Vasculitis Mimics Research Articles

Lane–Hamilton Syndrome: A Vasculitis Mimic Presenting with Diffuse Alveolar Haemorrhage and Positive ANCA Testing

Lane–Hamilton Syndrome: A Vasculitis Mimic Presenting with Diffuse Alveolar Haemorrhage and Positive ANCA Testing

Management of ANCA-associated vasculitides

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune-mediated inflammation of small and medium-sized vessels that can affect virtually any organ system and bears the risk of irreversible organ damage. Without treatment the mortality rates are high, which necessitates rapid diagnosis and initiation of treatment. Histological confirmation, which is not feasible in all cases, should be strived for, especially to delineate differential diagnoses and vasculitis mimics. The new American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria are primarily designed for study purposes and show limitations in the routine application. Globally, the recently updated EULAR recommendations represent the most up to date management guidelines. Therapeutically, rituximab and cyclophosphamide in combination with glucocorticoids remain the pillars of treatment in remission induction for severe organ-threatening and life-threatening diseases. For the first time, mepolizumab and avacopan represent approved treatment options for specific entities that make a significant contribution to steroid reduction. New attention has been paid to patient-reported outcomes, for which adisease-specific outcome questionnaire is now available.

Cocaine-induced vasculitis and mimics of vasculitis

Cocaine is apsychotropic tropane alkaloid and stimulant drug. Nasal insufflation of cocaine powder is acommon route of administration. In Germany, cocaine is frequently adulterated with levamisole, an anthelminthic drug with immunomodulatory effects. Both substances are linked to various autoimmune conditions. Cocaine-induced midline destructive lesions cause aprogressive destruction of osteocartilaginous structures within the upper respiratory tract and can mimic localized granulomatosis with polyangiitis. In addition, systemic vasculitis due to cocaine and levamisole has been reported. Differentiation of these conditions from primary vasculitis can be challenging because antineutrophil cytoplasmic antibodies (ANCA) are commonly detected. Early diagnosis of these conditions is crucial as clinical improvement is closely related to drug cessation.

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis.

To develop and validate revised classification criteria for granulomatosis with polyangiitis (GPA). Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 5 phases: 1) identification of candidate criteria items using consensus methodology, 2) prospective collection of candidate items present at the time of diagnosis, 3) data-driven reduction of the number of candidate items, 4) expert panel review of cases to define the reference diagnosis, and 5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators. The development set for GPA consisted of 578 cases of GPA and 652 comparators. The validation set consisted of an additional 146 cases of GPA and 161 comparators. From 91 candidate items, regression analysis identified 26 items for GPA, 10 of which were retained. The final criteria and their weights were as follows: bloody nasal discharge, nasal crusting, or sino-nasal congestion (+3); cartilaginous involvement (+2); conductive or sensorineural hearing loss (+1); cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3 ANCA positivity (+5); pulmonary nodules, mass, or cavitation on chest imaging (+2); granuloma or giant cells on biopsy (+2); inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1); pauci-immune glomerulonephritis (+1); perinuclear ANCA or antimyeloperoxidase ANCA positivity (-1); and eosinophil count ≥1 × 109 /liter (-4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having GPA if the cumulative score was ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 93% (95% confidence interval [95% CI] 87-96%) and the specificity was 94% (95% CI 89-97%). The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for GPA demonstrate strong performance characteristics and are validated for use in research.

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis.

To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA). Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 5 phases: 1) identification of candidate criteria items using consensus methodology, 2) prospective collection of candidate items present at the time of diagnosis, 3) data-driven reduction of the number of candidate items, 4) expert panel review of cases to define the reference diagnosis, and 5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators. The development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1 × 109 /liter (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3 ANCA positivity (-3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1), and hematuria (-1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% confidence interval [95% CI] 77-91%) and the specificity was 99% (95% CI 98-100%). The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for EGPA demonstrate strong performance characteristics and are validated for use in research.

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Microscopic Polyangiitis.

To develop and validate classification criteria for microscopic polyangiitis (MPA). Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 5 phases: 1) identification of candidate items using consensus methodology, 2) prospective collection of candidate items present at the time of diagnosis, 3) data-driven reduction of the number of candidate items, 4) expert panel review of cases to define the reference diagnosis, and 5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators. The development set for MPA consisted of 149 cases of MPA and 408 comparators. The validation set consisted of an additional 142 cases of MPA and 414 comparators. From 91 candidate items, regression analysis identified 10 items for MPA, 6 of which were retained. The final criteria and their weights were as follows: perinuclear antineutrophil cytoplasmic antibody (ANCA) or anti-myeloperoxidase-ANCA positivity (+6), pauci-immune glomerulonephritis (+3), lung fibrosis or interstitial lung disease (+3), sino-nasal symptoms or signs (-3), cytoplasmic ANCA or anti-proteinase 3 ANCA positivity (-1), and eosinophil count ≥1 × 109 /liter (-4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having MPA with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 91% (95% confidence interval [95% CI] 85-95%) and the specificity was 94% (95% CI 92-96%). The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for MPA are now validated for use in clinical research.

AB0783 “RICE AND SARDINES”: AN UNUSUAL CAUSE OF RASH AND JOINT SWELLING

Background:Systemic vasculitis presents with numerous widespread manifestations. Various diseases can masquerade as systemic vasculitis (1). It is crucial to carefully evaluate patients with unusual presentations to look for vasculitis mimics. Scurvy, a disease caused by severe and prolonged vitamin C deficiency, still occurs in industrialized countries (2). Ascorbic acid is involved in various biologic processes including the synthesis of mature collagen. Lack of ascorbic acid especially affects blood vessel integrity leading to haemorrhagic manifestations characteristic of scurvy (3).Objectives:Case report highlighting that scurvy is still prevalent in the west and may present with features resembling vasculitis.Methods:Information was obtained from the patient’s medical records.Results:A 61 year old Caucasian female presented with a 3 weeks’ history of bilateral lower limb pain, rash and swelling. She was referred to acute medicine to exclude deep vein thrombosis suspected by her general practitioner. She was previously fit and well apart from diagnosis of coeliac disease and various food intolerances. She believed her symptoms started following a recent course of Metronidazole for suspected dental infection which manifested with gum bleeding.She was found to have purpuric spots and ecchymosis bilaterally in her legs, from the toes to the groin. The left knee was swollen with evidence of hematoma around the joint.She denied fever, weight loss, night sweats, arthralgia, myalgia, rash, Raynaud’s, alopecia, mouth or genital ulcers, ear-nose-throat manifestations. She denied chest pain, difficulty in breathing or symptoms of peripheral neuropathy. There was no history of asthma, inflammatory bowel disease or uveitis. She reported occasional alcohol intake, she did not smoke cigarettes nor use recreational drugs. Examination otherwise revealed no other major abnormalities with normal vital signs.Initial differential diagnosis included platelet or coagulation disorders or vasculitis. Initial blood tests revealed normal full blood count, coagulation screen, ESR, CRP, liver and renal biochemistry.Urine dip was positive for blood but not for protein. Knee aspiration was attempted but no fluid could be aspirated. Knee x-ray was unremarkable. ANA, ANCA and anti-GBM antibody tests were negative.On further review and enquiry into her symptoms and lifestyle she admitted having a diet exclusively of “rice and sardines” for nearly 2 years. This information along with clinical features led to the suspicion of Vitamin C deficiency that was subsequently confirmed. Ascorbic acid levels were <2.8micromol/l (normal range 26.1-84.6.micromol/l).Replenishment of vitamin C was followed by a rapid and sustained recovery. Detailed investigation for additional macro and micronutrient deficiencies were undertaken with referral to a dietician and to gastroenterology.Conclusion:This case illustrates scurvy can present in previously “well” individuals and should be considered as a possible differential diagnosis of vasculitic lesions in the appropriate clinical context. Detailed dietary history is invaluable.

COVID-19: angiotensin II in development of lung immunothrombosis and vasculitis mimics.

From a radiological perspective, there is strong evidence that the initial pathological process of COVID-19 lung disease dominantly affects the pulmonary vessels via systemic microvascular immunothrombosis, as is supported by pathological reports.1 We read with considerable interest the recent Viewpoint by Dennis McGonagle and colleagues on COVID-19 vasculitis and vasculitis mimics.2

COVID-19 vasculitis and novel vasculitis mimics.

COVID-19 has been occasionally linked to histologically confirmed cutaneous vasculitis and a Kawasaki-like vasculitis, with these entities generally having minimal or no lung involvement and a good prognosis. Unlike these vasculitis types, patients with severe COVID-19 pneumonia can develop cutaneous vasculitis-like lesions and systemic arterial and venous thromboemboli, including cryptogenic strokes and other vasculopathy features. Proposed underlying mechanisms for these severe manifestations have encompassed immune dysregulation, including an anti-phospholipid syndrome-like state, complement activation, viral dissemination with direct systemic endothelial infection, viral RNAaemia with immunothrombosis, clotting pathway activation mediated by hypoxaemia, and immobility. In this Viewpoint, we highlight how imaging and post-mortem findings from patients with COVID-19 indicate a novel thrombosis in the pulmonary venous territory distal to the alveolar capillary bed, a territory that normally acts as a clot filtration system, which might represent an unappreciated nidus for systemic microembolism. Additionally, we suggest that this mechanism represents a novel vasculitis mimic related to COVID-19 that might lead to cryptogenic strokes across multivessel territories, acute kidney injury with haematuria, a skin vasculitis mimic, intestinal ischaemia, and other organ ischaemic manifestations. This finding is supported by pathological reports of extensive pulmonary venular thrombosis and peripheral organ thrombosis with pauci-immune cellular infiltrates. Therefore, severe COVID-19 pneumonia with extensive pulmonary intravascular coagulopathy might help to explain the numerous systemic complications of COVID-19, in which the demonstration of direct organ infection has not adequately explained the pathology.

Mimics of vasculitis

While prompt diagnosis of vasculitis is important, recognition of vasculitis mimics is equally essential. As in the case of vasculitis, an approach to mimics based on the anatomic size of vessels can be useful. Infections can mimic vasculitis of any vessel size, including the formation of aneurysms and induction of ANCAs. Genetic disorders and vasculopathies are important considerations in large and medium vessel vasculitis. Cholesterol emboli, thrombotic conditions and calciphylaxis typically affect the medium and small vessels and, like vasculitis, can cause cutaneous, renal and CNS manifestations. Reversible cerebral vasoconstriction syndrome is important to distinguish from primary angiitis of the CNS. As an incorrect diagnosis of vasculitis can result in harmful consequences, it is imperative that the evaluation of suspected vasculitis includes consideration of mimics. We discuss the above mimics and outline a systematic and practical approach for differentiating vasculitis from its mimics.

19 Calciphylaxis Presenting as a Vasculitis Mimic in a Patient with Acute Kidney Injury

19 Calciphylaxis Presenting as a Vasculitis Mimic in a Patient with Acute Kidney Injury

A Review of Primary Vasculitis Mimickers Based on the Chapel Hill Consensus Classification.

Primary systemic vasculitides are rare diseases that may manifest similarly to more commonly encountered conditions. Depending on the size of the vessel affected (large vessel, medium vessel, or small vessel), different vasculitis mimics must be considered. Establishing the right diagnosis of a vasculitis mimic will prevent unnecessary immunosuppressive therapy.

An ischemic stroke as the presenting manifestation of rapidly progressive primary angiitis of central nervous system in a 17-year-old boy

BackgroundChildhood primary angiitis of the central nervous system (cPACNS) is an increasingly recognized inflammatory brain disease in children. Case presentationWe present a case of a 17-year-old boy with recurrent ischemic events over a short time period. Diagnosis of angiography positive cPACNS was made based on neuroimaging findings while secondary causes or mimics of CNS vasculitis were meticulously excluded. The patient exhibited rapid deterioration of his condition with poor initial response to immunosuppressive treatment. ConclusionsRecognition of cPACNS remains a challenge because of rarity of disease, unexplained etiopathogenesis, protean clinical presentation, as well as lack of specific laboratory and neuroimaging markers.

Vasculitis mimics

Vasculitis mimics need to be differentiated from primary and secondary vasculitides as described in the Chapel Hill nomenclature. The clinical symptomatology resembles that of vasculitis of small and medium, rarely also large vessels and hence imitates the classical vasculitic disorders. Pathogenetically, the causes are partly genetic mutations, embolization syndromes, infections and substance abuse. Also, B‑cell lymphomas can mimic vasculitis. The present manuscript summarizes the vasculitis mimics.

Vasculitis: A Checklist to Approach and Treatment Update for Dermatologists.

Vasculitis poses a great diagnostic, investigative and therapeutic challenge to the treating physician. The classification of vasculitides itself still eludes universal acceptance. Comprehensive management comprises establishing the diagnosis of true vasculitis after ruling out vasculitis mimics, finding the etiology if feasible, assessing the caliber of the vessels involved, deciphering the pathological process of vessel damage, investigating for the existence and extent of systemic involvement and finally planning the therapy in the background of co-morbidities. Successful management also entails regular monitoring to foresee complications arising from the disease process itself as well as complications of immunosuppressive treatment. Although steroids remain first line drug, biologics are emerging as popular agents in the treatment of immune-mediated vasculitis. Triphasic treatment is the best plan of action comprising induction, maintenance of remission and treatment of relapses.

Vasculitis and vasculitis-like manifestations in monogenic autoinflammatory syndromes.

Monogenic autoinflammatory syndromes are a rare group of disorders characterized by periodic episodes of systemic inflammation of endogenous origin. Sometimes, these diseases may present with features akin to vasculitis. We conducted a literature review on such vasculitic manifestations in described monogenic autoinflammatory syndromes utilizing the Online Mendelian Inheritance in Man (OMIM), Medline, and Scopus databases. Our search identified that Familial Mediterranean fever (FMF) can manifest with features of either small, medium, large, or variable-vessel vasculitis. Stimulator of interferon gene (STING)-associated vasculopathy of infancy (SAVI) is an interferonopathy that can mimic the presentation of medium-vessel or small-vessel vasculitis, whereas deficiency of adenosine deaminase 2 (DADA2) is another such mimic of medium-vessel vasculitis, associated in a significant number of patients with features of immunodeficiency. Occasional reports exist of vasculitic manifestations in tumor necrosis factor (TNF) receptor-associated periodic fever syndrome (TRAPS) and chronic infantile neurologic cutaneous and articular disorder (CINCA), whereas mevalonate kinase deficiency can also mimic the presentation of small- or medium-vessel vasculitis. Clinicians should be aware of the possibility of autoinflammatory disease presenting as vasculitis to diagnose and treat the same appropriately.

Multiple Myeloma: A Rare Haematological Cause of Nasal Septal Perforation and a Vasculitis Mimic.

Multiple Myeloma: A Rare Haematological Cause of Nasal Septal Perforation and a Vasculitis Mimic.

Autoantibodies in systemic vasculitis.

Autoantibodies in systemic vasculitis.

Segmental arterial mediolysis, a vasculitis mimic: Case report and review of literature

Segmental Arterial Mediolysis (SAM) is a non-inflammatory vascular condition that most commonly affects the medium sized vessels in the abdomen including splanchanic and renal arteries. Its angiographic appearance closely mimics other conditions affecting these blood vessels, such as polyarteritis nodosa (PAN), infections such as syphilis and fibromuscular dysplasia. SAM is under-recognized, however it is important to distinguish it from PAN because the management of the two conditions differs significantly. Here we report a case of a woman presenting with intra-abdominal bleeding, with imaging evidence for large retroperitoneal hematoma. Arteriography revealed diffuse, segmental dilation and aneurysms of the arterial supply to the entire colon. Discussion includes review of current understanding of physiopathology, clinical features, imaging, and differential diagnosis of this rare disease. Recent highlights on the disease course are also discussed.

Several topics of pulmonary vasculitis and pulmonary hemorrhage syndromes

Vasculitis is defined as inflammation of a vessel wall. Hemorrhage is defined as the escape of blood from vessels. If pulmonary vasculitis and pulmonary hemorrhage are directly connected that inflammation of blood vessels leads to rupture and then hemorrhage, the classification of the pulmonary vasculitis and pulmonary hemorrhage syndromes would be simple. There are many schemes for classifying vasculitis, such as McCluskey 1983, Jennette 1997, Vuong 2002, and Saleh 2005. Classifications can be made by etiology, histologic reaction, size and type of vessels, and associated diseases. Recently CHCC1994 is revised to CHCC 2012. Vasculitis and mimics of pulmonary vasculitis includes idiopathic necrotizing vasculitis syndromes, miscellaneous systemic disorders including collagen-vascular diseases, pulmonary hemorrhage syndromes, vascular involvement in lymphoproliferative disorders, pulmonary hypertensive arterial changes, and granulomatous vasculitis. My presentation reviews all over and focuses on pathological features of pulmonary vasculitis and pulmonary hemorrhage syndromes.