Manifestations Of Systemic Vasculitis Research Articles

Damage to the organ of hearing and upper respiratory tract in vasculitis associated with antineutrophil cytoplasmic antibodies

Antineutrophil cytoplasmic antibodies associated vasculitis (ANCA-AV) is a systemic necrotizing granulomatous vasculitis affecting mainly small-caliber vessels. ANCA-AV occupy a special place among systemic vasculitis, which is characterized by a highly active life-threatening course of the disease, requiring rapid differential diagnosis and aggressive immunosuppressive therapy. The ANCA-AV group consists of 3 nosologies: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. The “calling card” of ANCA-AV is the lesion of the upper respiratory tract, especially the ENT organs (70–100 % of patients). The nasal cavity and paranasal sinuses are the most common areas of lesion in the head and neck (85–100 %), whereas ear damage occurs in about 35% (range, 19–61 %) of cases. Lesion of the ENT organs is typical for the debut of ANCA-AV, which makes early diagnosis difficult, since diseases of the upper respiratory tract are extremely common in all age groups. Diagnosis verification occurs mainly at the stage of generalized involvement of many organs and systems, causing severe course with the development of pulmonary-cardiac and renal insufficiency, which lead to the death of the patient. The main ENT manifestations of ANCA-AV can be grouped into several groups: sinonasal, otological, tracheobronchial, oral cavity lesions and others. Pseudotumors are often found in ANCA-AV. They are characterized by the appearance of parapharyngeal, parotid, submandibular, paratracheal volumetric formations. As a rule, the appearance of tumor-like formations is observed at an early stage of the disease and is associated with the presence of antibodies to proteinase 3, systemic manifestations of vasculitis. Pseudotumor in the ENT region may be accompanied by secondary neuropathies of cranial nerves, destruction of bone tissue, which requires histological verification of the disease.

АНЦА-ассоциированные интерстициальные заболевания легких и интерстициальная пневмония с аутоиммунными признаками

Interstitial lung disease (ILD) is a recognized manifestation of ANCA-associated vasculitis. In addition, patients with idiopathic interstitial pneumonia and ANCA positivity but without other manifestations of systemic vasculitis (ANCA-ILD) have also been reported.

Інфекційна еритема та феномен Рейно в дитини після перенесеної коронавірусної хвороби: опис клінічного випадку

Purpose - to analyze practice cases with an atypical course associated with comorbid pathology; to acquaint pediatricians and family physicians with the diagnostic search algorithm. The article summarizes literature data, and describes a clinical case of erythema infectiousum in combination with Raynaud's phenomenon, which occurred in a 9-year-old child І. with coronavirus disease in anamnesis. It was established that the nature of the pathological changes: periodic hyperemia of the cheeks and auricles, “slapped cheeks”, and, at the same time, palleness of the nasolabial triangle, a pronounced change in color, marbling of the skin on the limbs and on the buttocks, with a lacy pattern of vessels, which are provoked by exposure to cold air or psycho-emotional excitement, clearly indicate both the manifestations of parvovirus infection, and the Raynaud's phenomenon mentioned above. On the other hand, post-covid syndrome is also often accompanied by non-specific changes in the skin and its derivatives (up to 20.4% of cases) in the form of urticaria, erythema, and frostbite. In the same group of patients, there are maculopapular rashes and livedo reticularis, those could be Conclusions. During the pandemic of coronavirus infection, the issues of comorbid pathological conditions in pediatrics, which have a blurred clinical picture, an atypical course, common links of pathogenesis, in particular microcirculation disorders, and require an interdisciplinary approach to the management of these diseases, rehabilitation measures, are becoming more and more relevant. interpreted both as a manifestation of systemic vasculitis, and as independent disease. Thus, the patient was diagnosed with infectious erythema caused by parvovirus B19 in combination with Raynaud's phenomenon, probably caused by a history of coronavirus disease. The multicomponent nature of the patient's symptoms is highlighted, and the diagnostic search algorithm is shown. The study was conducted in accordance with the principles of the Declaration of Helsinki. An informed parental consent was obtained for the study in children. No conflict of interests was declared by the authors.

CHARACTERISTICS OF THE COURSE OF LEUCOCYTOCLASTIC VASCULITIS WITH SKIN LESIONS. ANALYSIS OF CLINICAL CASES

The article describes the features of the course of leukocytoclastic skin vasculitis with consideration of 3 clinical cases. Leukocytoclastic vasculitis of the skin (LCV) is an isolated cutaneous vasculitis without damage to internal organs. At present, vasculitis is considered a polyetiological disease. The most frequent causes of the development of vasculitis limited to the skin include various acute or chronic infections: bacterial, viral, and fungal. It can be caused by medications, and sometimes the cause cannot be found. When LVC is suspected, an extensive examination is usually required to determine whether the process is limited to the skin or is a manifestation of systemic vasculitis or other systemic diseases. Diagnosis and treatment of LCV is a complex problem in the practice of doctors of many specialties, such as rheumatologists, dermatologists, and therapists, family doctors, which requires close cooperation of specialists. We present 3 clinical cases of patients who were hospitalized in the clinic with symptoms of skin lesions and suspicion of LCV after taking medications. The paper describes various skin manifestations and the results of the examination and treatment. The differential diagnostics enabled us to diagnose LCV in 2 cases, in one case – generalized urticaria due to taking furazolidone. It should be noted that drug reactions with skin lesions constitute a serious medical problem, whose relevance is increasing every year. This is largely due to widespread uncontrolled drug intake among the population, and frequent polypharmacy during treatment under the supervision of medical professionals. Multidisciplinary problems, which are solved by doctors of various specialties at different stages and levels of medical care, including drug-induced skin lesions in patients, are particularly difficult. Strengthening medical control over the prescription and administration of medicinal products, and improving the awareness of doctors of all specialties regarding the diagnosis and treatment of medicinal reactions is the way to solve the problem.

Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review.

The presence of a lung lesion is common in microscopic polyangiitis (MPA), and interstitial lung disease (ILD) can lead to a poor prognosis. Although myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) are often present in patients with MPA, patients with ILD and MPO-ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Therefore, the possible association between MPO-ANCA, MPA, and idiopathic ILD remains unclear. This problematic matter has influenced the treatment strategy of MPO-ANCA-positive ILD patients without systemic vasculitis. Clinicians should undertake treatment with careful consideration of the four major causes of death in MPO-ANCA-positive ILD: acute exacerbation of ILD, progressive lung fibrosis, infectious comorbidities, and diffuse alveolar hemorrhage. Further, clinicians need to carefully judge whether inflammation or fibrosis is the dominant condition with reference to the patient’s clinical domain and radiopathological lung features. Recently, anti-fibrotic agents such as nintedanib and pirfenidone were shown to be effective in treating various etiologies associated with ILD and have thus led to the widening of treatment options. In this review, the clinical characteristics, radiopathology, prognosis, and therapeutic options in patients with MPO-ANCA-positive ILD are summarized using limited information from previous studies.

Cardiovascular complications of the coronavirus disease (COVID-19)

A new coronavirus infection (COVID-19), which has spread widely in the human population in the past few years, is often complicated by a damage to the cardiovascular system, central nervous system, gastrointestinal tract, and respiratory system organs. The review describes the features of the clinical course and treatment of SARS-CoV-2 infection, which can lead to the development of cardiac complications both in the early and long-term periods. It is assumed that there are three main causes for the development of such complications (the affinity of the virus to endothelial proteins, the damage to blood vessels and the heart as a result of the immune response of the body and the toxic effects of drugs used to treat infection). Among the pathogenic mechanisms of the course of a new coronavirus infection that can lead to the development of cardiovascular complications, the following can be distinguished: manifestations of systemic vasculitis, coagulopathy, thrombosis, the phenomenon of “cytokine storm”, the direct cytotoxic damage to the myocardium, metabolic disorders, imbalance of the ionic composition of the blood, hypoxia and its consequences. The most common complications include myocarditis with arrhythmia (sinus tachycardia, bradycardia, atrial fibrillation and flutter, QTc prolongation, sudden cardiac death), cardiomyopathy (biventricular, isolated dysfunction of the right or left ventricle), cardiogenic shock, coagulopathy, thromboembolism (including strokes), vasculitis. Following recovery from COVID-19, many patients retain (or develop) the symptoms caused by the heart damage: a pain in the region of the heart, palpitations, appearance or aggravation of chronic heart failure, decompensation of hypertension, acute coronary syndrome. The main proposed directions for minimizing the risk of developing cardiac complications in COVID-19 patients include ECG monitoring (assessment of the QT interval, rhythm, conduction and repolarization), echocardiography in patients at risk, 24-hour ECG recording in patients who report palpitations. In addition, it is recommended to adjust the dosages of cardiotoxic drugs and to be cautious when prescribing anticoagulant therapy.

Systemic Vasculitis Mimics Pancreatic Tumor

The systemic vasculitides are heterogeneous conditions of unknown etiology characterized by inflammation and necrosis of different sized blood vessels. The epidemiology of the systemic vasculitides is poorly documented, but it is a rare disease. The abdominal manifestation of systemic vasculitis (SV) is a rarity. A 67-year-old man with a history of diabetes insipidus and bilateral ear canal stenosis presented with bilateral lower abdominal pain for three days duration and postprandial nausea and regurgitation for two weeks duration. He also complained of weight loss during the last year, decrease in general performance, and impaired hearing associated with ear bleeding. The patient was diagnosed with unspecified acoustic meatus stenosis by ear, nose, and throat (ENT) specialist. Computed tomography (CT) scan revealed a pancreatic lesion, which was biopsied under endosonographic guidance without any informative result. For further histological evaluation laparotomy was indicated and multiple histological materials were taken. Unfortunately, histological results revealed no specific diagnosis either. In the blood tests there were no signs of autoimmune or rheumatologic disease. By the daily deterioration of the patient's general condition, we carried out a Positron emission tomography-CT scan, which revealed SV. Under cortisone therapy the patient’s complaints obviously declined, the patient rejected further investigation such as vascular biopsy. The intraabdominal vasculitis represents an unusual pathological finding, which, however, manifests itself only as a rare variation of an everyday medical constellation. The case impressively illustrates how difficult is to establish the correct diagnosis of abdominal vasculitis.

INTERLEUKIN-5 IS A NEW TARGET IN THE TREATMENT OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

Interleukin-5 (IL-5) is involved in the maturation and activation of eosinophils, its production is increased in patients with eosinophilic granulomatosis with polyangiitis (EGPA). This rare form of systemic vasculitides (SV) is characterized by peripheral eosinophilia and involves multiple organs and systems. The clinical introduction of glucocorticoids (GCs) and immunosuppressants has considerably improved the prognosis of EGPA, but their use is associated with significant adverse reactions and cannot be effective enough. The use of standard treatment regimens cannot always allow to achieve remission; the rate of EGPA relapses remains high. Mepolizumab is an IL-5 antagonist and a promising drug for the treatment of patients with EGPA. The presented literature review considers arguments in favor of using mepolizumab in EGPA patients and discusses its efficacy and safety. The currently accumulated data suggest that mepolizumab is effective and safe in treating patients with EGPA, what has been demonstrated in the registration double-blind, randomized, placebo-controlled MIRRA study. Treatment with the IL-5 antagonist allows one to control both the symptoms of asthma and the manifestations of SV, enhances the probability of achieving remission of EGPA, can help reduce the risk of relapse and minimize the dose of GS.

A RARE CAUSE OF DILATED CARDIOMYOPATHY IN THE SETTING OF MULTI-ORGAN INVOLVEMENT: VASCULITIS!

Cardiac involvement is one of the most serious and potentially fatal manifestations of systemic vasculitis. A 29 y/o male with a history of asthma presented with cough, vomiting, abdominal pain as well as anesthesia and weakness in his right leg. His investigations including immunological markers

Juvenile temporal arteritis: A clinicopathological multicentric experience.

Juvenile temporal arteritis (JTA) is a recently-described and little-known inflammatory disease and its etiology is undetermined. Less than forty cases have been published. This paper is aimed at reporting the largest JTA series and to compare it to literature data to better evaluate its characteristics at diagnosis, its evolution and treatment options. We conducted a retrospective and descriptive multicentric study in France by identifying adult patients under the age of 50 which had a pathological temporal artery biopsy owing to the presence of a temporal arteritis. Patients with temporal arteritis as a manifestation of systemic vasculitis were excluded. We included 12 patients and the literature review identified 32 cases described in 27 articles, thus a total of 44 patients - 34 men and 10 women - with a median age of 30 and a maximum of 44. All patients presented either a lump in the temporal region or prominent temporal arteries, and 47.7% of patients suffered from headaches. Only 11.4% of patients presented general symptoms and 6.8% a biological inflammatory syndrome; 34.1% had peripheral blood eosinophilia; 83.7% presented a single episode and complete excision without further treatment was documented for 72.7%. Pathology analysis revealed infiltrate of inflammatory cells in the arterial wall in 97.6% of patients but also sparse giant cells for 25% and granuloma for 22.9%, perivascular extension of the inflammation for 82.6%, and presence of lymphoid follicles or germinal centres for 60%. Clinical relapses were present in 16.3% of cases. JTA is a rare, localized and benign disease. The majority of cases have only one episode which is cured by local surgery.

For fulminant rapidly progressive glomerulonephritis in a 17-year-old female patient with ANCA-associated vasculitis.

A case of rapidly progressive glomerulonephritis in a 17-year-old patient associated with antibodies against the cytoplasm of neutrophils (ANCA) vasculitis - ANCA-associated vasculitis is associated with antibodies to proteinase-3 and morphological picture extracapillar glomerulonephritis with sclerotic lesion of up to 80% of the glomeruli. The peculiarity of the case is the presence of morphologically confirmed when alloimmune rapidly progressive glomerulonephritis type III a pronounced glow-focal granular nature of immunoglobulin classes G and M on the basement membrane of capillaries. The appointment of immunosuppressive therapy led to a decrease in systemic manifestations of vasculitis, but there was a rapid increase in terminal renal failure, which required substitution therapy with hemodialysis. The possible mechanisms of the rapid-training course of the disease in the observed patient, prospects for kidney transplantation are discussed.

Experience in managing patients with Churg-Strauss syndrome.

Our goal was to analyze the possibilities of impro­ving the diagnostics of CSS and to improve the effectiveness of treatment according to the existing literature and our own experience of long-term care for patients with eosinophilic granulomatosis with polyangiitis or Churg-Strauss syndrome (CSS). The medical histories of three female patients aged 26 to 46-years and a 20-year-old male patient were considered. The duration of the disease before the established diagnosis was 5-17 years. Anamnesis and medical documents analysis showed a typical CSS debut in the form of allergic rhinitis, nasal polyps, which were recurrent after polypectomy, and respiratory disorders, which were regarded as bronchitis or bronchial asthma – corresponding to the first phase, also called the prodromal or allergic stage of CSS. The prodromal period lasts up to 10 years or more and is characterized by various allergic manifestations, more often – pollinosis or bronchial asthma, that is difficult to control. But CSS can be suspected because of low effectiveness of the therapy with inhaled steroids, lack of effect of antibiotics and eosinophilia more than 10% that occurs periodically. Even in the third stage of CSS in systemic manifestations of vasculitis and severe secondary lesions of organs and tissues with functional impairment, constant intake of maintenance doses of corticosteroids and cytostatics allows to achieve stabilization of the process in patients with CSS.

Cutaneous vasculitis in rheumatologic disease: Current concepts of skin and systemic manifestations

Cutaneous vasculitis may be limited to the skin, a manifestation of systemic vasculitis, or a sign of an important underlying disease state. A thorough and systematic approach is required for accurate diagnosis and evaluation of such patients to enable appropriate management of the vasculitis and any associated condition. Occasionally, cutaneous vasculitis is a manifestation or presenting sign of connective tissue disease, such as systemic lupus erythematosus, Sjögren syndrome, or another condition. Such patients are at risk for poor outcomes related to systemic manifestations of vasculitis, as well as increased severity of the underlying disease. Recognition of this important subset of patients with skin vasculitis enables appropriate workup and successful management.

Systemic vasculitides: сlinical cases

Systemic vasculitides (SV) are a group of diseases characterized by vessel wall inflammation that leads to ischemic changes in the organs and tissues, which the respective vessels supply blood to; the spectrum of the clinical manifestations of SV depends on the type, size, location of the affected vessels, and on the activity of systemic inflammation. The etiology of the majority of primary vasculitides is unknown. It is assumed that many microorganisms may initiate inflammation of vessels of various sizes, but only some forms of SV (and in some patients) can be clearly related to certain causative and/or trigger factors (drug hypersensitivity, hepatitis B virus, hepatitis C virus, etc.). The paper gives clinical examples of the timely diagnosis and treatment of 2 SV cases in young men working on the railway of the town of Ruzaevka.

Pauci-immune glomerulonephritis in children: a clinicopathologic study of 21 patients.

Pauci-immune glomerulonephritis (GN) represents a severe form of glomerular injury and is the most common cause of crescentic GN in adults. To date, the clinicopathologic features of pauci-immune GN are not well characterized in the pediatric population. Twenty-six biopsies from 21 pediatric patients with pauci-immune GN were identified retrospectively from the pathology archives of the University of Chicago (biopsy incidence 5 % among pediatric patients). There was distinct female predominance (2.5:1) among the patient cohort. Serologic studies identified anti-neutrophil cytoplasmic antibodies (ANCA) in 85 % of patients, and 80 % had systemic manifestations of vasculitis. The median estimated glomerular filtration rate (eGFR) at presentation was 43 ml/min/1.73 m(2). Based on a previously proposed classification of ANCA-associated GN, we identified a spectrum of injury, including crescentic (n = 9), focal (n = 7), mixed (n = 5) and sclerotic GN (n = 5). Necrotizing arteritis was identified in a minority of patients (n = 3). The majority of those patients for whom data were available had been treated with cyclophosphamide and corticosteroids, with or without rituximab. Of the 21 pediatric patients, 58 % had developed chronic kidney disease at follow-up (eGFR <90 ml/min/1.73 m(2)), of whom 85 % of those had crescentic, mixed or sclerotic GN. Pediatric patients with pauci-immune GN are similar to their adult counterparts in terms of clinical manifestations and histopathologic findings. Among the 21 patients in our study, those with focal GN had the best outcomes while patients with crescentic, mixed or sclerotic GN overwhelmingly had a poor long-term outcome for kidney function.

Neurological manifestations of systemic vasculitis: Single centre experience from a tertiary care hospital in North India

Neurological manifestations of systemic vasculitis: Single centre experience from a tertiary care hospital in North India

AB0566 Arthritis in Systemic Vasculitis: A Retrospective Analysis

BackgroundFew data are available on the prevalence of arthritis in vasculitides.ObjectivesTo evaluate the prevalence and the features of arthritis in our cohort of patients with systemic vasculitis.MethodsData were collected from...

Diabetes insipidus and secondary hypogonadism due to vasculitis of the pituitary gland

A 36-year-old man was admitted to the hospital with polyuria, nycturia, polydipsia (he drank 8 – 10 liters a day yet he still felt thirsty) as well as headache, dizziness, fatigue and loss of efficiency since 4 weeks. His medical history was otherwise normal. Clinical examination revealed no abnormalities. The polydipsia in combination with low urine osmolality (102 mosm/kg) were highly suggestive of diabetes insipidus. Therefore we performed a water restriction test. With water deprivation, diuresis and low urine osmolality persisted and serum sodium levels rose from 145 mmol/l to 154 mmol/l. Oral desmopressin resulted in normalisation of serum sodium as well as urine osmolality, confirming the diagnosis of central diabetes insipidus. Further endocrine examinations were consistent with a secondary hypogonadism (Testosteron 6,98 nmol/l, LH 3,4 mIU/ml, FSH 1,9 mIU/l). The other hypothalamic-pituitary axes were not affected. Pituitary MRI revealed a contrast-pooling pituitary lesion (8 × 7 x 6 mm), suitable for either a pituitary adenoma, a craniopharyngeoma or a lymphocytic hypophysitis. A diagnostic biopsy was performed. The histology demonstrated an active vasculitis of the small vessels without depositions of complement or IgG or granulomas. All immunological tests (e.g. ANCA, IL-2, ACE, neopterin, immunglobulins) were normal. We found no other manifestations of systemic vasculitis. In the CT scan of the thorax we found no indication for sarcoidosis. The patient started treatment of high-dose steroids and azathioprine. He also requires testosterone and desmopressin substitution. In summary, we report the rare case of a patient presenting with diabetes insipidus and secondary hypogonadism due to restricted vasculitis of the pituitary gland without signs of systemic vasculitis.

AB0468 Relapsing and systemic nature of temporal arteritis

BackgroundTemporal arteritis (TA) with or without polymyalgia rheumatica (PMR) is a systemic disorder involving different size of arteries and even veins with a predilection for branches of temporal arteries. Some...

Genomics of vasculitis: lessons from mouse models.

A genome analysis of mouse models may shed some light on the complex clinicopathological manifestations of systemic vasculitis. In the study of susceptibility loci to vasculitis in MRL mouse models, we found that systemic vasculitis developed through the cumulative effect of multiple gene loci, each of which by itself did not have a significant effect in inducing the related phenotype, thus indicating a polygenic system. The mice developed vasculitis in an additive manner with a hierarchical effect. Some of the susceptibility loci seemed to be common to those in other collagen diseases. Moreover, the loci controlling tissue specificity of vasculitis were present. One of the positional candidate genes for vasculitis showed an allelic polymorphism in the coding region, thus possibly causing a qualitative difference in its function. As a result, a particular combination of polygenes with such an allelic polymorphism may thus play a critical role in leading the cascade reaction to develop vasculitis, and also a regular variation of systemic vasculitis. This is designated as the polygene network in systemic vasculitis. (J Jpn Coll Angiol, 2009, 49: 11-16).