Diagnosis Of Systemic Vasculitis Research Articles

Laboratory diagnostics for vasculitis beyond antineutrophil cytoplasmatic autoantibodies

The diagnosis of systemic vasculitis (SV) is amajor clinical challenge due to the very different forms of presentation and requires an interdisciplinary approach. Targeted laboratory diagnostics support making the diagnosis, differential diagnosis and classification and are also akey component in the detection of active organ manifestations and treatment complications. The basic laboratory tests include the erythrocyte sedimentation rate (ESR), C‑reactive protein (CRP), blood count, serum creatinine, urinalysis, specific autoantibodies, complement, immunoglobulins, cryoglobulins and hepatitisB and C serology. Antineutrophil cytoplasmic autoantibodies (ANCA), antiglomerular basement membrane antibodies (anti-GBM antibodies) and anti-C1q antibodies are valuable laboratory markers for the diagnosis of the various forms of small vessel vasculitis. There are no specific laboratory tests for the diagnosis of medium and large vessel vasculitis. Despite advances in our understanding of the pathogenesis of vasculitis, no biomarkers have yet been identified that can be reliably used to guide treatment or that are useful in distinguishing vasculitis from other inflammatory diseases such as infections or treatment complications.

Клинический случай тяжелого течения геморрагического васкулита

Diagnosis of systemic vasculitis often causes difficulties in clinical practice. A clinical case characterized by multiple organ and comorbid pathology is presented. The manifestation of intestinal bleeding was the reason for the diagnosis of severe ulcerative colitis. The appearance of a characteristic hemorrhagic rash, followed by renal pathology with hematuria and severe nephrotic syndrome, made it possible to establish the correct diagnosis.

Pattern of prescription drugs used for patients diagnosed with systemic vasculitis

IntroductionVasculitis is a heterogeneous group of diseases characterized by inflammation of the blood vessel wall, which can cause thrombosis, stenosis, or occlusion. Its pharmacological management involves corticosteroids and conventional and biological disease-modifying antirheumatic drugs. ObjectivesThe objective was to determine the different types of systemic vasculitis and their pharmacological treatment in a group of patients from Colombia, 2019–2020. Materials and methodsThis was a cross-sectional study that identified different types of systemic vasculitis and their pharmacological management in a group of patients from a drug-dispensing database of approximately 8.5 million people. Sociodemographic, comorbidity and pharmacological variables were considered. A descriptive analysis was performed. ResultsA total of 621 patients with a diagnosis of systemic vasculitis were identified. The patients had a median age of 55.0 years, and 74.4% were women. The most common vasculitis types were those limited to the skin (30.1%), granulomatosis with polyangiitis (17.6%), and necrotizing vasculopathy (10.8%). A total of 81.0% of cases received corticosteroid prescriptions, 44.0% received azathioprine, and 24.0% received methotrexate; only 1.6% were prescribed biological antirheumatic drugs. Cardiovascular diseases were the most common comorbidity. ConclusionsThe pattern of prescription medications used for patients diagnosed with systemic vasculitis is heterogeneous. Much of this population has associated comorbidities, which increases the use of medications according to the current management guidelines.

Mistakes in the diagnosis of systemic vasculitis in the practice of a therapist

Mistakes in the diagnosis of systemic vasculitis in the practice of a therapist

Pattern of prescription drugs used for patients diagnosed with systemic vasculitis

IntroductionVasculitis is a heterogeneous group of diseases characterized by inflammation of the blood vessel wall, which can cause thrombosis, stenosis, or occlusion. Its pharmacological management involves corticosteroids and conventional and biological disease-modifying antirheumatic drugs. ObjectivesThe objective was to determine the different types of systemic vasculitis and their pharmacological treatment in a group of patients from Colombia, 2019–2020. Materials and methodsThis was a cross-sectional study that identified different types of systemic vasculitis and their pharmacological management in a group of patients from a drug-dispensing database of approximately 8.5 million people. Sociodemographic, comorbidity and pharmacological variables were considered. A descriptive analysis was performed. ResultsA total of 621 patients with a diagnosis of systemic vasculitis were identified. The patients had a median age of 55.0 years, and 74.4% were women. The most common vasculitis types were those limited to the skin (30.1%), granulomatosis with polyangiitis (17.6%), and necrotizing vasculopathy (10.8%). A total of 81.0% of cases received corticosteroid prescriptions, 44.0% received azathioprine, and 24.0% received methotrexate; only 1.6% were prescribed biological antirheumatic drugs. Cardiovascular diseases were the most common comorbidity. ConclusionsThe pattern of prescription medications used for patients diagnosed with systemic vasculitis is heterogeneous. Much of this population has associated comorbidities, which increases the use of medications according to the current management guidelines.

Гранулематоз с полиангиитом: клинический случай

: Diagnosis of systemic vasculitis is difficult due to a low-symptomatic or atypical debut, as well as an insufficiently bright clinical manifestation. The article presents a clinical case of granulomatosis with polyangiitis in a young man with multiple organ symptoms, stretched over time.

Características clínicas y mortalidad de los pacientes con vasculitis sistémica

Introduction: Systemic vasculitis belongs to a group of rare, serious multisystemic diseases, that without treatment can be life-threatening. Objective: to study and to determine the clinical characteristics and mortality of patients with systemic vasculitis admitted in the period 2000-2020, in two public hospitals in Paraguay. Material and Method: a retrospective, descriptive, observational, cross-sectional study with an analytical component of 41 adult patients with a diagnosis of systemic vasculitis. The diagnosis was made by clinical, laboratory, imaging and histological findings. Risk factors for mortality were established by the chi-square test, considering a p value less than p <0.05 to be significant. Results: 41 patients were studied, 24 women (58.54%) and 17 men (41.46%), vasculitis associated with ANCA 31 (77.5%), 17 with granulomatosis with polyangiitis and 14 patients with microscopic polyangiitis. Polyarteritis nodosa 5 (12.5%), cryoglobulinemia 2 (5%), anti-glomerular basement membrane antibody disease 2 (5%), Takayasu arteritis 3 (7.32%), progression to chronic kidney disease 5 (12.50 %)). Mortality 16 (39%). The factors related to mortality were: alveolar hemorrhage, acute kidney injury, rapidly progressive nephritis, bilateral cottony infiltrate (p = ≤ 0.05). Conclusion: The most frequent vasculitis was associated with ANCA, followed by parteritis nodosa. We found a high mortality rate, mainly secondary to lung and kidney involvement.

The Delay and Costs of Diagnosing Systemic Vasculitis in a Tertiary-Level Clinic.

IntroductionThe diagnosis of systemic vasculitis is a challenge because of the heterogeneity of clinical manifestations. The aim of this study is to analyze the diagnostic delay in systemic vasculitis, the total costs during the first year of care, and how the diagnostic delay affects the costs in a tertiary health care facility.MethodsPatients with a new diagnosis of systemic vasculitis between 2010 and 2018 were identified from hospital records. The diagnostic delay and health care costs were evaluated during the diagnostic period and within 12 months after the first contact with tertiary health care. Vasculitis-related costs were recorded as true costs charged. A total of 317 patients fulfilled the study criteria. The diagnoses were grouped into three clinically relevant groups: IgA vasculitis and other small-vessel vasculitis (n = 64), ANCA-associated vasculitis (AAV) (n = 112), and large-vessel vasculitis (LVV) (n = 141).ResultsThe diagnostic delay from the first referral to tertiary-level clinic was shortest in the LVV group and longest in the AAV group. Total costs during the diagnostic period were the highest in the AAV group (median = €6754 [IQR €8812]) and lowest in the LVV group (median = €3123 [IQR €4517]), p < 0.001. There was a significant positive correlation between the diagnostic delay and total costs during the diagnostic period and 12 months (rs = 0.38, p < 0.001 and rs = 0.34, p < 0.001, respectively). In a linear model, the inpatient days and the number of laboratory tests were the strongest predictors (p < 0.001) of a higher treatment cost during the diagnostic period.ConclusionsThere is a substantial diagnostic delay that correlates significantly with the costs in tertiary-level health care when diagnosing systemic vasculitis.Electronic supplementary materialThe online version of this article (10.1007/s40744-020-00266-9) contains supplementary material, which is available to authorized users.

Abstract 13574: A Rare Case of Giant Cell Arteritis With Coronary Artery Involvement

Introduction: Coronary artery involvement in patients with systemic vasculitis is relatively rare, but can be life threatening. There is a well-known occurrence of coronary artery vasculitis in Kawasaki disease and Takayasu arteritis, however, coronary arteritis caused by giant cell arteritis (GCA) has been documented in only a handful of reports. Case report: A 65-year-old woman with a history of GCA complicated by cerebrovascular stenosis s/p bypass and coronary artery disease (CAD) s/p DES to mid-left anterior descending (LAD) 3 years prior, without cardiac risk factors, who presented with shortness of breath and 2-3 hours of progressive non-radiating pressure like midsternal chest pain. Vital signs were stable with an unremarkable physical exam. EKG showed new T-wave inversions in V4-V6, I, II, aVL. Labs were significant for Trop T 0.17 and CRP 2.02. Echocardiogram showed EF 30% with wall motion abnormalities (ECHO 9 months prior showed EF 69% with no wall motion abnormalities). Coronary angiography revealed 99% in-stent-restenosis (ISR) of mid-LAD, 50% tubular OM1 stenosis, and 100% RCA occlusion with right-to-right collateral (figure 2). The patient underwent CABG with a left internal mammary artery graft to LAD and saphenous vein graft to RCA. Post-operative EF was 50%, and she was discharged on post-operative day 5. Discussion: In patients with systemic vasculitis and no identifiable CAD risk factors presenting with acute coronary syndrome, there has to be a high suspicion for coronary arteritis. Our case strongly suggests that GCA can involve the coronary arteries given the rapid progression of ISR with triple vessel disease in the absence of cardiac risk factors. Although advancements in imaging techniques have led to improvements in the diagnosis of systemic vasculitis, standardized imaging for evaluating coronary vasculature is lacking. Larger retrospective case studies are needed in order to clearly delineate the association between GCA and CAD.

ANCA-associated vasculitis in a 14 years-old patient: a clinical case

Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.

49. Giant cell arteritis and Takayasu arteritis: two sides of the same coin in an elderly female?

49. Giant cell arteritis and Takayasu arteritis: two sides of the same coin in an elderly female?

Investigations in systemic vasculitis. The role of the laboratory.

The diagnosis of systemic vasculitis is challenging. Laboratory testing may provide useful information. Routine laboratory tests include erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), blood count, serum creatinine, urinalysis, specific autoantibodies, complement, immunoglobulin, cryoglobulin, and Hepatitis B and C serology. Although ESR and CRP are often helpful for the diagnosis of vasculitis, they are nonspecific and do not help in distinguishing between vasculitis disease activity and a concomitant infection or another source of inflammation. A few autoantibodies are helpful for diagnosis, such as anti-neutrophil cytoplasmic antibodies (ANCAs) (in ANCA-associated small-vessel vasculitis), anti-glomerular basement membrane (GBM) antibodies (in anti-GBM antibody disease), and anti-C1q antibodies (in immune complex-associated small-vessel vasculitis). The 2017 revised consensus recommendations on ANCA testing state that high-quality antigen-specific immunoassays are the preferred screening methodology for the diagnosis of ANCA-associated vasculitis. ANCA subtypes (proteinase-3-ANCA and myeloperoxidase-ANCA) are associated with different epidemiological, genetic, and clinical features.

Systemic Diseases in Dakar (Senegal): Spectrum, Epidemiological Aspect and Diagnostic Time-Limit

Introduction: Systemic diseases have been the subject of few studies in the African literature and have probably been under-estimated. The objective of our study was to specify their spectrum, their epidemiological aspects and diagnostic delay in Internal Medicine Departments of Dakar (Senegal). Material and Method: It was a multicentric retrospective and descriptive study regarding all systemic diseases during 119 months from 1st January 2005 to 30 November 2014 in 5 hospital centers down Dakar. Systemic diseases were retained according to their international consensus criteria. Results: During the studying period, 726 patients were included with 632 women and 94 men (sex ratio of 0.14). The average age was 43.76 years. Inflammatory rheumatoid family history was noted in 10.06% of cases. Rheumatoid arthritis (RA) was the predominant affection, recorded on 564 patients, isolated or associated with other systemic diseases. It was followed in a decreasing order, in the systemic auto-immune diseases sub-groupe, by systemic lupus (56 cases), Sjögren’s syndrome (32 cases), Systemic Sclerosis (26 cases), Idiopathic inflammatory myopathies (21 cases), Undifferentiated connective tissue diseases (20 cases), Anti Phospholipid’s syndrome (6 cases) and Mixed connective tissue disease (6 cases). A diagnosis of systemic vasculitis was recorded in 19 patients. The other systemic affections were represented by systemic sarcoidosis (8 cases), Adult-onset Still’s disease (03 cases), amyloidosis (02 cases) and 02 cases of systemic syndrome associated to immunodeficiency. The mean diagnostic delay duration before the diagnostic was 3.46 years. Conclusion: Systemic diseases in internal medicine are characterized by their diversity, the clear predominance of RA, and significant diagnostic delay.

A CLINICAL CASE OF POLYARTERITIS NODOSA

Polyarteritis nodosa refers to the group of systemic vasculitides affecting small and medium arteries. It is characterized by inflammation of the vascular wall with the development of aneurysmus and thrombosis. The article presents a rare clinical case of polyarteritis nodosa, which has not been diagnosed for a long time and gives the description of the diagnostic search, performance of differential diagnosis as a result of which the diagnosis of systemic vasculitis has been defined.

Myeloperoxidase-antineutrophil Cytoplasmic Antibodies (MPO-ANCA) and Proteinase 3-ANCA without Immunofluorescent ANCA Found by Routine Clinical Testing.

Concurrent testing for serum antineutrophil cytoplasmic antibodies (ANCA) by indirect immunofluorescence (IF) and by antiproteinase 3 (PR3)/antimyeloperoxidase (MPO) antibody assays may identify patients with PR3-ANCA or MPO-ANCA despite a negative IF (IF-negative MPO/PR3-positive); however, the significance of this result is not clear. We sought to determine whether IF-negative, MPO/PR3-positive results identified any cases of clinically meaningful systemic vasculitis at our institution. We conducted a retrospective chart review of all IF-negative, MPO/PR3-positive patients identified at our institution over a 2-year period. Of the 2345 samples tested over 2 years, 1998 were IF-negative. Among these IF-negative samples, 49 samples (2.5%) derived from 38 patients tested positive for MPO-ANCA or PR3-ANCA. Only 1 IF-negative, MPO/PR3-positive patient was subsequently diagnosed with ANCA-associated vasculitis (AAV). Eleven IF-negative, MPO/PR3-positive patients (29%) had been previously diagnosed and treated for AAV, all with positive IF and antibody tests prior to treatment. Four patients had evidence of cutaneous vasculitis not attributed to AAV, while several of the remaining IF-negative, MPO/PR3-positive patients had other immunologic disorders, including systemic lupus erythematosus (5 patients) and inflammatory bowel disease (3 patients). In this real-life cohort assayed simultaneously by IF and multiplexed bead assays, the detection of MPO-ANCA or PR3-ANCA without a positive IF rarely led to a new diagnosis of systemic vasculitis, and was more likely to occur in the context of a non-vasculitic inflammatory condition. Our results suggest that concurrent IF and MPO/PR3 testing may be of limited use in preventing a missed diagnosis of new-onset AAV.

Systemic Vasculitis in Various Organs of 33 Rheumatoid Arthritis and 11 Progressive Systemic Sclerosis Patients – A Comparative Postmortem Study

Objective: The aim of this study was (1) to compare the prevalence and severity of Systemic Vasculitis (SV) in various organs of Rheumatoid Arthritis (RA) and of progressive Systemic Sclerosis (SSc) patients, (2) To determine the complications and lethal outcome caused by vasculitis in RA and SSc. Patients and Methods: Twelve organs (heart, lung, liver, spleen, kidneys, pancreas, gastrointestinal tract, adrenal glands, skeletal muscle, peripheral nerve, skin and brain) of 33 RA and of 11 SSc with SV were studied microscopically. RA and SSc were confirmed clinically according to the criteria of the American College of Rheumatology. The basic disease, the complications, and the lethal outcome caused by vasculitis were determined and analyzed retrospectively, reviewing the clinical and pathological reports, tissue samples, and the histological slides. The existence (prevalence) of vasculitis in various organs was determined based on the presence of vasculitis in blood vessels of different calibers. The severity of vasculitis in various organs was evaluated by semi-quantitative, visual estimation on a 0 to 3 plus scale (based on the number of involved vessels/light microscopic field x40). Results and Conclusions: SV was observed in all of 33 RA and 11 SSc patients with variable prevalence and severity. The average value of prevalence and severity/organ in SSc was appreciably higher than that of RA. In RA patients, not all investigated organs were involved with SV and the existence of blood vessels with vasculitis in each organ was different. In SSc patients SV was present in each of the investigated organs, but with different severity. In RA the heart, skeletal muscle, pancreas, lung, kidneys and gastrointestinal tract were most markedly involved, while in SSc the kidneys, spleen, lung, pancreas, heart and gastrointestinal tract were. The high prevalence of SV and the consecutive fibrosis in these organs identify the main targets of autoimmune processes in RA and SSc prior to the more visible skin involvement. The most markedly involved organs denote a favorable site for histological diagnosis of SV, for example skeletal muscle with peripheral nerve (sural nerve) or gastrointestinal tract in RA and the gastrointestinal tract in SSc. The course of the disease, more precisely mortality due to SV,

AB0576 Gastrointestinal Involvement among Patients with Systemic Vasculitis

BackgroundGastrointestinal involvement can be a challenging complication of systemic vasculitis. It is a relatively common finding in polyarteritis nodosa and IgA realted vasculitis, an infrequent manifestation of ANCA associated vasculitis,...

Value of muscle and nerve biopsy in the diagnosis of systemic vasculitis

Objectives: To describe our 16 years of experience performing muscle and nerve biopsy on patients with suspected systemic vasculitis. Methods: Retrospective study from our Muscle Research Unit clinical records. From January 1997 to March 2013, we conducted 234 muscle and nerve biopsies. Biopsies were performed by residents tutored by a senior specialist in Internal Medicine, who also did the pathologic study of the samples. Only one patient had a local infection and another one a local hematoma, both resolved in a few days. Two hundred and fourteen were conducted on patients with suspected systemic vasculitis because of both clinical and electromyographic features. Ten were made to search for amyloidosis and in the remaining 10 there was not enough information on our clinical records. We collected data regarding gender, age at the time of the biopsy, pathological diagnosis and type of vasculitis. Results: The mean age at the time of the biopsy was 63.4 years (21-88), and 109 were men (50.9%). Histologic diagnosis of vasculitis was observed on 85 patients (39.4%). In 39 cases in both muscle and nerve samples (45.9%), in 40 only in the nerve sample (47%) and in 6 in the muscle sample, only (6%). Five patients (2.4%) were diagnosed of vasculitis through an additional biopsy: temporal artery (4) and skin (1). With regard to the nerve samples, in 79 vasculitis was present while in the remaining 137 the vessels were normal. On the histologic study of the muscle samples, in 45 vasculitis could be demonstrated (21.0%), in 129 denervation signs were evident (60.3%) and 22 were normal (10.3%). Other unexpected diagnosis were made in 17 patients: mitochondrial changes (4), glycogenosis (3), inclusion body myositis (2), polymyositis (1) and 7 had other diagnosis. Follow-up clinical data was collected from 115 patients (53, 2%). Fourty-three patients had medium vessel vasculitis (51.2%), 24 had vasculitis affecting both small and medium vessels (28.6%) and 17 had small vessel vasculitis only (20.2%). Conclusion: Muscle and nerve biopsies are an easy, safe and fast technique to help in the diagnosis of patients with suspected systemic vasculitis. Once a nerve biopsy is being conducted, adding a muscle biopsy in the same procedure allows to diagnose 7% more patients with vasculitis, and sometimes provides an alternative diagnosis. The pathological study of these samples offers a good yield when reviewed by an expert consultant, helping to confirm or rule out the suspected diagnosis. With a short training, muscle and nerve biopsies can be successfully performed by residents under a senior specialist supervision, providing a useful technique to help in the diagnostic process of diseases frequently found in an Internal Medicine Department, such as systemic vasculitis.

FRI0230 Stable incidence rates of anca-associated vasculitides and giant cell arteritis in northern germany (schleswig-holstein) from 1998 to 2010

BackgroundLittle data is available regarding the epidemiology of systemic vasculitides (SV), particularly on their incidence. Available data is heterogenous and often derived from referral centers or small populations.ObjectivesTo assess the...

AB0466 Prevalence of systemic vasculitis in various organs – a comparative postmortem study of 36 rheumatoid arthritis and 11 progessive systemic sclerosis patients

ObjectivesThe aim of this study was to compare the incidence and severity of systemic vasculitis (SV) in various organs of rheumatoid arthritis (RA) and progressive systemic sclerosis (SSc) patients.MethodsTwelve organs...