Vasculitis Syndrome Research Articles

From membranoproliferative glomerulonephritis to a final diagnosis: Hypocomplementemic urticarial vasculitis syndrome

Membranoproliferative glomerulonephritis describes a glomerular-injury pattern common to a heterogeneous group of diseases. Evaluation based on clinical and laboratory presentation and immunofluorescence staining on kidney biopsy allows identification of underlying pathophysiological processes and may facilitate proper diagnosis and treatment. Hypocomplementemic urticarial vasculitis syndrome is a rare autoimmune disease of multi-organ involvement. The diagnosis is based on well-defined clinical and laboratory criteria. The pathophysiology is not completely understood but the presence of anti-C1q antibody seems to be involved. Renal involvement occurs in up to 50% of cases. It can be heterogeneous and can be indistinguishable from lupus nephritis. Serological findings and skin involvement distinguish these two entities. We report the case of a 40-year-old female who presented with urticarial skin lesions, hypocomplementemia and nephrotic syndrome. Kidney biopsy showed membranoproliferative glomerulonephritis with full house immune complex deposits. The diagnosis of hypocomplementemic urticarial vasculitis syndrome was made and the patient was successfully treated with prednisolone and mycophenolate mofetil.

Comprehensive overview of IRVAN syndrome: a structured review of Case Reports and Case Series.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare spectrum of retinal vasculitis, aneurysms, and neuroretinitis affecting young individuals in their third decade. Most of our current knowledge is based on case reports, case series, and a handful of collaborative studies. There is much diversity in treatment approaches and outcomes in the reported literature. We have aggregated published case reports and case series into quantitative and narrative synthesis to draw evidence-based conclusions toward clinical features, atypical and rare findings, systemic associations, disease course, and treatment outcomes. The analysis suggested the disease mostly affects young individuals with a female predilection. Anterior chamber and vitreous inflammation are common than previously believed. The most prevalent pattern of retinal vasculitis in IRVAN eyes is mixed vasculitis, followed by arteritis and phlebitis. Most eyes at the time of presentation have capillary nonperfusion and require treatment. Most eyes retain good visual acuity; however, treatment is required to maintain visual function. Intravitreal antivascular endothelial growth factors administered as an adjunct to retinal laser photocoagulation are more likely to improve visual outcomes. Besides, we have discussed the different hypotheses on the etiopathogenesis of the disease and stronger evidence suggests an inflammatory origin of the disease.

Emergence of “urban scrub typhus” during Monsoon season in an urban pocket and biodiversity hotspot of New Delhi, India

Introduction: Scrub typhus is an endemic disease transmitted within the “epidemiological tetrad” of rains, rodents, chigger mites, and scrub vegetation prevalent in “Tsutsugamushi Asia-Pacific triangle,” predisposing one billion population at risk and annual incidence of one million. Scrub typhus is difficult to differentiate clinically from coendemic vector-borne acute undifferentiated febrile illness (AUFI). Untreated scrub typhus may cause disseminated vasculitis, serositis, and hemophagocytic syndrome. Outbreaks of scrub typhus have been reported from Thailand, China, Korea, and rural India. Transmission of scrub typhus in urban areas is relatively rare. Three consequent outbreaks in a urban pocket in New Delhi, India, highlight emergence of urban scrub typhus. Materials and Methods: A cross-sectional, clinicoepidemiological, ambispective outcome surveillance study was conducted among all clinicodemographically homogenous patients presenting with AUFI during 2016–2018. Clinically suspected or intuitively investigated scrub typhus was screened by IgM/IgG immunochromatography and confirmed through IgM enzyme-linked immunosorbent assay and real-time polymerase chain reaction. Spatial, temporal, and vector surveillance through epidemiological mapping, line listing, and mite surveillance was done. Results: Scrub typhus outbreaks affected 161 patients during 2016–2018. Mean age was 31.77 ± 17 years. Most common clinical presentation was fever with headache. Eschar was present in 46.88% patients commonly on abdomen, chest, perineum, and extremities. Coinfections and comorbidities were seen in 3.75% and 14.37% patients, respectively. Seventy percent and 14.8% patients were managed in acute care and intensive care. Mean hospital stay was 8.96 ± 3.86 days. Doxycycline and doxycycline-azithromycin combination were given in 92.5% and 7.5% patients, respectively. All-cause mortality was 6.25%. 126/160 (78.75%) patients were geospatially distributed from urban landscape depicted in epidemiological maps. Temporal-seasonal distribution revealed bell-shaped curve from May to November. Mite carriage was seen in 16% rodents. Conclusion: Urban scrub typhus is emerging in microhabitats fulfilling the epidemiological tetrad and chigger mites undergoing transovarian transmission. Geospatial and temporal mapping are required in urban neighborhoods for risk stratification, outbreak management, vector control, and community education. A high index of suspicion in AUFI and early initiation of doxycycline therapy are required.

Central and Peripheral Nervous System Complications of Vasculitis Syndromes From Pathology to Bedside: Part 1-Central Nervous System.

Purpose of ReviewThe aim of this review is to provide a comprehensive update on the clinical assessment, diagnosis, complications, and treatment of primary central nervous system vasculitis (PCNSV).Recent FindingsThe developments in neuroimaging, molecular testing, and cerebral biopsy have enhanced clinical assessment and decision making, providing novel insights to prevent misdiagnosis increasing diagnostic certainty. Advances in imaging techniques visualizing the wall of intracranial vessels have improved the possibility to distinguish inflammatory from non-inflammatory vascular lesions. Large recent studies have revealed a more varied histopathological pictures and disclosed an association with amyloid angiopathy. Unfortunately, therapy remains largely empiric.SummaryPCNSV is a heterogeneous group of disorders encompassing different clinical subsets that may differ in terms of prognosis and therapy. Recent evidence has described a more benign course, with good response to therapy. New diagnostic techniques will play soon a pivotal role in the appropriate diagnosis and prompt management of PCNSV.

Evaluation of Oxidative Stress Biomarkersin Patients with Henoch-Schönlein Purpura.

Henoch-Schönlein Purpura (HSP) is a systemic vasculitic syndrome characterized by non-thrombocytopenic purpura, arthritis/arthralgia, abdominal pain, and glomerulonephritis. The pathogenesis of HSP has not been clearly identified. Oxidative damage has a role in the pathogenesis of most cases. This study aimed to evaluate changes of oxidative stress by studying parameters like superoxide dismutase (SOD), catalase (CAT), and malondialdehyde (MDA) in an attempt to identify the role of oxidative stress in HSP from another perspective. This study enrolled 23 pediatric patients (ten girls and thirteen boys) diagnosed with HSP who were under follow-up at Sutcu Imam University School of Medicine Department of Pediatrics between 2014 and 2016 and twenty healthy children as the control group. The parents of all subjects gave informed consent to participate in the study. In the HSP group, the beginning season of the illness and the systemic involvement during follow-up were determined. Blood specimens were obtained at presentation before any treatment was started. SOD, CAT activities, and MDA values in erythrocyte and plasma samples were compared between the patient group and the healthy children. Twenty-three patients with HSP (13 males, 10 females) and 20 healthy children participated in this study. The mean age of the HSP cases was 8.21±3.78 years (range 2-16 years) and of the controls was 8.6±4.2 (range 3-14 years). The mean MDA value was 2.95±0.71 nmol/ml in the patient group and 2.67±0.66 nmol/ml in the control group (p=0.787). The mean level of the CAT enzyme was 1.32±0.35 U/g Hb in the patient group and 7.8±1.74 U/g Hb in the control group (p=0.001). The mean levels of the SOD enzyme were 3.06±0.85 U/g Hb in the patient group and 0.97±0.36 U/g Hb in the control group (p=0.001). Although high MDA levels support the role of lipid peroxidation in the pathogenesis of HSP, statistical significance was not reached owing to a limited number of our patients. The reduced CAT enzyme activity is consistent with the findings of previous reports. This finding supports the notion that oxidative stress can play a role in the pathogenesis of HSP. Our findings support the notion that oxidative stress can play a role in the pathogenesis of HSP.

Large artery intracranial stenosis in young adults with ischaemic stroke

Large artery intracranial stenosis (ICS) is a common finding in stroke patients, but is much less prevalent in Western countries than in Asia and in young adults than in the elderly. We investigated the prevalence and causes of ICS among French young adults with ischaemic stroke. Clinical and radiological data of patients aged 18–54 years treated consecutively for acute ischaemic stroke in the anterior circulation at a tertiary stroke centre were analysed retrospectively. Patients with>50% ICS were identified. ICS was evaluated using TOF-MRA, vessel wall-MRI, digital subtraction angiography and CT-angiography. A total of 316 patients were included. ICS was diagnosed in 29 patients, resulting in a prevalence of 9.2% (95% CI, 6.2 to 13.3). The leading cause of ICS was atherosclerosis (n=13), ahead of moyamoya disease (n=4), dissection (n=2), vasculitis (n=2), and reversible cerebral vasoconstriction syndrome (n=1). The cause of ICAS could not be determined in 7 patients. ICS was found in nearly one in 10 ischaemic strokes among French young adults. Atherosclerosis was the leading cause of ICS. The cause of ICS could not be determined in almost a quarter of the patients.

Vascular abnormalities visualized by multislice computed tomography of the abdomen: accidental findings or immediate causes of pain syndrome? (topic review)

Abnormalities of the abdominal aorta branches may cause chronic mesenteric ischemia, progressive pathological changes of the internal organs in this setting, and chronic pain syndrome. The causes of chronic mesenteric ischemia may be divided into atherosclerotic and non-atherosclerotic. Atherosclerosis of the unpaired branches of the abdominal aorta involves stenosis or occlusion. Other causes include fibromuscular dysplasia, vasculitis (Takayasu, segmental mediolytic arteriopathy), and median arcuate ligament syndrome. These syndromes, the pathogenesis of some of which remains controversial, lead to nonspecific complaints such as abdominal pain, weight loss, and others. Digital subtraction angiography or duplex ultrasound may provide hemodynamic information in cases of vascular disease in this area. However, multislice spiral computed tomography is in many cases the first choice because it allows for a comprehensive assessment of the state of blood vessels and associated morphological changes of internal organs. Structural changes accompanying these syndromes can also occur in patients who are undergoing a medical examination for other reasons. However, these syndromes should not be diagnosed solely on the basis of imaging; instead, the findings should be compared with the clinical presentation, which implies collaboration of radiologists and clinicians.

353 SARS-CoV-2 pulmonary vasculitis and Takotsubo myocarditis syndrome. Hypoxia, endothelial dysfunction, and inflammation are the trigger?

353 SARS-CoV-2 pulmonary vasculitis and Takotsubo myocarditis syndrome. Hypoxia, endothelial dysfunction, and inflammation are the trigger?

Novel pediatric granulomatosis with polyangiitis with a marked bloody pericardial effusion and bloody stool: a case report

BackgroundGranulomatosis with polyangiitis (GPA) is a syndrome of refractory vasculitis involving the upper respiratory tract, lungs, kidneys, and systemic small and medium-sized arteries that affects all age groups. No pediatric case with a bloody pericardial effusion resulting in cardiac tamponade and co-existing hematochezia has been reported.Case presentationA 14-year-old boy was referred for evaluation of prolonged fever, chest pain, and intermittent hematochezia. Diagnostic imaging showed a prominent pericardial effusion. Immediately after admission, his systolic blood pressure decreased. Emergent pericardiocentesis resulted in aspiration of a massive amount of bloody pericardial fluid. This was diagnosed as cardiac tamponade because his blood pressure recovered immediately after the drainage. The patient had an elevated serine proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) level on serological examination. Head MRI showed thickening of nasal and sinusoidal mucosa and a cystic mass in the left sphenoid sinus. After ruling out malignancy based on the cytology of the effusion, chest MRI, and gallium scintigraphy, total colonoscopy showed multiple irregular-shaped aphthae from the right transverse colon to the cecum on the contralateral side of the mesenteric attachments. Biopsy specimens of aphthous lesions confirmed necrotizing granulomatous inflammation. A diagnosis of GPA was made based on these findings, and oral prednisolone (PSL) and azathioprine were started. The hematochezia disappeared rapidly, and no recurrence of pericardial effusion was seen after PSL tapering was completed. The PR3-ANCA level decreased into the normal range immediately after the initial therapy.ConclusionsPericarditis is a common cardiac complication of GPA, but there have been no reports of resultant cardiac tamponade. This is the first case of pediatric GPA with cardiac and gastrointestinal complications preceding the common symptoms such as respiratory or renal symptoms. A case of pediatric GPA with hematochezia is also extremely rare. In conclusion, serial measurement of ANCA levels is important in patients with persistent fever and bloody stool, such as in inflammatory bowel disease, to make the diagnosis of a vasculitic syndrome.

Co-culture of peripheral blood mononuclear cell (PBMC) and human coronary artery endothelial cell (HCAEC) reveals the important role of autophagy implicated in Kawasaki disease.

BackgroundKawasaki disease (KD) is a systemic vasculitis syndrome that commonly occurs in children. Autophagy has been increasingly shown to be involved in various cardiovascular diseases, including endothelial dysfunction and vascular endothelial injury. However, whether autophagy is implicated in the pathogenesis of KD remains poorly understood, and particularly, how the dysfunction of human coronary artery endothelial cells (HCAECs) is associated with autophagy in peripheral blood mononuclear cells (PBMCs) from KD patients awaits further investigation.MethodsPeripheral blood samples were collected from KD patients, common fever patients, and healthy controls. The PBMC samples were isolated from KD blood samples collected at three different phases: the acute phase before therapy (acute-KD), 1 week (subacute-KD), and 4 weeks (convalescent-KD) after drug administration.ResultsThe autophagy flux was significantly increased in the PBMCs of KD patients at acute phase. The PBMCs of acute KD patients could induce autophagy in HCAECs and promote the secretion of chemokines and pro-inflammatory factors after cocultured with HCAECs whereas 3-methyladenine (3-MA) drug could partly reverse this process.ConclusionsAutophagy is involved in the inflammatory injury of vascular endothelial cells associated with PBMCs in KD patients, and may play a crucial role in regulating inflammation. Hence, we identify a novel regulatory mechanism of vascular injury in this disease.

Multisystem Imaging Manifestations of Kawasaki Disease.

Kawasaki disease (KD) is a common pediatric vasculitis syndrome involving medium- and small-sized arteries that is especially prevalent in early childhood (ie, age 6 months to 5 years). The diagnosis of KD is made on the basis of clinical features, such as fever, characteristic mucocutaneous changes, and nonsuppurative cervical lymphadenopathy. However, early diagnosis is often challenging because many children with KD present with atypical symptoms. The most serious complication of KD is coronary artery aneurysm caused by coronary arteritis. Prompt intravenous immunoglobulin therapy reduces the risk of cardiac morbidity. In addition, the systemic extension of KD-related vasculitis during the acute phase causes a variety of multisystem manifestations, including encephalopathy, stroke, retropharyngeal edema, pericarditis, myocarditis, KD shock syndrome, pulmonary lesions, intestinal pseudo-obstruction, gallbladder hydrops, arthritis, and myositis. These complications tend to be more common in affected children with atypical presentation. Radiologists can play an important role in the timely identification of diverse KD-associated morbidities and thus may contribute to the early diagnosis of atypical KD. Online supplemental material is available for this article. ©RSNA, 2021.

From Churg-Strauss Syndrome to Eosinophilic Granulomatosis With Polyangiitis: A Historical Review of Nomenclature and Diagnostic Criteria.

Eosinophilic granulomatosis with polyangiitis (EGPA) is rare vasculitis syndrome that involves the skin and other organ systems manifesting as asthma, eosinophilia, and pulmonary infiltrates. The understanding of EGPA, previously known as Churg-Strauss Syndrome, has continued to evolve from its earliest documentation in the literature in 1951. Herein, we review key historical advances in the diagnosis, classification, and nomenclature of EGPA that have shaped our understanding of this protean disorder over time.

Rheumatic Manifestations and Diseases From Immune Checkpoint Inhibitors in Cancer Immunotherapy.

Immune checkpoint inhibitors (ICIs), which can enhance antitumor immunity and inhibit cancer growth, have revolutionized the treatment of multiple cancers and dramatically decreased mortality. However, treatment with ICIs is directly associated with immune-related adverse events (irAEs) because of inflammation in off-target organs and autoimmunity resulting from non-specific immune activation. These irAEs can cause rheumatic diseases and manifestations such as inflammatory arthritis, polymyalgia rheumatica, myositis, vasculitis, Sicca and Sjogen's syndrome, and systemic lupus erythematosus. Early diagnosis and treatment of these adverse events will improve outcomes and quality of life for cancer patients. The treatment of rheumatic diseases induced by ICIs requires multidisciplinary cooperation among physicians. Furthermore, the underlying mechanisms are not fully understood and it is difficult to predict and evaluate these side effects precisely. In this review, we summarize available studies and findings about rheumatic irAEs, focusing mainly on the clinical manifestations, epidemiology, possible mechanisms, and guiding principles for treating these irAEs.

Single-cell profiling of T lymphocytes in deficiency of adenosine deaminase 2.

Deficiency of adenosine deaminase 2 (DADA2) is a monogenic vasculitis syndrome caused by autosomal-recessive loss-of-function mutations in the ADA2 gene (previously known as CECR1). Vasculitis, vasculopathy, and inflammation are dominant clinical features of this disease; the spectrum of manifestations includes immunodeficiency and lymphoproliferation as well as hematologic manifestations. ADA2 is primarily secreted by stimulated monocytes and macrophages. Aberrant monocyte differentiation to macrophages and neutrophils are important in the pathogenesis of DADA2, but little is known about T lymphocytes in this disease. We performed combined single-cell RNA sequencing and single-cell TCR sequencing in order to profile T cell repertoires in 10 patients with DADA2. Although there were no significant alterations of T cell subsets, we observed activation of both CD8+ and CD4+ T cells. There was no clonal expansion of T cells: most TCRs were expressed at basal levels in patients and healthy donors. TCR usage was private to individual patients and not disease specific, indicating as unlikely a common pathogenic background or predisposition to a common pathogen. We recognized activation of IFN pathways as a signature of T cells and STAT1 as a hub gene in the gene network of T cell activation and cytotoxicity. Overall, T cells in DADA2 patients showed distinct cell-cell interactions with monocytes, as compared with healthy donors, and many of these ligand-receptor interactions likely drove up-regulation of STAT1 in both T cells and other immune cells in patients. Our analysis reveals previously undercharacterized cell characteristics in DADA2.

A case report of a severe neonatal systemic vasculitis on the first day of life

BackgroundNeonatal systemic vasculitis syndromes have been reported in infants born to mothers with systemic lupus erythematosus, Sjögren’s syndrome, Behҫet’s disease, cutaneous polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitides. Here we report a novel association of a case of new-onset maternal seronegative inflammatory arthritis associated with a transient systemic vasculitis in a neonate.Case presentationIn the first 24 h of life, a preterm Caucasian baby boy was noted to have blue discoloration to all four extremities. Despite antibiotics, fresh frozen plasma and anticoagulation, the discoloration remained, particularly in the left index finger. This was associated with fever and a maximum C-reactive protein (CRP) of 148 mg/L. Intravenous immunoglobulin (IVIG) was given with short-term improvement. Initial echocardiogram showed enlarged coronary arteries with normalization on repeat 1 week later. Clinical signs and symptoms responded to high dose oral steroid administration. MRI angiography (MRA) of the body and heart showed tortuosity of arteries in the upper and lower extremities with gadolinium uptake, suggestive of vasculitis. Autoantibody profile negative. Genetic panel for hereditary autoinflammatory diseases was negative as was whole exome sequencing performed on the trio. The baby was weaned off steroids by 5 months of age. A small distal autoamputation of the left index finger occurred.He was born to a 28-year-old woman who developed new onset severe symmetrical polyarthritis at 8 weeks gestation. This was presumed a reactive arthritis secondary to a dental infection. Infectious work up and autoantibodies were negative. She was treated with high dose prednisone for the remainder of her pregnancy.The mother was weaned off prednisone, treated with hydroxychloroquine for 8 months post-partum and remains in remission. A repeat MRA done at 1 year old showed mild residual tortuosities of the arteries in the forearms. The remainder of the medium and large vessels were within normal limits with no gadolinium enhancement to suggest active disease. The child is now 4 years old with normal growth and development.ConclusionThis is a unique case of new-onset seronegative presumed reactive arthritis in a mother with the rare development of a successfully treated medium vessel vasculitis in an infant.

Direct-Acting Antiviral Therapy Effect on Extrahepatic Rheumatic Manifestations in Egyptian Chronic Hepatitis C (HCV) Patients

Background: Hepatitis C virus infection is an important cause of many rheumatic diseases, especially cryoglobulinemic vasculitis, fibromyalgia, arthralgia and Sicca Syndrome. Direct acting antivirals are found to be highly effective for the treatment of chronic Hepatitis C virus infection. Objective: The aim of this study was to evaluate the clinical and serological course of extrahepatic rheumatic manifestations in chronic hepatitis C Egyptian patients after treatment with new direct-acting antivirals. Patients and Methods: This study was carried out on 60 Egyptian patients with chronic hepatitis C with one or more of the HCV- related rheumatic manifestations treated with sofosbuvir (400 mg daily) plus daclatasvir (60 mg daily) as a direct-acting antivirals for 3 months. All patients were subjected to clinical and serological course of extrahepatic rheumatic manifestations evaluation. Results: show that among the 60 patients, 21 patients (35.0%) met the criteria of mixed cryoglobulinemia syndrome (MC), 19 patients (31.7%) were complaining of arthralgia, 17 patients (28.3%) were complaining of fibromyalgia and 3 patients (5.0%) were complaining of Purpura without other criteria of mixed cryoglobulinemia syndrome. Complete clinical response was achieved in a significant number of patients with mixed cryoglobulinemia syndrome (MCS), the results also show significant clinical improvement in patients with fibromyalgia, arthralgia and purpura. Significant reduction but not complete normalization in auto-antibodies at short term follow-up was observed. Conclusion: It could be concluded that sustained virologic response correlates with clinical and immunological improvement in most patients with extrahepatic rheumatic manifestations among the studied group of chronic hepatitis C Egyptian populations.

Ischemic stroke as presentation of DADA2: Case report and literature review

Deficiency of adenosine deaminase type 2 (DADA2) is a rare monogenic vasculitis syndrome caused by mutations in the ADA2 gene that encodes the adenosine deaminase 2 (ADA2) protein. It is an autosomal recessive disease characterised by a large phenotypic variability due to the many organs that can be affected. Ischemic and/or hemorrhagic stroke could be a manifestation of the disease. We described a case of ischemic stroke as presentation of DADA2.

RECURRENT DIFFUSE ALVEOLAR HEMORRHAGE IN SYSTEMIC SCLEROSIS AS COMPLICATION OF COVID-19

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a rare but severe life-threatening condition characterized by widespread bleeding of the pulmonary vasculature into the alveolar space. DAH have a myriad of underlying etiologies. Rheumatic diseases have shown association with DAH. The Coronavirus disease 2019 (COVID-19) pandemic has increased the complexity associated with diagnosing DAH. Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infects the endothelium using the angiotensin-converting enzyme 2 receptor causing a lymphocytic endotheliitis. The increased inflammatory state secondary to COVID-19 has been associated with development of vasculitis syndromes. Although systemic sclerosis (SSc) has been linked to recurrent DAH, there is scarce evidence associating COVID-19 and SSc with DAH. CASE PRESENTATION: A 61-year-old female with a past medical history of SSc, interstitial lung disease (ILD) and DAH presented with worsening shortness of breath of 2 days duration. She was found to have acute hypoxic respiratory failure. Computed tomography (CT) of thorax showed diffuse bilateral ground-glass opacities. SARS-Cov-2 polymerase chain reaction was positive. She was started on broad spectrum antibiotics, dexamethasone and received two units of COVID-19 convalescent plasma. Due to respiratory distress, she was intubated. A decline in her hemoglobin prompted a diagnostic bronchoscopy that showed increasing frank blood in the bronchoalveolar lavage. Antinuclear antibody with centromere pattern and rheumatoid factor were positive with low complement C3 and C4. Steroids were switched to high-dose IV methylprednisolone. Three cycles of plasmapheresis were given with no clinical improvement. DISCUSSION: COVID-19 pandemic has changed the traditional approach to dyspnea. This change in the paradigm, as a result of limited resources and increased occupational risk, may hinder the diagnosis of complex disorders like SSc-ILD and DAH. It's of paramount importance to test rapidly for SARS-CoV-2 infection. ILD is a common complication of SSc. The high-resolution CT similarities between the 2 diseases make it difficult to distinguish COVID-19-ILD superinfection from worsening of SSc-ILD. Bronchoscopy and surgical lung biopsy should always be weighed against their possible complications, occupational exposure and prognosis. CONCLUSIONS: COVID-19 may overlap and complicate the diagnosis of SSc-ILD. COVID-19 associated acute respiratory distress syndrome is attributed to cytokines release (cytokine storm). This likely worsens the underlying inflammatory process in SSc-ILD and could explain the development of DAH. Although there is limited evidence to guide specific treatment, steroid therapy has proven to have mortality benefit in severe COVID-19. The role of steroids, plasmapheresis, immunomodulators and antifibrotic therapy in COVID-19 with SSc-ILD complicated with DAH is unknown. REFERENCE #1: Colby TV, Fukuoka J, Ewaskow SP, Helmers R, Leslie KO. Pathologic approach to pulmonary hemorrhage. Annals of Diagnostic Pathology. 2001;5(5):309-19. REFERENCE #2: Varga Z, Flammer AJ, Steiger P, Haberecker M, Andermatt R, Zinkernagel AS, et al. Endothelial cell infection and endotheliitis in COVID-19. The Lancet. 2020;395(10234):1417-8. REFERENCE #3: Wong AW, Fidler L, Marcoux V, Johannson KA, Assayag D, Fisher JH, et al. Practical Considerations for the Diagnosis and Treatment of Fibrotic Interstitial Lung Disease During the Coronavirus Disease 2019 Pandemic. Chest. 2020;158(3):1069-78. DISCLOSURES: No relevant relationships by Vedesh Babu, source=Web Response No relevant relationships by Alejandra Garcia Fernandez, source=Web Response No relevant relationships by ahmad hamdan, source=Web Response No relevant relationships by Carlos Matute Martinez, source=Web Response

A Late Presentation of Unilateral Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) Syndrome

Although uncommon, idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome can lead to vision threatening complications if not diagnosed and treated appropriately. This case report describes an atypical presentation of unilateral IRVAN syndrome, which resulted in significant visual loss in an elderly male. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/a-late-presentation-of-unilateral-idiopathic-retinal-vasculitis-aneurysms-and-neuroretinitis-irvan-syndrome)

Relevance of cutaneous manifestations and antineutrophil cytoplasmic antibody status in eosinophilic granulomatosis with polyangiitis

AbstractEosinophilic granulomatosis with polyangiitis (EGPA) belongs to the family of antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis syndromes. Although increasing evidence indicates the correlation of clinical and histological features with the presence or absence of ANCAs in EGPA, the variations in cutaneous features according to their ANCA status have not been fully described. Here, we retrospectively reviewed the clinical and histological findings of 9 cases of EGPA, who presented with cutaneous lesions. Our data indicate that ANCA‐positive patients often present with blisters, systemic inflammatory symptoms, and are prone to receive a higher dose of oral prednisolone and additional immunosuppressive therapies.