immune-mediated inflammatory diseases. in patients with COVID-19 there is also evidence that high titers of autoantibodies, with variable clinical relevance, can be detected. Method(s): We describe the case of a 71-year- old lady diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) in 2010 with paraesthesia, myalgia, eosinophilia and severe asthma. After induction of remission, the patient has shown regression of the vasculitis but persistence of the uncontrolled asthma. For this reason, since February 2019 she started Mepolizumab 100 mg/month. In December 2020 she tested positive for the SARS-CoV- 2 virus, manifesting a mild form then she tested negative in January 2021. In April 2021 she was vaccinated with a single dose of BNT162b2 mRNA vaccine. After about 10 days, she started to complain arthromyalgia and after a week of gait alteration, paraesthesia, dyspnoea and worsening cough associated with chest pain. Blood tests showed an increase in creatinephosphokinase (CPK 955 U/L) and hypereosinophilia (4.3x10
ABSTRACT
Viral infections, such as cytomegalovirus (CMV) infection, may affect the clinical course of ulcerative colitis (UC). CMV can cause chronic inflammation of the intestinal mucosa. In inflammatory bowel disease, chronic inflammation caused by CMV can deter the regeneration of the mucosa of the colon. However, the relationship between CMV and inflammatory bowel disease still needs to be clarified, especially in immunocompetent patients, such as younger patients not treated with immunosuppressants. Herein, we describe our experience with a middle-aged immunocompetent female patient diagnosed with fulminant UC positive for myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA). Her initial response to high-dose prednisolone was favorable; however, remission was not achieved. Immunohistochemical staining revealed the presence of CMV. Subsequently, the patient was successfully treated with prednisolone, adalimumab, and azathioprine, along with the anti-CMV treatment comprising valganciclovir. This case shows that the presence of CMV in the mucosa and blood may make patients with UC refractory to immunosuppression; furthermore, the positivity of MPO-ANCA in patients with UC can necessitate the administration of high-dose immunosuppressants to taper the dose of prednisolone.
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Myocarditis can lead to myocardial infarction in the absence of coronary artery obstruction. We report a case of probable myocarditis, complicated by myocardial infarction with non-obstructive coronary arteries. A 19-year-old man presented with chest pain typical of myocarditis. He was a smoker but was otherwise well. Electrocardiogram revealed diffuse ST-elevation and echocardiography revealed a thin, akinetic apex. Troponin-T levels on admission were raised leading to an initial diagnosis of myocarditis being made. However, late gadolinium enhancement study on cardiac magnetic resonance imaging demonstrated transmural enhancement typical of ischaemia. Coronary angiogram was normal, leading to a likely diagnosis of myocardial infarction with non-obstructive coronary arteries. It is important to highlight that coronary assessment remains important when working up for myocarditis, as myocardial infarction with non-obstructive coronary arteries can often complicate myocarditis in cases of normal angiography. Another important lesson was on how cardiac magnetic resonance imaging provided vital evidence to support underlying ischaemia despite normal coronary angiogram, leading to a diagnosis of myocardial infarction with non-obstructive coronary arteries. Myocardial infarction with non-obstructive coronary arteries remains a broad 'umbrella' term and cardiac magnetic resonance imaging, as well as more invasive coronary imaging techniques during angiography, can further assist in its diagnosis. Our case provides a reminder that myocardial infarction with non-obstructive coronary arteries, although increasingly recognised, remains under-diagnosed and can often overlap with peri-myocarditis, highlighting the need to employ multi-modality imaging in guiding management.Copyright © The Author(s) 2021.
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Livedoid vasculopathy (LV) is a noninflammatory thrombotic disease caused by occlusion of dermal small vessels associated with systemic autoimmune disorders and coagulopathies. However, LV is often reported as being 'idiopathic', despite extensive investigation. We report a case of severe LV in an otherwise healthy 27-year-old woman, associated with parvovirus infection. The patient presented with a short history of a livedoid rash initially covering her torso, which spread to acral sites. Burning pains in the lower limb caused reduced mobility;systemically, she remained well and stable throughout. Examination revealed generalized acral skin pallor, livedoid patches of violet erythema and purpura with deep serpiginous ulcerations over extensor aspects of upper and lower limbs with a more broken/racemosa nonulcerated livedoid appearance on the trunk. On admission a transaminitisareas continued to ulcerate. Codeine was present with a creatine kinase of 1569 U L.1, but other blood test results were unremarkable including erythrocyte sedimentation rate, complement, cryoglobulins, antinuclear antibodies, antineutrophil cytoplasmic antibodies, extractable nuclear antigen, rheumatoid factor, myositis screen, antiphospholipid screen and thrombophilia screen. Parvovirus IgG and IgM were both positive and tested for, as the patient's young daughter had recently been diagnosed with 'slapped cheek disease'. Magnetic resonance imaging of the thighs showed a diffuse mild myositis;electromyography, nerve-conduction studies, barium swallow and computed tomography of the chest, abdomen and pelvis were all normal. An incisional skin biopsy was performed, which revealed a blood vessel with organizing (Solimani F, Mansour Y, Didona D et al. Development of severe pemphigus vulgaris following SARS-CoV-2 vaccination with BNT162b2. J Eur Acad Dermatol Venereol 2021;35: e649- 51) have been reported. The main proposed mechanisms for AstraZeneca vaccine-induced pemphigus could be a hyperimmune reaction in genetically predisposed individuals, with eventual formation of anti-desmoglein antibodies. An alternative hypothesis is that vaccine components could act as foreign antigens resulting in a cross-reaction with pemphigus antigens. The close association of COVID-19 vaccination with the acute onset of pemphigus in our patient, as well as exacerbations after subsequent vaccine administration, is more than coincidental. Considering the recent pandemic with COVID-19 and the widespread administration of the COVID-19 vaccine, continued observation and documentation of true adverse events is essential.
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An 86-year-old woman presented to the emergency department with acute shortness of breath. She was treated with intravenous furosemide for acute-on-chronic heart failure. Her past medical history included atrial fibrillation, hypertension, diverticulosis and hypothyroidism. Rivaroxaban and levothyroxine were her only long-term medications. On day 5 of hospital admission, she developed painful haemorrhagic and purulent bullae on her dorsal hands, head and neck. These evolved to large suppurative, vegetative plaques over a 72 h period and she developed additional lesions on her trunk, upper back and thighs. The patient had routine blood tests, which showed a raised C-reactive protein at 260 mg L-1, and an acute kidney injury with a glomerular filtration rate of 54 mL-1 min-1. She had a negative COVID-19 swab, and swabs from the lesions for bacterial culture and viral polymerase chain reaction were negative. She had a normal serum protein electrophoresis, immunoglobulin, antinuclear antibody and antineutrophil cytoplasmic antibody. She had computed tomography of her chest 24 h prior to the onset of her lesions, which showed mild bilateral pleural effusions in keeping with fluid overload secondary to heart failure. A biopsy taken from her hand showed orthokeratosis and parakeratosis, and there was bulla formation subepidermally. There was a dense neutrophilic infiltrate with microabscess formation with scattered eosinophils and lymphocytes. There was no evidence of vasculitis. Direct immunofluorescence was negative and a tissue culture for atypical mycobacteria was negative. The patient was commenced on high-dose intravenous methylprednisolone at 500 mg for 3 days followed by 40 mg prednisolone orally for 1 week, but there was a limited response. Our initial differential was Sweet syndrome or pyoderma vegetans;however, the patient had no fevers and no risk factors (malignancy, inflammatory disease, infection, etc.). She also had no response to high-dose oral prednisolone. Given the timing of her CT examination in relation to her acute dermatosis and the use of radioiodine for contrast, we assessed the patient's serum iodine and urine iodine. These were both high at 1.02 mmol L-1 (reference interval 0.32- 0.63) and 3.46 mmol L-1 (reference interval 0.0-2.43), respectively. A diagnosis of iododerma was made. The patient's eruption slowly resolved and at 12 weeks there was evidence of postinflammatory skin changes only. Her urine and serum iodine were rechecked, and both had normalized. In the last 20 years there have been approximately 20 case reports of iododerma. Most have been following iodine contrast use in patients with abnormal kidney function, like our patient. Most describe an acneiform eruption that subsequently evolves to vegetative plaques (Chalela JG, Aguilar L. Iododerma from contrast material. N Engl J Med 2016;374: 2477). Iododerma is largely a diagnosis of exclusion, but histopathology and urine and serum iodine levels can help support diagnosis.
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Background: Antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis presenting for the first time in pregnancy is very rare, but awareness is important as it can cause significant maternal and fetal morbidity and is potentially life-threatening if not recognised or under-treated. Method and Results:We describe a 19-year-old woman who developed ANCA-associated vasculitis in the second trimester of her first pregnancy. She initially presented with a petechial rash and cough at 25 weeks' gestation, and then developed breathlessness. Significant pulmonary haemorrhage was shown on Cross Sectional imaging of the chest, with a corresponding reduction in haemoglobin. She rapidly improved with prednisolone, cyclophosphamide and plasma exchange. SARS-CoV-2 infection identified on routine screening further complicated the management. At 34 weeks' gestation she experienced a flare, with the possibility of superimposed pre-eclampsia (increase in liver enzymes, creatinine and sFlt/PlGF ratio). After multidisciplinary team discussion she underwent a caesarean section. Postnatally she continued cyclophosphamide and started azathioprine. Conclusion(s): ANCA-associated vasculitis can result in life-threatening complications. The initial features can be non-specific, so a high index of suspicion is required, particularly in women with multisystem abnormalities. Close monitoring for potential complications is advised as urgent imaging may be needed. Aggressive immunosuppressive treatment is recommended as steroids alone are usually insufficient. Cyclophosphamide can be used in later pregnancy and can result in a dramatic improvement, as was seen here. If delivery needs to be expedited, mode of birth (i.e. caesarean delivery vs vaginal birth) is dictated by the obstetric picture, with caesarean delivery being indicated for the usual obstetric reasons.
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The coronavirus disease 2019 (COVID-19) vaccination campaign has progressed worldwide. Rare but severe adverse events of COVID-19 vaccination such as anaphylaxis and myocarditis have begun to be noticed. Of note, several cases of new-onset antineutrophil cytoplasmic antibody-associated vasculitis (AAV) after COVID-19 mRNA vaccination have been reported. In contrast, relapse of AAV in remission has not been recognized enough as an adverse outcome of COVID-19 vaccination. We report, to the best of our knowledge, a first case of renal-limited AAV in remission using every 6-month rituximab administration that relapsed with pulmonary hemorrhage, but not glomerulonephritis, following the first dose of the COVID-19 vaccine. Notably, the patient received the COVID-19 vaccine more than 6 months after the last dose of rituximab according to the recommendations. Ironically, his CD19 positive B cell counts were found to be increased after admission, indicating that our case might have been prone to relapse after COVID-19 vaccination. Although our case cannot establish causality between AAV relapse and COVID-19 mRNA vaccination, a clinical vigilance for relapse of AAV especially in patients undergoing rituximab maintenance therapy following COVID-19 vaccination should be maintained. Furthermore, the elapsed time between rituximab administration and COVID-19 mRNA vaccination should be carefully adjusted based on AAV disease-activity (Nishioka et al. Front Med 2022. in press).
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Case Report: Acute motor and sensory axonal neuropathy (AMSAN) syndrome is a rare subtype of Guillain-Barre syndrome (GBS) with poor recovery [1]. While respiratory and gastrointestinal infections may precipitate AMSAN, an underlying autoimmune disorder is seldom reported in literature. We herein report the complex case of a patient with undiagnosed, asymptomatic mixed connective tissue disease (MCTD) who developed AMSAN syndrome. Case: A 44-year-old Asian male without medical history presented with progressively worsening weakness of both upper and lower extremities and inability to perform daily activities. His symptoms started 12 weeks prior with difficulty standing from a seated position. He felt subjectively better for some time until a week prior, when he became bedbound. He had diarrhea 6 months ago, with 5-6 loose bowel movements a day for a few weeks. Vital signs on admission was normal. On neurological examination, he was alert and oriented, with bilateral upper and lower extremity flaccid paralysis, diffuse muscle atrophy, bilateral hand and foot drop, negative Hoover sign, diffuse areflexia, and intact sensation. Cerebrospinal fluid (CSF) analysis showed WBC 0 and protein level 136. MRI cervical, thoracic, and lumbar spine were normal. EMG revealed sensory involvement with positive sharp waves in proximal muscles along with fibrillations. Intravenous immunoglobulin (IVIG) was initiated at 0.4 mg/kg for 5 days. Infectious workup for COVID-19, stool culture, HIV, TB, RPR and campylobacter jejuni antibody (Ab), was negative. ANA was positive in a speckled pattern with titres 1:1280, with a positive RNP Ab, SS-A, and RF IgM, IgG and IgA. Rest of the autoimmune workup (anti-dsDNA, anti-CCP, SS-B, aldolase, anti-Jo-1, anti-Scl-70, p-ANCA, c-ANCA, anti-GM1, anti-GQ1b, and anti-GD1a ganglioside Ab) was negative. The myositis specific 11 Ab panel was negative. Despite IVIG therapy, he developed dysphagia, respiratory distress, with a negative inspiratory force of -0, requiring intubation. He had a tracheostomy and PEG tube placed and remains quadraplegic nearly 120 days later. Discussion(s): The authors report a unique case of a patient who became progressively weak over 3 months, leading to complete quadriplegia. Interestingly, this is more consistent with chronic inflammatory demyelinating poly-neuropathy (CIDP), as AMSAN typically develops over a short period of 2 to 4 weeks [2]. Despite having negative anti-GM1 and anti-GD1a Ab (in which positive Ab are pathognomonic but not always present in AMSAN syndrome), the patient had weakness that began in the lower extremities, progressing to paralysis, along with albuminocytological dissociation on CSF analysis, pointing to a GBS diagnosis [3]. He had sensory involvement in the EMG, thus making the diagnosis as AMSAN. He had an undiagnosed, asymptomatic autoimmune process most consistent with MCTD. Whether the two disease processes are related to each other is a concept that has not yet been investigated. Pediatric Clinical Case Reports Concurrent Session Saturday February 4, 2023 1:00 PM Copyright © 2023 Southern Society for Clinical Investigation.
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Introduction/Background: Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss Syndrome, is a rare, small to medium vessel ANCA associated vasculitis. Hallmarks of EGPA include asthma, chronic rhinosinusitis, and peripheral neuropathy. EGPA is characterized by a prodrome of asthma and allergic rhinitis, followed by peripheral blood hyper-eosinophilia and accumulation of extravascular eosinophils, and finally systemic vasculitis. Extrapulmonary involvement is common, sometimes with fatal outcomes. The onset of EPGA is typically between 25-50 years;however, EGPA also occurs during childhood and has a significant morbidity and mortality. Description/Method: Our patient presented to the emergency department with a 2-week history of lethargy, wheeze and left sided neck swelling. After testing COVID-19 positive eight months prior to this, she developed wheezy episodes and was subsequently diagnosed with asthma which was managed with bronchodilators as required. She was reviewed by an allergist who confirmed a dust mite allergy and prescribed Montelukast. She remained well during the summer months however during winter she had 3 distinctive episodes of wheeze and cough which were managed by antibiotics and prednisolone. In the emergency department, an echocardiogram was performed which showed a cardiac tamponade. She was transferred to CICU where she had a pericardial drain inserted. The fluid was abundant with inflammatory cells. Multiple investigations were performed as follows: Hb: 135g/L, wbc: 20.30 x 10 9/L, Eosinophils: 12.77 x 10 9/L, CRP: 51 mg/L, ESR: 75 mm/hr, LDH: 1188 IU/L, IgE: 8000 UI/ml, ANA, ANCA: negative. CT chest showed mediastinal lymphadenopathy and patchy bilateral infiltrate and cardiac MRI showed myopericarditis and LV fibrosis. BMA showed no malignant cells and sinusitis was confirmed by CT. On examination, she was underweight. Her nasal mucosa looked inflamed. Otherwise systemic examination was unremarkable. In the context of poor ejection fraction (20%) with LV fibrosis, urgent MDT was arranged and concluded that our working diagnosis was EGPA. The decision was made to start IV methylprednisolone 10mg/kg/day for 3 days and Ivermectin. That night our patient had a VF arrest which required a single shock conversion 4J/kg. There was 7-minute downtime. Treatment was escalated to include cyclophosphamide, rituximab and plasmapheresis. The patient made a remarkable recovery, extubated and transferred to a normal ward. Her eosinophils count and inflammatory markers improved dramatically following treatment. However, she developed severe neuropathic left leg pain and NCS confirmed peripheral neuropathy Discussion/Results: EGPA is a very rare disease and diagnosis can be challenging especially with the absence of histopathology diagnosis. Early empirical treatment especially in a very ill child in intensive care unit can save lives and divert the progress of the disease. This patient has fulfilled the American College of Rheumatology criteria to diagnose EGPA including asthma, eosinophil count > 10% of upper normal, peripheral neuropathy, pulmonary infiltrates on CT thorax and paranasal sinuses abnormalities. Cardiac biopsy of the fibrotic mass may be a useful tool for diagnosis;however, this invasive procedure may expose this patient with high risk of fatal arrhythmias. Since other causes of eosinophilia were ruled out including parasitic infections, lymphoproliferative disorders, and rare primary immunodeficiency syndromes (hyper-IgE syndrome due to STAT3 or DOCK8 deficiency and Omenn syndrome) and the patient responded well to treatment, the diagnosis of EGPA was supported. Key learning points/Conclusion: Asthma not responding to bronchodilator could be another diagnosis Eosinophilia should be interpreted with caution. Defer the need for histopathology diagnosis in critically ill children Cardiac involvement is a life-threatening marker Early diagnosis prevents life threatening complications.
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Background: The Covid-19 vaccines administration programs implemented in most countries, WHO showed 5.22 billion persons vaccinated at least one dose up to June 29th 2022. EULAR and ACR guidelines suggested autoimmune and inflammatory rheumatic diseases (AIIRDs) patients with stable or low disease activity should receive Covid-19 vaccination. ANCA-associated vasculitis (AAV) patients are AIIRDs subgroup during pandemic, the safety of Covid-19 vaccination has not been reported in large scale study. We analyzed publications of patients with AAV who had relapse post-vaccination and further discussed the challenges for AAV patients under B-depleting therapy with appropriate vaccination recommendations. Method(s): We used descriptive thematic analysis to find out the flare features including severity, organ involvement, therapy strategy in AAV patients after Covid-19 vaccination. This article conforms with the Scale for Assessment of Narrative Review Articles (SANRA) guidelines. After eligibility criteria and selection process, there were 6 matched articles which had 10 individual cases. Result(s): AAV could involve several organs throughout the body, most cases involved kidney and lung injury. Among the 10 cases, there were 2 cases of EGPA, 3 of MPA and 1 RLV patient with clearly written diagnosis and all patients were assessed as being in disease remission prior to vaccination. The cases were special in age characteristic with 71-85 years old. Seven patients had anti-ANCA type test in 3 cases were anti-PR3 type and another 4 cases were anti-MPO type both with higher titers than during remission. Four had new onset organ involvements rather than the recurrence of the original organ relapse after vaccination. Among them, the 74-year- old male suspended rituximab for 6 months interval before vaccination, he developed diffuse alveolar hemorrhage and infection followed under restart rituximab treatment and eventually died of respiratory failure. Another same age male was diagnosed with new crescentic pauci-immune glomerulonephritis and discharged with creatine level as 307umol/L. Conclusion(s): Patients with AAV in remission rarely experienced severe relapse after vaccination, and some involved new-onset organ while others are worsened of the original involvement. Pulmonary new onset involvement might be triggered by neutrophil extracellular traps promotion. The elderly receiving B-cell depleting therapy who are at infection risk with low humoral response should be under careful evaluation between last dose of rituximab and next dose of vaccination. Kidney injury presented mostly with good clinical treatment response. However, longer observation should be considered whether those patients received kidney replacement therapy earlier than expected. (Figure Presented).
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Case report - Introduction: Bacterial community-acquired atypical pneumonia is sometimes complicated by myositis or by renal parenchymal disease. They can present with myositis and present with muscle weakness, pain or swelling, and elevated muscle enzymes. We present the case of a patient with lower limb weakness and raised creatinine kinase with atypical pneumonia caused by Legionella pneumophila. Case report - Case description: A 76-year-old Caucasian man, who was previously fit and independent and walked 3 miles every day presented with a 1-week history of progressive leg weakness, and inability to mobilize. He had a fall and was on the floor for 2 hours. He had a background history of hypercholesterolemia and was on atorvastatin for 15 years. On his vital observation, he was found tachypnoeic, tachycardic, and hypoxic. He had a right upper lobe crackle but he didn't have respiratory symptoms. His muscle power in his leg was 3/5 with carpet burns on knees and elbow. Initial investigation showed raised inflammatory marker CRP 412mg/L, AKI stage 1, and CK 43400 IU/L. His CXR showed dense right upper lobe consolidation. Legionella urinary antigen was positive. Myositis myoblot, ANA, ANCA negative. COVID-19 swab negative. Treated with IV antibiotic, supplemental oxygen, and IV fluid. Transferred to ITU due to worsening of hypoxia and kidney function. Interestingly, the CK level had improved significantly within 48 hours along with clinical improvement in his symptoms. There was no role of steroid or immunosuppressant due to his significant clinical improvement. On day 7 he was off oxygen, kidney function improved, had physiotherapy, and transferred to ward and on day 10 he was ambulant and discharged home. Case report - Discussion: To date, very few case reports of myositis in a patient with atypical pneumonia have been reported. The mechanism underlying acute myositis in atypical pneumonia is still unknown. The present analysis points out that the organism underlying atypical bacterial pneumonia may occasionally invade the muscle tissue thereby inducing both myositis and secondary kidney damage. Case report - Key learning points: We should be aware of this rare complication of atypical pneumonia and the resolution of symptoms that occur with the treatment of pneumonia. This would avoid unnecessary investigation and use of steroid.
ABSTRACT
Purpose of Study: Report a rare case of onset of seronegative, juvenile dermatomyositis likely potentiated by Covid-19 infection Methods Used: Case analysis and literature research Summary of Results: A 7 year-old previously healthy male presented with 3 weeks of progressive, bilateral upper and lower extremity weakness, difficulty swallowing, voice changes, periorbital edema, and rash. Recent history was notable for diagnoses of COVID-19 one month prior to presentation and streptococcal pharyngitis 2 months prior to presentation. Notably, there is a family history of systemic lupus erythematosus. On examination, the patient demonstrated bilateral periorbital swelling with purple discoloration of the upper eyelids, a violaceous, pruritic, macular rash on his upper extremities and on his abdomen. Musculoskeletal exam was significant for severe axial (strength 2/5) and proximal (strength 3/5) muscleweakness with notable inability to sit unsupported or maintain head control. His neurologic exam was nonfocal;however, diffuse hyporeflexia in both upper and lower extremities were elicited. Initial screening labs were notable for mild transaminitis;positive ANA (1:80 in speckled pattern), negative ANCA, negative dsDNA/Anti- Sm, elevated aldolase of 10.3, CK 464, and LDH 665;normal thyroid studies and normal inflammatory markers. MRI with and without contrast of the spine indicated diffuse myositis of all muscle groups. Due to concern for autoimmune mediated myositis, Rheumatology was involved early in the patient's course. Empiric treatment was initiated early in the patient's presentation with IVIG, steroids, methotrexate, and plaquenil leading to gradual improvement in symptoms. Subsequent muscle biopsy was consistent with juvenile dermatomyositis (JDM). Conclusion(s): JDM is rare, occurring in 1 to 15 per million children. It classically presents with proximal myopathy and dermatologic findings of Gattron's papules, a heliotrope and malar rash. Its pathophysiology is not yet well defined but is thought to be a humoral mediated autoimmune disease. Muscle biopsies characteristically show perifascicular and perivascular infiltration. Early diagnosis and treatment with steroids, immune modulators, and physical therapy is critical to limit muscle atrophy. Viral infections are known triggers of rheumatologic diseases broadly;however, the more pronounced type 1 interferon response associated with COVID-19, which is known to be a driving pathway of JDM, may be a risk factor for severe, recalcitrant disease. Future research is needed to better identify involved pathophysiology and target future treatment efforts. Additionally, more education and case reports could focus on dermatologic presentations of individuals with pigmented skin. Copyright © 2023 Southern Society for Clinical Investigation.
ABSTRACT
Background/Purpose: Kikuchi-Fujimoto disease (KFD) is a rare, self-limited histiocytic necrotizing lymphadenitis. Although it is of uncertain aetiology, it is associated with viral infections and autoimmune diseases. Hence, it is crucial to identify KFD from other conditions with lymphadenopathy. Here we present a case of KFD after COVID-19 infection. Method(s): Medical records were traced and reviewed Results: A previously healthy 13-year- old girl was admitted in April 2022 with four weeks of fever, dry cough, loss of weight, followed by 1 week history of painful cervical lymphadenopathy and nonspecific maculopapular rash. She received her second dose of Covid 19 vaccine in January 2022. Unfortunately, she was diagnosed with CAT II, COVID 19 infection in March 2022. There was no history of allergy, recent traveling and cat scratch injury. Clinically there was no strawberry tongue, erythema of the lips, conjunctivitis or distal extremities changes to suggest Kawasaki disease. She was initially diagnosed with infection related lymphadenitis, treated with oral azithromycin for three days and intravenous ceftriazone for one week with no improvement. Her laboratory results showed hypochromic microcystic anaemia with leucopenia, raised inflammatory markers and lactate-dehydrogenese levels. Extensive workup for infection was unremarkable. Immunology test showed ANA, ANCA, ENA were negative with normal complements. Ultrasound abdomen was normal. Excisional lymph node biopsy revealed confluent areas of necrosis surrounded by histiocytes (CD68+) with absent of neutrophils. No granuloma or atypical lymphoid cells seen. Based on histopathology report, diagnosis of KFD was established. As she was not able tolerate orally, IV hydrocortisone was started and subsequently switched to oral prednisolone. She responded well to corticosteroids with fever subsided within a day and cervical lymphadenopathy reducing in size and resolved in one month. Prednisolone was able to taper off by two months. She showed complete recovery with no recurrence during follow-up. Conclusion(s): In persistent febrile painful lymphadenopathy, excision lymph node biopsy is essential to establish definite diagnosis. This case highlights the possible association between COVID-19 and KFD.