Involvement In Systemic Vasculitis Research Articles

Primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (CNS) is an uncommon inflammatory disorder, with highly variable clinical presentation. It needs to be differentiated from several mimickers, such as CNS involvement in systemic vasculitides, connective tissue disorders, infectious disease, and leukodystrophy as well as neoplastic diseases. The diagnosis requires a combination of clinical and laboratory investigations, multimodal imaging, and histopathological examination, which should be available for confirmation. In the present paper, the histopathological features of primary angiitis of the CNS are described and highlighted to help pathologists avoid misdiagnosis of a treatable acquired disease.

Abdominal involvement as a primary manifestation of systemic or isolated gastrointestinal vasculitis

Systemic vasculitides can cause a wide variety of gastrointestinal manifestations (GI) ranging from mild and frequently nonspecific abdominal pains to potentially life-threatening bowel perforations. Vascular involvement in systemic vasculitides can affect any GI blood vessel, most commonly mesenteric, hepatic, or splenic arteries. Inflammatory changes affecting different layers of arterial vessel walls can lead to aneurysmatic dilatation or blood vessel occlusion with subsequent organ ischemia leading to mucosal ulcerations, GI bleeding, perforations, or bowel obstruction. While the presence of extraintestinal manifestations may aid in diagnosis, delays in making appropriate diagnoses and rapid initiation of glucocorticoid and immunosuppressive treatment can have detrimental consequences. Awareness of isolated gastrointestinal vasculitis is of particular importance as it frequently remains undiagnosed until end-stage organ damage becomes apparent. Vasculitis mimics such as vascular Ehlers-Danlos syndrome or fibromuscular dysplasia add another lay of complexity in approaching patients with suspected GI vasculitis and should always be carefully considered.

Diffused coronary involvement in Takayasu arteritis with concomitant malignancy

Large vessel vasculitis (LVV) is composed of conditions in which inflammation of blood vessel walls affects mainly large arteries, such as the aorta and its main branches, and in some cases the coronary arteries. Coronary artery involvement in systemic vasculitis is associated with significant morbidity and mortality. We present a case of a young patient diagnosed with extensive coronary disease diagnosed as Takayasu arteritis, when whom a concomitant diagnosis of Hodgkin's lymphoma was made. The literature review revealed ten cases of malignancies associated with Takayasu arteritis. We discuss the complexity of the management of concurrent hematological malignancy with TAK and extensive coronary arteritis. This complicated and cross-disciplinary case also represents the pivotal importance of multi-disciplinary team decision in order to achieve the best clinical outcome of both disorders.

Cerebral involvement in systemic vasculitides

Cerebral vasculitis is a rare disorder but plays a major role in the differential diagnosis of stroke, encephalopathy and headache. This guideline was developed in order to support clinicians in the diagnosis and treatment of cerebral manifestations of systemic vasculitides and rheumatic diseases. It is based on a medline research and was developed in a modified Delphi process and approved by the involved societies.This article is an abridged and translated version of the guideline published in DGNeurologie: Berlit, P. & Krämer, M. DGNeurologie (2018) 1: 17. https://doi.org/10.1007/s42451-018-0001-y

Cardiovascular magnetic resonance in the diagnosis and management of cardiac and vascular involvement in the systemic vasculitides.

Cardiac manifestations in systemic vasculitides, either primary or secondary due to infection, malignancy or autoimmune rheumatic diseases may be life-threatening. Cardiovascular (CVD) magnetic resonance (CMR) has been recently proposed as an ideal noninvasive tool to evaluate systemic vasculitides. In the present article, we present an overview of CMR in the diagnosis and follow-up of cardiac involvement in systemic vasculitides. CMR is a noninvasive, nonradiating modality, capable to assess cardiac function, perfusion and tissue characterization that can be of great diagnostic value in both primary and secondary systemic vasculitides. It has been already documented that CMR is superior to other imaging modalities, because it has great versatility and higher spatial resolution that allows the detection of early CVD phenomena occurring during systemic vasculitides. Magnetic resonance angiography and oedema-fibrosis imaging detect early CVD involvement such as acute and/or chronic inflammation, coronary macro-micro-circulation abnormalities and/or small vessel vasculitis. CMR due to its great versatility gives valuable information about cardiac function, perfusion, type of fibrosis and vascular integrity that may significantly contribute to treatment decisions beyond vascular scores, other disease activity or severity indices or the acute phase response.

Pulmonary Involvement in Systemic Vasculitis.

The purpose of this study is to describe the most relevant advances concerning lung involvement in the ANCA-associated vasculitides (excluding eosinophilic granulomatosis with polyangiitis which may have different disease mechanisms). Focus is on pathophysiology, recent important imagenological procedures, treatment, and outcome. Emerging information exists on potential newly investigated diagnostic procedures (v.g. transbronchial cryobiopsies), detailed tomographic abnormalities, the potential favorable role of rituximab and the still uncertain one of plasma exchange in the treatment, and the increasing description of interstitial lung disease. Survival is reduced in case of both, diffuse alveolar hemorrhage and diffuse parenchymal disease. There is the need to expand the knowledge concerning better long-term treatment options with specific regimes, and to incorporate other measures regarding integral treatment in patients afflicted with lung involvement these maladies, as the outcome seems adverse in this scenario.

Systemic vasculitis and the lung.

The purpose of this review is to provide a critical analysis of the recent literature on this topic, with particular focus on the most relevant studies published over the last year. Many studies are published every year on the diagnosis, pathogenesis and treatment of pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). The main subjects covered by this article are the pathogenesis, diagnosis and clinical aspects of lung involvement in ANCA-associated vasculitis and non-ANCA-associated vasculitis. Lung involvement is a common feature in systemic vasculitis. The lungs are one of the most frequently involved organs in systemic vasculitis. In order to provide an update on the recent advances in the pathogenesis, clinical features and novel treatments of lung involvement in systemic vasculitis, a systematic MedLine search has been performed.Most of the data analyzed have confirmed that lung involvement seems to develop more frequently in patients with myeloperoxidase-ANCA-positive AAV, mainly in those with a diagnosis of microscopic polyangiitis (MPA), compared with patients with proteinase 3 ANCA-positive AAV. Moreover, among non-ANCA-associated vasculitis lung involvement may represent a worrying complication of the disease, mainly when associated with vascular involvement.

Sinonasal Involvement in Systemic Vasculitides and Cocaine-Induced Midline Destructive Lesions: Diagnostic Controversies

Multiple systemic diseases produce various clinical manifestations in the sinonasal area. They usually appear as difficult-to-diagnose disease processes with slow, atypical clinical courses. The aim of this study was to evaluate the sinonasal manifestations of systemic vasculitides, highlighting key points for diagnosis and differential diagnosis with other pathological entities, especially cocaine-induced midline destructive lesions (CIMDL). A retrospective study was performed of 10 patients treated in our hospital during the last 5 years with an initial diagnosis of systemic vasculitides with sinonasal involvement: eight patients with granulomatosis with polyangiitis (GPA; new nomenclature for Wegener granulomatosis) and two patients with Churg-Strauss syndrome (CSS). The study variables were clinical presentation, nasal endoscopy results, maxillofacial scan results, nasal biopsy results, erythrocyte sedimentation rate, and autoimmune antibody levels. The definitive diagnosis was GPA in six (60%) patients, CSS in two (20%) patients, and CIMDL in two (20%) patients. Nasal symptoms were similar in all patients, but nasal polyps were present in only one patient with CSS. Systemic manifestations were absent in patients with CIMDL. Likewise, peripheral eosinophilia was observed only in the two patients with CSS. Specific positive biopsy specimens were obtained in six patients (all six patients with GPA, one with CSS, and one with CIMDL). Antineutrophil cytoplasmic antibodies (ANCA) were positive in all patients with GPA (proteinase 3 antigen in five patients and myeloperoxidase in one patient), and perinuclear ANCA was positive in one patient with CIMDL; however, this patient showed an undefined pattern. Finally, the response to treatment was adequate in all patients excluding those with CIMDL. GPA and CIMDL syndromes pose a difficult differential diagnosis because they have common clinical, serological, and histological presentations. Negative histological results do not exclude the diagnosis of sinonasal vasculitides. The absence of systemic manifestations and the lack of response to treatment will lead to the confirmation of CIMDL syndrome in a cocaine user. Otolaryngologists play an important role in the early and differential diagnosis of these diseases.

Coronary Angiitis and Cardiac Arrest in Antineutrophil Cytoplasmic-Antibody Associated Systemic Vasculitis

A 58-year-old man was admitted with a 6-week history of fever, lethargy, arthralgia, and dyspnea on exertion. He had no prior illness or cardiovascular risk factors, and with the exception of a pyrexia, his physical examination was unremarkable. Investigations were performed, including blood and urine cultures and a connective tissue screen. He was subsequently discharged with a view to an early outpatient review when he collapsed at home with cardiac arrest. The initial rhythm was ventricular fibrillation, which was successfully cardioverted. His postarrest electrocardiogram revealed transient right bundle-branch block and his plasma troponin I concentration was raised at 8.5 μg/L. His renal function was normal, but markers of …

Video capsule endoscopy and intestinal involvement in systemic vasculitis

We describe a case of a 26-years-old woman whom we have followed since the age of six because of a systemic chronic leucocytoclastic vasculitis of the small and medium vessels. Her disease started with skin involvement characterised by diffuse purpuric lesions, followed by neurological episodes characterised by aphasia, headaches and conscience disorders due to acute cerebral ischaemic events.

Cerebral vasculitis

This review focuses on isolated CNS-angiitis and cerebral involvement in systemic vasculitides and collagen vascular diseases. CNS-vasculitis presents with headache, multifocal symptoms, and encephalopathy, respectively. Clinical diagnosis is based on MRI, angiography and both CSF and serum examinations. But neither laboratory nor neuroradiological findings are specific - in all vasculitides with the exception of the Behçet-syndrome a biopsy is necessary before immunosuppressive treatment is started. Therapy of choice is the combination of corticosteroids and cyclophosphamide.

Symposium 18A: Renal involvement in systemic vasculitis

Symposium 18A: Renal involvement in systemic vasculitis

Manifestations ophtalmologiques des vascularites systémiques : analyse de six observations et revue de la littérature

Purpose. – Study of characteristics of ocular involvement in systemic vasculitis. Methods. – We describe six cases of systemic vasculitis with ocular involvement observed between 1992 and 2000. These cases are compared with those reported in the literature. Results. – Our patients suffered from Wegener’s granulomatosis (four cases), periarteritis nodosa and Churg-Strauss syndrome. Ocular manifestations were conjunctivitis, scleritis, orbital pseudotumor, optic neuritis and extraocular muscle palsy. These manifestations are similar to those reported in the literature. Their treatment requires steroids and immunosuppressive drugs. In one of our cases, intravenous immunoglobulins were effective in controlling an optic neuritis. Conclusion. – Ocular involvement in systemic vasculitis may concern any orbital structure. It usually occurs during the course of vasculitis but may be one of its first manifestations. It requires an appropriate treatment to prevent ophthalmic complications and especially blindness.

Renal involvement in systemic vasculitis.

Renal involvement in systemic vasculitis.

Renal Involvement in Systemic Vasculitis

Renal Involvement in Systemic Vasculitis