Outcomes and Impact of Covid-19 on Disease Activity in Patients with Systemic Vasculitides: Data from the Reuma-Cov Brazil Register

Objectives: To assess the impact of COVID-19 on disease activity and severity outcomes in patients with systemic vasculitis. Method(s): The Reuma-CoV Brazil is a longitudinal, multi-stage cohort study, designed to monitor patients with immune-mediated rheumatologic disease (IMRD) during the SARS-CoV-2 pandemic. Systemic vasculitis patients with COVID-19 were compared with those without COVID-19. Vasculitis activity was evaluated by the patient global assessment (PGA) and Birmingham Vasculitis Activity Score 3 (BVAS 3). The prognosis was assessed by the Five-Factor Score (FFS). Result(s): Between May 2020 and January 2021, 53 patients with vasculitis were included and followed for six months, 32 (60.3%) with COVID-19 and 21 (39.6%) in the control group. In total, 79.5% were female with a mean age (SD) of 49 (16.5) years. Both groups were homogeneous regarding sex, age, and comorbidities. Thirty-eight (71.8%) patients had at least one comorbidity. Thirty-two patients were classified as small vessels vasculitis (SVV), 10 as large vessels (LVV) and 11 as vasculitis of variable caliber. There was no difference in PGA, BVAS and FFS when comparing before and after SARSCoV-2 infection (Table 1). In the group of patients with LVV, two had clinical or laboratory worsening post infection. Compared to controls, patients with vasculitis and COVID-19 were at higher risk of intensive care unit (ICU) hospitalization [OR (IC95%) = 7.98 (3.78 - 16.8), p alpha 0.001], mechanical ventilation [OR (IC95%) = 7.45 (3.16 - 17.5), p = alpha0,001] and death [OR (IC95%) = 9.69 (3.87 - 24.3), p alpha 0,001]. Of the 7 patients who died, 40%were using high-dose prednisone (>20 mg/d) and 38.8% were using rituximab. Conclusion(s): In this sample of patients with systemic vasculitis, there was no worsening of disease activity after COVID-19, but there was a higher risk of poor outcomes, possibly related to immunosuppression.

Safety and Efficacy of Covid-19 Immunization in Systemic Vasculitides - a Multicentric Cohort

Objectives: Patients with immune-mediated rheumatic diseases (IMRDs) develop more severe outcomes of Coronavirus disease 2019 (COVID-19). Recent studies have contributed to understand the safety and efficacy of COVID-19 vaccines in IMRDs, suggesting that different diseases and therapies may interfere on immunization efficacy. In this study we analyze the immunogenicity of COVID-19 vaccines in patients with Systemic Vasculitides (VASC), the rate of COVID-19 and the frequency of disease relapse following immunization. Method(s): We included patients with VASC (n = 73), a subgroup of the SAFER study (Safety and Efficacy on COVID-19 Vaccine in Rheumatic Disease), a longitudinal, multicenter, Brazilian cohort.We analyzed the geometric means of IgG antibody against receptor-biding domain of protein spike of SARS-CoV-2 (anti-RBD) after two shots of CoronaVac (Inactivated vaccine), ChadOx-1 (AstraZeneca) or BNT162b2 (Pfizer-BioNTech). IgG anti-RBD was measured by chemiluminescence test. We assessed new-onset COVID-19 episodes, adverse events (AE) and disease activity for each VASC. Result(s): The sample included Behcet's disease (BD) (n = 41), Takayasu arteritis (TAK) (n = 15), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (n = 14), polyarteritis nodosa (n = 7) and other small vessel VASC(n = 6). The majority of patients were female (69%) without comorbidities (49%) and a median age of 37 years. The most common medication was conventional synthetic disease-modifying anti-rheumatic drugs, followed by biologic drugs. No patient received rituximab at baseline. Most patients received CoronaVac (n = 25) or ChadOx-1 (n = 36), while four received BNT162b2. Baseline IgG-RBD means were 1.34 BAU/mL. They increased to 3.89 and 5.29 BAU/mL after the 1st and 2nd vaccine dose, respectively. ChadOx-1 had higher antibody titers than CoronaVac (p = 0.002). There were no differences between different VASC. There were 3 cases of COVID-19 after immunization with CoronaVac. BD patients had a tendency for more cutaneous-articular activity following ChadOx-1. There were no severe relapses and no serious adverse events. Conclusion(s): Our results show the safety of different SARS-CoV-2 vaccines in VASC population. A progressive increase of IgG-RBD antibodies was observed after each dose. ChadOx-1 led to higher IgG-RBDgeometricmeans compared toCoronaVac. Finally, even though ChadOx-1 presented a tendency of triggering mild disease activity, there were no significant disease activity following vaccination in VASC patients. .

A Systematic Review of New Rheumatic Immune-Mediated Inflammatory Diseases (R-Imid) Following Sars-Cov-2 Vaccinations

Background/Aims There are sporadic reports about the development of new rheumatic immune-mediated inflammatory diseases (R-IMIDs) in adults after receiving SARS-CoV-2 vaccines. This systematic review (SR) aimed to critically review and summarize the clinical profile, patient demographics, treatment, and prognosis of new-onset R-IMIDs following SARS-CoV-2 vaccination. Methods We retrieved English-language articles (Case reports and series and observational studies) on new-onset R-IMIDs following SARS-CoV-2 vaccination, published until June 2022, from standard databases (MEDLINE, Embase, Cochrane). The search strings used during the literature search incorporated 'SARS-CoV-2 vaccination' (along with related MeSH terms) and various key terms for R-IMIDs [which included (but was not limited to) inflammatory arthritis, connective tissue disease (CTD), vasculitis, systemic lupus erythematosus, Sjogren's syndrome, sarcoidosis, systemic sclerosis, idiopathic inflammatory myositis, anti-synthetase syndrome, Adult-onset Stills disease (AOSD), giant cell arteritis (GCA), and polymyalgia rheumatica (PMR)]. The protocol was registered in PROSPERO (CRD42022318561). Results Of the total 2179 articles retrieved, 1986 articles were excluded following the title- screening, and 107 articles that did not meet inclusion criteria. We included the remaining 86 articles (130 cases) upon full-text screening. Furthermore, we added four articles (six cases) based on a manual search, comprising 90 articles (136 cases) for final analysis. These 136 new R-IMID cases were reported from 27 different countries. Of these, more than one-third of the cases were reported from three countries (viz., Italy, Japan, and the USA). The patients had a mean age of 57 (range:17-90) years, and the majority were females (63.0%). Most patients developed R-IMIDs after receiving Pfizer-BioNTech vaccine (76;55%), followed by Oxford AstraZeneca vaccine (35;25%). The mean duration between SARSCoV- 2 vaccination and R-IMIDs development was 9.2 (range:1-90) days. The second dose of the vaccine resulted in more R-IMIDs (74;54%) than the first (53;39%). CTDs (34;25%) and small vessel vasculitis (33;24%) were the commonest R-IMID manifestations, followed by inflammatory arthritis and AOSD, each in 13 (9.5%) cases. Nearly half of the patients with CTDs had Idiopathic Inflammatory Myositis. PMR and GCA accounted for 16 (11.7%) and 5 (3.6%) cases, respectively. However, no cases of axial spondylarthritis were reported. Most (118;86%) R-IMID patients were treated with corticosteroids, with a small number receiving steroid-sparing drugs, such as methotrexate, rituximab and cyclophosphamide. Most (125;91%) went into either disease remission or improvement following the treatment. Only three patients were admitted to the intensive care unit (ICU) to manage their disease;One of them died due to fatal myositis and rhabdomyolysis;two surviving ICU patients had ANCA-associated vasculitis with lung involvement. Conclusion Although rare, this SR highlights the emergence of de novo R-IMIDs following SARS-CoV-2 vaccination. We cannot confirm the causality between the vaccination and the onset of R-IMID. However, further research is warranted in this area.

COVID-19 vaccine induced vasculitis-A case report

Introduction: As of June 2021, a total of 120.2 million cases of corona virus disease 2019 (COVID19), with Seven and half lakh COVID 19 related deaths occurred, as estimated by CDC Globally. Vaccination was started by the Government of India to prevent new cases. With the ongoing pandemic, several COVID-19 vaccine agents have received emergency use approval, several adverse effects are being reported with increasing administration of COVID-19 vaccines. Finding(s): Here we describe a case of Henoch Schoenlein purpura, a small vessel vasculitis which is usually seen in children with excellent prognosis in adults, on contrary HSP developed in adult male following COVID-19 vaccination had a rapid deteriorating course. Millions of people are being vaccinated around the world, and thus it is conceivable that people may develop other diseases temporally associated with vaccination but which are unrelated to the vaccine itself. Conclusion(s): Auto Immune Diseases (AID) can be triggered by vaccine but how do they behave when compared to primary AID both in terms of presentation, prognosis and treatment response are not known, thereby we concluded that Henoch Schoenlein purpura can develop post covid vaccination in adults and may have a rapid course and poor prognosis.

An unusual presentation of malignant Invasive Mucinous Adenocarcinoma of Lung mimicking Pulmonary Renal syndrome

Background: A 54-year- old male presented to our centre with a chronic non-productive cough and breathlessness. Recent history of COVID treated and resolved few months back. He had a history of brain surgery performed five years back but details not known. Physical examination revealed no oedema and bilateral coarse creps with bronchiolar breathing. Laboratory findings indicated neutrophilic leucocytosis, elevated inflammatory markers, with elevated troponin I and D dimers. Urine analysis suggested microscopic haematuria with sediments. While 24 hour quantification revealed sub nephrotic proteinuria. As auto immune workup and vasculitis profile was negative and patient has not improved in spite of standard of therapy hence we went ahead with CT-Chest indicating ground-glass opacities in bilateral lung parenchyma and prominent interlobular/intralobular septal thickening. Then Bronchoscopy done which revealed the blood-stained secretions in the main stem bronchi and diffuse alveolar haemorrhage in bilateral bronchial segments indicating an inflammatory study, while tuberculosis diagnostic panel and infective bio fire panel in BAL was negative. Meanwhile, his repeat BAL culture suggested Carbapenem resistant Acinetobacter baumannii complex infection. As the patient did not respond to the standard of care for vasculitis. Probability considered was a small vessel vasculitis (namely Granulomatous polyangiitis) was considered due to lung manifestation involving upper respiratory tract with epistaxis, neutrophilic leucocytosis, elevated acute reactive protein, and renal manifestation including microscopic haematuria and proteinuria. However he responded poorly to conventional standard of treatment including pulse steroids and IVIG. Hence after MDT discussion we proceeded with lung biopsy which showed linear cores of lung tissue infiltrated by a malignant neoplasm and acinar pattern suggesting Invasive mucinous adenocarcinoma. Hence we went ahead with the biopsy diagnosis for the treatment plan. As he was to be started on chemotherapy, but he suddenly collapsed and went into hypotension, bradycardia, and cardiac arrest. In spite of high supports and post 4 cycles of CPR, was unable to revive and sadly succumbed to his illness. Discussion(s): In this rare case, the original diagnosis pointed to the pulmonary-renal syndrome, an autoimmune disease characterized by diffuse pulmonary haemorrhage and glomerulonephritis. However, negative autoimmune antibodies and vasculitis profile along with lung biopsy results indicated an unusual case of malignant lung adenocarcinoma presented with pulmonary renal syndrome. Conclusion(s): In cases suggesting pulmonary-renal syndromes, if autoimmune work up is negative and response is suboptimal relook the diagnosis.

A CASE OF GRANULOMATOSIS WITH POLYANGIITIS

SESSION TITLE: Critical Systemic Disease Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Granulomatosis with polyangiitis(GPA) is an autoimmune small vessel vasculitis that is included in the group of anti-neutrophilic cytoplasmic antibody(ANCA)- associated small vessel vasculitides (AAVs). GPA is a systemic disease, however acronym ELK is used to describe the most common involvement of Ear, nose, throat, Lungs, and Kidneys. We report a case of GPA, highlighting its presentation. CASE PRESENTATION: 59-year old female presented with vaginal bleeding, malaise, blurry vision, non productive cough and shortness of breath few days after receiving COVID-19 vaccine. Physical exam was remarkable for bilateral conjunctival injection with right sided ptosis and inguinal lymphadenopathy. Laboratory findings were significant for acute kidney injury and anemia. Computed tomography (CT) of chest revealed bilateral bronchovascular nodules and masses with interlobular septal thickening and enlarged mediastinal lymph nodes. Fine needle aspiration of left inguinal lymph node was negative for malignancy. Bronchoscopy with bronchial brush revealed alveolar hemorrhage with capillaritis;bronchoalveolar lavage(BAL) showed hemosiderin laden macrophages. Tissue biopsy was negative for malignancy. Testing for pulmonary renal syndrome was positive for C-ANCA and proteinase-3 (PR-3) antibodies. Anti-GBM antibody and anti-MPO antibody was negative. Plasmapheresis (PLEX) and pulse dose steroids were initiated however the patient was unable to tolerate the treatment. Her clinical condition continued to decline requiring multiple pressors, broad spectrum antibiotics and continuous renal replacement therapy. She was transitioned to comfort care per family's wishes and passed away. DISCUSSION: GPA is a rare necrotizing granulomatous vasculitis of small to medium sized vessels that can affect any organ but mainly involves the upper and lower respiratory tract. Necrotizing glomerulonephritis is common. Pulmonary involvement can include consolidation, tracheal or subglottic stenosis, diffuse alveolar hemorrhage, pleural effusion and interstitial lung disease. Lymphadenopathy, as seen in our patient is a rare presentation. Tissue biopsy is crucial for the diagnosis. Association with PR-3 ANCA is seen in more than 80% of GPA patients. Cases of AAVs after administration of COVID vaccine have been reported in the literature, although it is difficult to demonstrate causal relationship. Treatment of GPA with immunosuppression, usually corticosteroids, rituximab or cyclophosphamide, is recommended. The role of PLEX continues to evolve with emerging data, but use of this therapy is reasonable for patients with severe kidney injury and DAH secondary to active vasculitis refractory to immunosuppressive therapy. CONCLUSIONS: Early diagnosis of GPA is challenging as it can mimic metastatic lung malignancy. It should be considered in a broad range of differentials when evaluating patients presenting with pulmonary nodules. Reference #1: Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016 Jun;29(2):151-9. doi: 10.1177/0394632015617063. Epub 2015 Dec 18. PMID: 26684637;PMCID: PMC5806708. Reference #2: Kitching, A. R., Anders, H. J., Basu, N., Brouwer, E., Gordon, J., Jayne, D. R., Kullman, J., Lyons, P. A., Merkel, P. A., Savage, C., Specks, U., & Kain, R. (2020). ANCA-associated vasculitis. Nature reviews. Disease primers, 6(1), 71. https://doi.org/10.1038/s41572-020-0204-y Reference #3: Szymanowska-Narloch, A., Gawryluk, D., Błasińska-Przerwa, K., & Siemińska, A. (2019). Atypical manifestations of granulomatosis with polyangiitis: the diagnostic challenge for pulmonologists. Advances in respiratory medicine, 87(6), 244–253. https://doi.org/10.5603/ARM.2019.0062 DISCLOSURES: No relevant relationships by Sean Davidson No relevant relationships by Eric Flenaugh No relevant relationships by Marilyn Foreman No relevant relationships by KOMAL KAUR No relevant relationships by Gabriela Oprea-Ilies

COVID-19 PNEUMONIA: A CAUSE OF CAVITARY LUNG LESIONS

SESSION TITLE: Pathologies of the Post-COVID-19 World SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Cavitary lung lesions are a relatively common finding on imaging with a vast differential diagnosis. CASE PRESENTATION: A 36-year-old female with history of gestational diabetes, preeclampsia and obesity presented to an outside hospital for evaluation of acute onset shortness of breath and chest pain. The patient tested positive for COVID19 two weeks prior. She initially developed some mild symptoms including headache, nasal congestion, generalized body aches, cough with mild sputum production and shortness of breath. Despite improvement in these symptoms, she suddenly developed chest discomfort that radiated to the back and was worse with inspiration and cough. The patient had been fully vaccinated against COVID-19 and had received a booster dose as well. Upon evaluation in the emergency room, she was afebrile, respiratory rate was 20, blood pressure was 144/90 mmHg, heart rate was 88 and her oxygen saturation was 99% on room air. Her physical exam was unremarkable. Basic laboratory work up including CBC and chemistry was normal. EKG and troponins were normal as well. A chest x-ay was performed which showed bilateral nodular densities with possible cavitation. A CT chest was later performed and showed multiple bilateral subsolid pulmonary nodules, some with apparent central cavitation. The patient was admitted for further work up at our institution. The patient's subsequent evaluation was largely unrevealing. An autoimmune panel testing for SLE, rheumatoid arthritis, ANCA vasculitis, and Goodpasture's syndrome revealed only a weakly positive ANA with negative anti-DNA and anti-Smith antibody. HIV and QuantiFERON tests were negative. Blood cultures were negative as well. Unfortunately, the patient was not able to expectorate and therefore no sputum cultures were obtained. Due to the patient's clinical improvement and absence of hypoxemia, diagnostic bronchoscopy was deferred, and the patient was subsequently recommended to undergo short interval chest imaging. Repeat chest computed tomography scan one month later showed complete resolution of the previously seen cavitary pulmonary nodules. At the time of outpatient clinical follow up, the patient remained asymptomatic from a respiratory perspective. DISCUSSION: Cavitary pulmonary nodules on chest imaging is an atypical presentation of COVID19 pneumonia that has been rarely described in the literature. In our patient, the temporal correlation between her pulmonary nodules and COVID19 infection as well as her negative work up for other common infectious and inflammatory causes known to cause cavitary lung lesions, makes COVID19 the most plausible cause for her findings. The pathophysiology of these findings remains unclear but may be explained by endothelialitis and small vessel vasculitis may be implicated in the formation of these lesions. [1] CONCLUSIONS: We presented a rare case of cavitary pulmonary nodules due to COVID19 pneumonia. Reference #1: Ackermann M, Verleden SE, Kuehnel M, Haverich A, Welte T, Laenger F, Vanstapel A, Werlein C, Stark H, Tzankov A, Li WW, Li VW, Mentzer SJ, Jonigk D. Pulmonary Vascular Endothelialitis, Thrombosis, and Angiogenesis in Covid-19. N Engl J Med. 2020 Jul 9;383(2):120-128. DISCLOSURES: No relevant relationships by Karim Anis No relevant relationships by Carolyn Garcia

COVID-19 INFECTION MAY BE TRIGGER for DEVELOPMENT of IMMUNE-MEDIATED DISEASES (IMDS)

Background: As considerable evidence indicates viruses play an important role in the pathogenesis of infammatory rheumatic diseases as environmental factors. The most prominent pathogenic viruses which have been proposed in the triggering and initiation of autoimmune diseases include Parvovirus B19, Epstein-Barr-virus (EBV), Cytomegalovirus (CMV), Herpes virus-6, HTLV-1, Hepatitis A and C virus, and Rubella virus1. It is possible that COVID-19 infection is also a trigger. Because SARS-CoV-2 infection can break immune tolerance and trigger autoimmune responses, it is also likely to induce clinical autoimmunity2. Objectives: Find out a possible association between Covid-19 infection and development of IMDs. Methods: We analyzed data of 21 patients (Male 4/19 %/, female 17/81%/, mean age 45.5 ± 13,9 years), who were admitted to Rheumatology department of 'Mikayelyan' University Hospital after Covid-19 infection with newly diagnosed IMDs from June till December 2021. All of included had never had such kind of disorder before. EULAR/ACR criteria were used for diagnosis and assessment of disease activity. Results: After SARS-CoV-2 infection some patients presented with preserved fever, high levels of CRP and ESR, had rash and arthritis. Particularly, 3 (14.3%) developed systemic lupus erythematosus, 3(14.3%)-antiphospholipid syndrome, 4 (19%)-rheumatoid arthritis, 2(9.5%)-spondyloarthritis, 3 (14.3%)-sarcoidosis, 4 (19%)-erythema nodosum, 1 (4.8%)-small-vessel vasculitis, 1 (4.8%)-undifferentiated arthritis, and 1(4.8%)-Tietze syndrome. 11 (52.4%) experienced severe course of Covid-19 with pneumonia and respiratory failure, in 10 (47.6%) patients the course of disease was mild. We've found a signifcant association between severe course of Covid-19 and development of erythema nodosum. (p< 0.05). Also an association between female gender and severe course of Covid-19 was determined (p<0,05). Conclusion: In acute progression of the COVID-19 along with development of antiviral immunity, a dysregulated response of immune system may occur, represented by the marked cytokine release syndrome, macrophage activation, and systemic hyperinfammation.3 We analyzed the data of patients who didn't have any typical symptom of rheumatic diseases before coronavirus infection, therefor, on our opinion, virus played an important role to induce clinical autoimmun-ity and autoinfammation and subsequently-IMDs. Possibly, Covid-19 infection may be included in the group of trigger viruses for.

INCIDENCE and RISK FACTORS of COVID-19 in PATIENTS with VASCULITIS: A DANISH NATIONWIDE COHORT STUDY

Background: Patients with small vessel vasculitis (SVV) and large vessel vas-culitis (LVV, including giant cell arteritis (GCA)) are considered at higher risk of infections compared to the general population, owing to their underlying condition and the use of immunosuppressive drugs. Thus, the risk of COVID-19 infection and related outcomes during the global pandemic is of immediate concern to rheumatologists worldwide. Objectives: To estimate the incidence of COVID-19 hospitalisation in patients with vasculitis, and to evaluate the impact of glucocorticoid treatment on the outcome between March 2020 and February 2021. Methods: With use of the Danish nationwide registers, a cohort of patients with LVV and SVV, respectively, and general population controls (GPCs) matched on age and gender was established. Hazard ratios (HR) for COVID-19 hospitalisation was estimated. National COVID-19 surveillance data was used to calculate the odds ratio (OR) of having had a positive SARS-CoV2 PCR test. Lastly, a nested case-control design and conditional logistic regression was used to estimate the impact of glucocorticoids on the risk of hospitalisation. Results: Patients with SVV (n=1090) had an increased incidence of COVID-19 hospitalisation compared with GPCs (comorbidity-adjusted HR 2·73;95% CI 1·64-4·55), whereas no increased risk was seen in patients with LVV. Patients with vasculitis had similar likelihoods of having had a positive PCR test as GPCs. Glucocorticoids did not increase the HR of hospitalisation among patients with LVV or SVV. Conclusion: Patients with SVV were more likely to be admitted with COVID-19 than the GPCs. The impact of glucocorticoid treatment on the risk of hospitalisation needs further investigation.

HEALTH-RELATED QUALITY OF LIFE AMONG PATIENTS WITH ANCA VASCULITIS

BACKGROUND AND AIMS: Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a debilitating disease that can have a significant impact on a patient's quality of life. The aim of this study was to assess the longitudinal quality of life amongst those diagnosed with AAV using the EQ-5D instrument, which allows for calculation of quality-adjusted life years (QALYs.) METHOD: A total of 343 patients with AAV participated in this study, of which 191 (55.7%) were male, resulting in 2746 episodes. The EQ-5D-5L standardised instrument was used to evaluate health-related quality of life in the domains of mobility, self-care, usual activities, pain/discomfort, anxiety/depression and to generate a summary index score. Overall health was also rated using a visual analogue scale (0-100). EQ-5D questionnaires were completed during routine nephrology clinic attendances and through a vasculitis patient support smartphone app. We used a random effects model to control for multiple entries relating to individual patients. RESULTS: A lower quality of life was seen amongst those with AAV (median index value 0.80, overall population average 0.856). The mean visual analogue scale score was 75.6 ± 17.3 (overall population average 82.8, Fig. 1). Patients' pain and discomfort level (mean 1.95) was most affected while self-care (mean 1.33) was least affected (Fig. 1). An increase in BVAS tightly correlated with a reduction in quality of life. Using the random effects model, the index score was seen to decrease with increasing age with a 2.7% reduction in index score per decade. A 7% reduction in index score was seen during periods of disease activity compared with periods of remission. Patients with end-stage kidney disease requiring dialysis had an 8% reduction in index score. A reduced quality of life was seen following COVID-19 lockdown with a 5% reduction in index score seen. Using a median survival rate of 6.16 years for patients with small vessel vasculitis, we calculated the QALYs for this population as 4.9 years. CONCLUSION: We have defined for the first time the EQ-5D index value over the full disease course in patients with AAV. Notably, we have identified a reduction in quality of life during periods of disease activity. Other studies have demonstrated a reduction in quality of life during active disease using the AAV-PRO questionnaire and the Medical Outcomes Study Short Form-36. A decrease in work productivity has also been noted. Previously reported mean index values of 0.72 and 0.76 were lower than our observed values, although both are significantly reduced compared with population norms. In conclusion, this research highlights the negative impact of AAV on patients' lives.

Isolated pauci-immune pulmonary capillaritis associated with an extensive bilateral hemothorax and pleuro-pericarditis

Introduction: Isolated pauci-immune pulmonary capillaritis (IPIPC) is a rare disorder characterized by small vessel vasculitis limited to alveolar capillaries in the absence of systemic manifestations. There are very few case reports of this disorder in the medical literature. Case Report: A 37-yo male with no known history of autoimmune pathology who was admitted to the hospital for evaluation and treatment of dyspnea and thoracalgia. Peripheral blood cultures, serum studies to detect Legionella and Pneumococcus antigens, and a nasopharyngeal swab test for covid-19 were all negative. Chest imaging revealed bilateral pleural effusions from the base to the apices with concomitant atelectasis of the adjacent lung parenchyma. Although the results of an 18F-PET-CT scan revealed no pathological uptake, video-assisted thoracoscopy revealed diffusely edematous pleura with crater-like patches with new onset of venous vessel varicosities, intra-alveolar hemorrhages associated with disordered vascularization, suggesting small vessel vasculitis. Histologic findings included widespread intra-alveolar hemorrhage with organizing injury, hemosiderin-laden macrophages, scattered intra-arterial thrombi, and diffuse perivascular neutrophilic infiltrates consistent with a diagnosis of capillaritis. Conclusions: Given the negative immune studies (save for a weakly-positive lupus anticoagulant and no evidence for extra-pulmonary vasculitis, the diagnosis was Isolated pauci-immune pulmonary capillaritis. The patient recovered in response to immunosuppressive/anti-inflammatory therapy.

A late diagnosis of eosinophilic granulomatosis with polyangiitis

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a medium and small vessel vasculitis. Discussion: A 58-years man was admitted to the Emergency Department in January 2022 for myalgia and weakness of lower limbs in recent COVID-19 infection. He had a clinical history of allergic asthma and eosinophilic pneumonia (ANCA negative) diagnosed as secondary to sensitization work-related in 2001. Blood test showed a severe hypereosinophilia (absolute eosinophil count: 9875/microL) and elevated creatine kinase (CK: 7555 U/L). He was hospitalized in HUB COVID. During hospitalization reported paraesthesia of upper and lower limbs and fever;blood test showed elevation of inflammation markers. Autoimmune screening showed a antineutrophil cytoplasmic antibodies positivity (ANCA anti-MPO 178UI/mL). A sinus CT showed nasal polyposis. A neurological evaluation and electromyography were performed with the evidence of polyneuropathy. Muscle biopsy showed eosinophil-associated vascular occlusion and eosinophilassociated tissue damage. The investigation excluded renal, cardiac, pulmonary and gastro-intestinal involvement. A steroid therapy (Prednisone 1 mg/kg/die) was started with clinical improvement. Conclusions: EGPA is a multisystemic disorder, typically suspected based on a combination of clinical findings, such as asthma, nasal and sinus symptoms, peripheral neuropathy, and eosinophilia ≥1500/microL. ANCA antibodies are positive in around 40% of patients and diagnosis can often be challenging and delayed.

A case report of granulomatosis with polyangiitis in a middle aged women

Background: Wegener's granulomatosis (Granulomatosis with polyangiitis) is a rare systemic autoimmune disease of unknown etiology characterised by triad of necrotising granulomatous inflammation of upper and lower respiratory tract, glomerulonephritis, and disseminated vasculitis. Case Study: A 50 year old female presented with complaints of running nose since last 8 months. Patient also had low grade fever, generalised body pain, productive cough, shortness of breath on exertion, pedal oedema for last 2 months. She had an 2-3 episodes of blood in sputum 15 days back. On examination saddle nose found. HRCT chest shows multiple cavitating nodules with consolidation. Lab findings shows raised ESR, CRP, COVID RT PCR negative and positive C-ANCA (Anti neutrophilic cytoplasmic antibody), red blood cell casts, and albumin present in urine. Sputum for AFB negative. Patient discharged on corticosteroids and cyclophosphamide and advised for regular follow up. Discussion: Wegener's granulomatosis is an antineutrophilic cytoplasmic antibody associated small vessel vasculitis. Prevalence of this disease varies from 3/1,00,000 to 16/1,00,000. Typically this involve the lungs and kidneys. It can be of generalised severe form or localised limited form. Our patient clinical course, CT findings and his strongly positive C-ANCA were considered diagnostic of wegener's granulomatosis. Treatment include corticosteroids and immunosuppressants. Conclusion: The early diagnosis and prompt treatment of multisystem disorder is necessary to prevent complication such as diffuse alveolar haemorrhage CT is the imaging modality of choice for diagnosis, surveillance and follow up in patients with wegener's granulomatosis.

Leukocytoclastic vasculitis (cutaneous small-vessel vasculitis) after COVID-19 vaccination.

Vaccinations may induce cutaneous adverse events, due to nonspecific inflammation or immuno-mediated reactions. Several types of vasculitis have been observed. We report on a 71-year-old woman who developed cutaneous small-vessel vasculitis after the second dose of Vaxzevria COVID-19 vaccination, showing leukocytoclastic vasculitis on histopathological examination of a skin biopsy. Cutaneous small-vessel vasculitis is a rare condition which can be idiopathic or secondary to underlying infections, connective tissue disorders, malignancy, and medications. The pathogenesis involves immune complex deposition in small blood vessels, leading to activation of the complement system and recruitment of leukocytes. Exacerbation of small-vessel vasculitis has been reported following the administration of various vaccines, particularly influenza vaccine. It is expected that SARS-CoV-2 vaccine results in the activation of B- and T-cells and antibody formation. We hypothesize that leukocytoclastic vasculitis caused by immune complex deposition within cutaneous small vessels could be a rare side effect of Vaxzevria COVID-19 vaccination.

Coronavirus disease 2019-associated immunoglobulin A vasculitis/Henoch-Schönlein purpura: A case report and review.

Immunoglobulin A (IgA) vasculitis or Henoch-Schönlein purpura is a predominantly pediatric disease occurring after a triggering viral or bacterial infection. Conversely, drug exposure is the most common inciting event in adult cases of IgA vasculitis. Recently, data has suggested a temporal association between coronavirus disease 2019 (COVID-19) and the development of IgA vasculitis in children and adults. Here, we describe a case of IgA vasculitis with nephritis in a 70-year-old man with COVID-19 and perform a comprehensive review of eight reported cases of suspected COVID-19-associated IgA vasculitis. When compared to classical IgA vasculitis, COVID-19-associated IgA vasculitis exclusively affects males (p < 0.00002) and is more common in adults (p < 0.005). Among cases of COVID-19-associated IgA vasculitis, adult cases were associated with significantly more arthralgia than pediatric cases (p = 0.04). In cases where skin biopsy was obtained, direct immunofluorescence (DIF) was negative for IgA in 50% of cases; thereafter, kidney biopsy DIF was positive for IgA in all cases. With this study, we provide support for an association between IgA vasculitis and severe acute respiratory syndrome coronavirus 2 infection and provide clinical information differentiating its manifestations from classical IgA vasculitis.

Colonic Gangrene: A Sequela of Coronavirus Disease 2019.

Initially considered to be a respiratory disease, coronavirus disease 2019 (COVID-19) is now recognized as a multisystem disease known to affect all the major organs, including the gastrointestinal system. Based on recent studies, severe acute respiratory syndrome coronavirus 2 causes dysregulation of multiple biological pathways, triggers an exaggerated immune response, and affects multiple organs. The gastrointestinal symptoms in COVID-19 are common but often overlooked. We report the case of a 50-year-old female with a recent history of COVID-19 presenting with complaints of abdominal pain and constipation. Initially, the patient was treated for respiratory symptoms and discharged home. Subsequently, she was re-admitted and diagnosed with colonic obstruction on radiology. Laparotomy revealed descending and sigmoid colonic gangrene requiring left colectomy. This case highlights the uncommon but severe gastrointestinal manifestations of COVID-19.

Exuberant bullous vasculitis associated with SARS-CoV-2 infection.

We described a case of exuberant cutaneous small-vessel vasculitis in a 27-year-old male with mild CoVID-19 in Brazil. The patient presented painful purpuric papules and vesicobullous lesions with hemorrhagic content located in the larger amount in the lower limbs and, to a lesser extent in the region of the back and upper limbs, saving palms and soles of the feet. Influenza-like syndrome with anosmia and ageusia was reported seven days before the skin lesions. A real-time reverse transcription polymerase chain reaction was positive on a nasopharyngeal swab for SARS-CoV-2. Histopathological study showed leukocytoclastic cutaneous vasculitis affecting small vessels and microthrombi occluding some vessels. The patient presented an improvement in skin lesions by the fifth day of prednisone therapy. This case highlights the importance of the SARS-CoV-2 test in investigating the etiology of cutaneous vasculitis during this pandemic.