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SESSION TITLE: Systemic Disease with Diffuse Lung Symptoms Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Rapidly progressive interstitial lung disease (RP-ILD) is a rare and potentially fatal manifestation of dermatomyositis (DM) and has considerable impact in terms of the prognosis. CASE PRESENTATION: A 52-year-old male demonstrated DM-typical rash, fever, mialgias, and mild muscle weakness 3 months after asymptomatic COVID-19 infection. Two weeks later dysphonia and progressive dyspnea appeared. Lung CT scan showed the picture of organizing pneumonia. His COVID-19 PCR test was negative multiple times. Laboratory tests revealed the following numbers: ALT 210 IU/L, AST 748 IU/L, LDH 613 IU/L, CPK 1165 IU/L, ferritin 1145ϻg/l, CRB 11 mg/l. The patient was tested positive for anti-Ro52 antibodies, while anti-synthetase and scleroderma-associated antibodies were not discovered. Anti-melanoma differentiation-associated gene 5 (MDA5) test was not available due to the lack of the necessary test systems in the country. The patient was diagnosed with DM. Combined immunosuppressive therapy was administered, including: oral prednisolone 60 mg per day and 720 mg intravenously, dexamethasone 64-24 mg intravenously per diem, ciclosporin 200 mg и cyclophosphamide 600 mg, and 3 plasmapheresis sessions followed by an intravenous immunoglobulin. As a result of the therapy, muscle weakness disappeared and CPK levels returned to normal limits, however dyspnea progressed and ferritin levels hit 3500ϻg/l. After the following 3 weeks of intensive combined immunosuppressive therapy, the patient demonstrated symptoms of severe respiratory failure (RF). CT scan showed multiple traction bronchiectasis, wide areas of ground glass opacity, pneumomediastinum and subcutaneous emphysema of a neck and supraclavicular regions. Ciclosporin was replaced with tofacitinib with the dose of 10 mg per diem, IL-6 inhibitor (olokizumab 256 mg) was injected intravenously, massive broad-spectrum antibiotic therapy was administered. RF progressed and the patient was put on mechanical ventilation. The patient died of acute RF and sepsis a week later. DISCUSSION: RP-ILD is a common manifestation of severe MDA5+ DM, which is also associated with necrotizing vasculitis and amyopathic/hypomyopathic muscle involvement. In this case acute ILD in a patient with typical DM could also have been provoked by previous COVID-19 infection. CONCLUSIONS: The courses of disease for COVID-19 and MDA5+ DM have several similarities, which means it can be the same for their pathogenesis and clinical manifestations. In spite of early screening and intensive immunosuppressive therapy in such cases, the prognosis of patients with DM and RP-ILD is still poor and is associated with high mortality. Reference #1: Wang G, Wang Q, Wang Y, et al. Presence of Anti-MDA5 Antibody and Its Value for the Clinical Assessment in Patients With COVID-19: A Retrospective Cohort Study. Front Immunol. 2021 Dec 20;12:791348. doi: 10.3389/fimmu.2021.791348. PMID: 34987516;PMCID: PMC8720853. DISCLOSURES: No relevant relationships by Lidia Ananyeva No relevant relationships by Maria Aristova No relevant relationships by Liudmila Garzanova No relevant relationships by Anna Khelkovskaya-Sergeeva No relevant relationships by Dmitry Kulikovsky
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Background: Although there have been expansion of knowledge about the course of COVID-19 in rheumatologic diseases, it still remains unclear the effect of vaccination status and variants on the disease course. Objectives: We aimed to investigate the general clinical characteristics of our patients with infammatory rheumatic disease (IRD) who had COVID-19 disease, their vaccination status and the time periods in which different variants were dominant during the disease. Methods: During the routine follow-up of our patient's with IRD, whether the patients had COVID-19 disease, when they were vaccinated (Pfzer/Biontech or Sinovac in our Country) and main clinical characteristics and their comor-bid diseases were recorded. The last patient was included in the study on January 25, 2022. They were divided into those who received insufficient or no vaccine and those who received a full dose of vaccine. The patients were divided into 3 groups according to the period they had the disease: Those who had the disease between March 2020 and June 2021accepted as '1st period patietns', the period when the Alpha and Beta variants, the initial forms of the disease, were dominant variants in populations;those who had the disease between July 2021 and November 2021, when the Delta variant dominated the World and in our country accepted as '2nd period patients';and those who had the disease in December 2021 and later, when the Omicron variant was dominant throughout the world and in our country, was accepted as ' 3rd period' patients. Results: Total 463 (294 woman) IRD patients enrolled to the study. Distrubution of these patients included Behcet's syndrome:15;familial mediterranean fever: 5 7, rheumatoid arthritis:134, Sjogren's syndrome:24, systemic lupus erythema-tosus:26, Spondyloarthritis:141, necrotising vasculitis:6 and Others:50 cases. Mean age of patients were 46±13,2 (18-83) years. 354 (77%) of our patients got sick in the 1st period, 80 (17%) in the 2nd period and 28 (6%) in the 3rd period. When patients were compared in terms of their clinical complaints in these periods, dyspnea was signifcantly higher in patients in the 1st period (1st. period 36% vs 3rd period 18%;p:0.039), but there was no difference between other complaints including lung involvement and the frequency of hospitalization (p>0.05). 53% of patients had received at least 2 doses of mRNA vaccine. 84% of the patient has had COVID19 before full vaccination with any valid vaccine. When the patients who were full vaccinated and those who were not vaccinated or inadequately vaccinated at the time of illness were compared in terms of clinical features, lung involvement frequency and hospitalization frequency, no difference was found between them. (p>0.05, for all). However, hospitalization and lung involvement were less in those who received a booster dose of any valid vaccine (p: 0.03). While the average hospitalization rate was 17% for all groups, this rate was 50% for necrotizing vasculitis and was signifcantly higher (p:0,005). The probability of lung involvement and hospitalization were found to be sig-nifcantly higher in patients using prednisolone 5mg (or equivalents) or more;(p:0.008 and p:0.000, respectively). Pulmonary involvement was signifcantly higher among patients receiving sulphasalasine (p:0.008). Among the patients on Rituximab, the probability of hospitalization was higher than those who did not (p: 0.01). There was no statistical difference in terms of hospitalization and pulmonary involvement between patients who took other drugs (p>0.05, for all). A total of 5 cases died, including 2 GPA, 1 EGPA, 1 RA and 1 FMF patients. Only 8 patients had a second history of COVID19. Conclusion: The frequency of COVID-19 among IRD cases seems to decrease over time. Full vaccination seems effective for the prevention of COVID-19. It should be recommended that IRD patients have the full dose of vaccines and boosters. The risk of lung involvement and hospitalization increases in patients using certain drugs, such as corticosteroids, sulphasalasine and ituximab. These patients should be followed more closely.
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CASE: A 48-year-old female with no medical history presented with 2 days of decreased vision in the right eye. She reported painless blurry vision that progressed to near complete vision loss. The vision loss was accompanied by one month of progressively worsening cough, body aches, and subjective fevers. She denied smoking and reported no sick contacts. Physical exam was notable for submandibular lymphadenopathy, bilateral conjunctival injection, and grossly decreased vision of the right eye. She also endorsed decreased sensation in bilateral lower extremities distally. Her initial labs showed leukocytosis (13), thrombocytosis (754), and elevated inflammatory markers (ESR 105 and CPR 359). A chest CT showed bilateral upper lobe consolidations and scattered mass like opacities bilaterally. Ophthalmic exam of the right eye revealed multiple small retinal infarctions consistent with paracentral acute middle maculopathy. A CT head was negative and TTE showed no vegetation. Additional testing revealed negative TB, COVID, and normal complements. Initial ANCA testing was negative, however a repeat test was strongly positive for ANCA with PR3 significantly elevated to 428. She was diagnosed with granulomatosis with polyangiitis (GPA) vasculitis and treated with prednisone and started induction therapy of Rituximab. IMPACT/DISCUSSION: GPA is a small-medium vessel necrotizing vasculitis and the most common anti-neutrophil- cytoplasmic-antibody (ANCA) associated vasculitis. GPA classically involves the upper respiratory tract, lungs, and kidneys referenced by the ELK criteria (ENT, Lung, Kidney) commonly used for diagnosis. ENT findings are present in 70-100% of cases with the nasal cavity and paranasal sinuses most commonly involved. Roughly 50% have pulmonary involvement on presentation, as in this patient, while only 10-20% have initial renal involvement. A prodrome of systemic symptoms including body aches and fevers is often present. GPA is closely associated with c-ANCA, with autoantibodies to proteinase 3 (PR3) positive in over 80% of cases. This patient did have prodromal symptoms yet her primary presenting symptom of vision loss was atypical. Eye involvement is not part of the diagnostic triad yet it can occur in GPA. When it does present, it usually manifests as scleritis, conjunctivitis, or uveitis. Retinal infarctions, as seen in this patient, are uncommon and make this case an atypical presentation of GPA. Additionally, ANCA positivity is related to disease activity and a negative ANCA should not exclude GPA from a differential. Not all patients will be ANCA positive on initial presentation and 10% of patients with GPA will remain ANCA negative. CONCLUSION: Providers should consider atypical presentations of GPA in addition to the classic triad of ENT, Lungs, and Kidneys. Renal manifestations are often missing initially and involvement of other systems, such as ocular, can take place. With a positive c-ANCA and high clinical suspicion, treatment should not be delayed.
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Objective: To study the radiological presentations of mucormycosis in the study cohorts.The patients were categorized as COVID-19 and diabetes millitus;COVID-19 and non-Diabetes;Non COVID-19 and non-diabetes and these cohorts were analysed. Background: There has been a recent surge of mucormycosis with COVID 19 infection particularly in patients with diabetes.Multicompartmental and extrasinonasal tissue infarction is possible without overt bone involvement and caused by the dissemination of fungal elements from the nasal cavity via perineural and perivascular routes.Fungal vasculitis results in internal carotid artery occlusion and cerebral infarction.Imaging features reflect the angioinvasive behavior of the Mucoraceae family, which cause necrotizing vasculitis and thrombosis resulting in extensive tissue infarction. Design/Methods: This was a Prospective observational study of Rhinoorbitocerebral mucormycosis cases presenting from May to July 2021 to our hospital.The clinical examination findings and neuroimaging features were assessed among the study cohorts. Results: Of the 544 patients the mean age of patients was 51.9 years with a male preponderance (71%).Uncontrolled hyperglycemia was present in 90% of all patients.Ethmoid sinus was the most common paranasal sinus involved by imaging( 86%).The most common site of extrasinus involvement was orbit (76%) and face (57%), followed by orbital apex, masticator space,pterygopalatine fossa.Intracranial extension with involvement of cavernous sinus was seen in 34% of patients, brain abscess(19.2%),internal carotid artery(17%),brain infarction(45.2%),meningeal thickening and enhancement(29.2%),bone and skull base(14%).The most common neuroradiological presentation seen in all cohorts was cavernous sinus thrombosis.The propensity for multiple cerebral abscess and skull base osteomyelitis was more in patients of Rhinoorbitocerebral mucormycosis with diabetes and COVID-19 cohort.Internal carotid artery involvement was predominantly seen in COVID-19 and non diabetic cohort.Limited sinonasal disease was more commonly seen in Non-COVID-19 and diabetes patients of mucormycosis Conclusions: Central to early diagnosis is a high index of suspicion by the clinician and the radiologist.The cavernous sinus was the most common site of intracranial involvement in our study.