Acute Neurologic Manifestations of Systemic Immune-Mediated Diseases

2021 ◽  
Vol 41 (05) ◽  
pp. 541-553
Author(s):  
Kristin Galetta ◽  
Shamik Bhattacharyya
Keyword(s):  
Lupus Erythematosus ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Treatment Options ◽  
Systemic Autoimmune Diseases ◽  
Immunoglobulin G4 ◽  
Diagnostic Strategies ◽  
Immune Mediated ◽  
The Common ◽  
Eosinophilic Granulomatosis

AbstractSystemic autoimmune diseases can affect the peripheral and central nervous system. In this review, we outline the common inpatient consultations for patients with neurological symptoms from rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, sarcoidosis, immunoglobulin G4–related disease, Behçet's disease, giant cell arteritis, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis, polyarteritis nodosa, and ankylosing spondylitis. We discuss the symptoms, diagnostic strategies, and treatment options.

scholarly journals Natural Killer Cells in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - A Review of the Literature

2022 ◽  
Vol 12 ◽  
Author(s):  
Sina Fuchs ◽  
Andrea Scheffschick ◽  
Iva Gunnarsson ◽  
Hanna Brauner
Keyword(s):  
Nk Cells ◽  
Natural Killer ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Lymphoid Cells ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Systemic Autoimmune Diseases ◽  
Anca Associated Vasculitis ◽  
And Function ◽  
Eosinophilic Granulomatosis

Anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitis (AAV) is a group of systemic autoimmune diseases characterized by inflammation of small- and medium-sized vessels. The three main types of AAV are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). A growing number of studies focus on natural killer (NK) cells in AAV. NK cells are innate lymphoid cells with important roles in anti-viral and anti-tumor defense, but their roles in the pathogenesis of autoimmunity is less well established. In this review, we will present a summary of what is known about the number, phenotype and function of NK cells in patients with AAV. We review the literature on NK cells in the circulation of AAV patients, studies on tissue resident NK cells and how the treatment affects NK cells.

Connective tissue diseases

2020 ◽  
pp. 127-184
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Granulomatosis With Polyangiitis ◽  
Connective Tissue Diseases ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Assessment Tools ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Key Points ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis

This chapter covers the connective tissue diseases including systemic lupus erythematosus, Sjögren’s syndrome, scleroderma, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (including granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis (formerly known as Churg–Strauss syndrome), and microscopic polyangiitis), polyarteritis nodosa, and Behçet’s disease. For each example of a connective tissue disease it provides an overview of the condition and classification criteria, alongside the prognosis. Techniques and tricks for diagnosis, clinical features, assessment tools, and treatment are all covered. Key points of nursing care are described, including the nurse’s role in treatment with thalidomide and cyclophosphamide, and any particular organs that can be affected is detailed.

scholarly journals An Autopsy Case of a 5-Year-Old Child with Acute Pancreatitis Caused by Eosinophilic Granulomatosis with Polyangiitis-like Necrotizing Vasculitis

2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Haruna Yagi ◽  
Seishiro Takahashi ◽  
Tetsuo Kibe ◽  
Kenji Shirai ◽  
Isao Kosugi ◽  
...  
Keyword(s):  
Acute Pancreatitis ◽  
Gastric Ulcer ◽  
Lupus Erythematosus ◽  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Infiltration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Gi Bleeding ◽  
Necrotizing Vasculitis ◽  
Eosinophilic Granulomatosis ◽  
Histiocytic Infiltration

In children, acute pancreatitis has been reported in IgA vasculitis, Kawasaki disease, systemic lupus erythematosus-associated vasculitis, and juvenile dermatomyositis-associated vasculitis. However, its frequency in these vasculitides has been shown to be low. In other childhood-onset vasculitides, acute pancreatitis is seldom reported. The patient was a 5-year-old Japanese boy who suddenly presented with gastrointestinal (GI) bleeding. Therapy with antiulcer drugs successfully stopped bleeding, but subsequently, high fever, leukocytosis, and hypoxia appeared. He died 12 days after he presented with GI bleeding. An autopsy unexpectedly revealed that necrotizing vasculitis with marked eosinophilic and histiocytic infiltration of the pancreas led to acute pancreatitis, and gastric ulcer with eosinophilic infiltration was shown to be the origin of GI bleeding. In addition, eosinophilic infiltration was found in the small intestine, lungs, and bone marrow. Necrotizing vasculitis with eosinophilic and histiocytic infiltration of the pancreas, eosinophilic infiltration of the airway wall, and eosinophilic gastroenteritis with gastric ulcer were histologically confirmed, suggesting that the present case may be an early stage of eosinophilic granulomatosis with polyangiitis- (EGPA-) like vasculitis. To our knowledge, this might be the first reported case of EGPA-like vasculitis presenting with acute pancreatitis in a child.

scholarly journals Immunopathogenesis of ANCA-Associated Vasculitis

2020 ◽  
Vol 21 (19) ◽  
pp. 7319
Author(s):  
Andreas Kronbichler ◽  
Keum Hwa Lee ◽  
Sara Denicolo ◽  
Daeun Choi ◽  
Hyojeong Lee ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Autoimmune Disorder ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Proteinase 3 ◽  
Disease Biomarkers ◽  
Disease Phenotypes ◽  
Anca Associated Vasculitis ◽  
Clinical Presentations ◽  
Eosinophilic Granulomatosis

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small- and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.

scholarly journals AB0001 INTERFERON SIGNATURE IN CHILDREN WITH CHRONIC NON-BACTERIAL OSTEOMYELITIS AND IT’S DYNAMIC AFTER BISPHOSPHONATES TREATMENT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1036.2-1036
Author(s):  
M. Kostik ◽  
R. Raupov ◽  
R. Mulkidzhan ◽  
A. Kosmin ◽  
E. Suspitsin
Keyword(s):  
Lupus Erythematosus ◽  
Treatment Options ◽  
Basic Research ◽  
Cytokine Release ◽  
Interferon Signature ◽  
Immune Mediated ◽  
Main Adverse Event ◽  
Inflammatory Drugs ◽  
Anti Inflammatory ◽  
Basic Research Grant

Background:Chronic non-bacterial osteomyelitis (CNO) is an immune-mediated chronic inflammatory bone disease which predominantly affects children and adolescents. The pathogenesis of CNO related to imbalance between pro-inflammatory and anti-inflammatory cytokines. Interferon-I mediated pathway is associated with pathogenesis of different pediatric rheumatic diseases, such as juvenile systemic lupus erythematosus (jSLE), juvenile dermatomyositis (JDM), systemic onset of juvenile idiopathic arthritis (soJIA), and, most of all, with macrophage activation syndrome. The data on interferon-I- regulated pathway in CNO is absent. NSAIDs, non-biologic and biologic anti-inflammatory drugs and bisphosphonates (BF) are treatment options for patients with CNO. The main adverse event of BF is a flu-like syndrome probably caused by the excessive cytokine release stimulated by BF.Objectives:The aim of our study was to evaluate activity of Interferon-I mediated pathway in CNO patients and it’s dynamics after BF treatment.Methods:This prospective study included children with CNO requiring BF treatment (n=9), patients with soJIA (n=8), JDM (n=11) and jSLE (n=40) and healthy controls (HC, n=21). The activity of Interferon-I mediated pathway was assessed using interferon I score (IFN1 score). The score represented the median expression of 5 IFN1-regulated genes (IFI44L, IFI44, IFIT3, LY6E, MX1) measured by quantitative real-time PCR. Patients with CNO were treated with standard 3-day regimen (1 mg/kg/day). We measured interferon score before pamidronate (Day 0, n=9) and after (Day 3, n=7).Results:Median interferon score was 1.09 (0.96; 1.67) in CNO patients, 1.95 (1.3; 5.75) in soJIA, 7.6 (1.78; 29.0) in JDM and 16.9 (2.55; 40.3) in jSLE and 0.95 (0.82; 1.17) in HC (p=0.00001). Where were no difference in the IFN1 score between CNO and HC (p=0.222). In 6/7 CNO patients interferon score increased after pamidronate (p=0.015). The median interferon score after pamidronate increased and became 3.06 (0.87; 4,9, p=0.043); this may possibly explain the development of BF-related flu-like symptoms (cytokine release syndrome).Conclusion:While interferon I-regulated pathway is not directly associated with CNO pathogenesis, BF likely activates interferon-I-regulated pathway and thus could be a possible cause of flu-like syndrome.This work supported by the Russian Foundation for Basic Research (grant № 18-515-57001).Disclosure of Interests:None declared

Rheumatology

2016 ◽  
Keyword(s):  
Lupus Erythematosus ◽  
Basic Science ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Relapsing Polychondritis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Basic Training ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Chapter 19 covers the basic science and clinical topics relating to rheumatology which trainees are required to learn as part of their basic training and demonstrate in the MRCP. It covers basic science, the synovium, autoantibodies, osteoarthritis, rheumatoid arthritis, septic arthritis, crystal arthropathies, spondyloarthritides, psoriatic arthritis, low back pain, systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, Sjögren syndrome, giant cell arteritis/polymyalgia rheumatic, polyarteritis nodosa, Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis), granulomatosis with polyangiitis (Wegener), treating systemic vasculitis, relapsing polychondritis, and Behҫet disease.

Immune-mediated tubulointerstitial nephritis

2018 ◽  
Author(s):  
Liviu Segall ◽  
Adrian Covic
Keyword(s):  
Immune Complex ◽  
Lupus Erythematosus ◽  
Interstitial Fibrosis ◽  
Tubular Atrophy ◽  
Immunoglobulin G4 ◽  
Immune Mediated ◽  
Inflammatory Bowel ◽  
Complex Deposition ◽  
Stage Renal Disease ◽  
End Stage

Immune-mediated tubulointerstitial nephritides (TINs) are generally encountered in the context of systemic or extrarenal autoimmune diseases, such as sarcoidosis, Sjögren syndrome, systemic lupus erythematosus, inflammatory bowel disease, TIN and uveitis (TINU) syndrome, and immunoglobulin G4-related disease. The pathogenesis of these TINs is complex and more or less unclear; it usually involves leucocyte activation, autoantibodies, immune complex deposition, complement activation, and release of inflammatory cytokines and growth factors. Tubulointerstitial inflammation most commonly has a chronic pattern, although acute forms of TIN may also occur. Furthermore, inflammation may be granulomatous (as in sarcoidosis or Crohn’s disease) or non-granulomatous. Immunofluorescence staining can sometimes reveal immune complex deposits and even antitubular basement membrane autoantibodies. Systemic immunosuppressive therapies are almost always required to prevent progression to irreversible interstitial fibrosis, tubular atrophy, and end-stage renal disease.

scholarly journals Palisaded Neutrophilic and Granulomatous Dermatitis in a Patient with Granulomatosis with Polyangiitis

2020 ◽  
Vol 12 (1) ◽  
pp. 52-56
Author(s):  
Mai Akagawa ◽  
Yuki Hattori ◽  
Yoko Mizutani ◽  
En Shu ◽  
Tatsuhiko Miyazaki ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Elevated Serum ◽  
Systemic Steroid ◽  
M2 Macrophages ◽  
Normal Range ◽  
Systemic Steroid Therapy ◽  
Scd163 Level ◽  
Granulomatous Dermatitis

Palisaded neutrophilic and granulomatous dermatitis (PNGD) shows various clinical features and is histologically characterized by palisaded granulomas surrounding degenerated collagen. PNGD is known to be associated with a variety of systemic conditions such as rheumatoid arthritis and systemic lupus erythematosus. Furthermore, PNGD has been reported to be associated with antineutrophilic cytoplasmic antibody-associated vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis. Here, we report a case of PNGD associated with GPA, which showed the infiltration of CD163-positive M2 macrophages in the skin lesion with elevated serum level of soluble CD163 (sCD163). The serum sCD163 level was reduced to normal range after systemic steroid therapy. Thus, M2 macrophages may play a role in the pathomechanisms of PNGD associated with GPA.

scholarly journals Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2318
Author(s):  
Stefano Palmucci ◽  
Corrado Inì ◽  
Salvatore Cosentino ◽  
Luigi Fanzone ◽  
Stefano Di Pietro ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Case Series ◽  
Interstitial Lung Diseases ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Immune Mediated ◽  
Antineutrophil Cytoplasmic Autoantibody ◽  
Interstitial Disease ◽  
Eosinophilic Granulomatosis

Vasculitides represent a heterogeneous group of immune-mediated disorders, characterized by a systemic inflammatory destructive process of the blood vessels resulting either in ischemia or hemorrhage. The organ involved and vessel size influence the pattern of presentation of the pathology. The lung is commonly involved in systemic vasculitides, with heterogeneous clinical, radiological, and histopathological presentations. Primary vasculitides most commonly associated with lung parenchymal involvement include small-vessel antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides, such as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Several studies have reported cases of interstitial lung diseases (ILDs) associated with systemic vasculitis, particularly those positive for ANCA associated vasculitis/vasculitidis: AAV. We have selected from our case series different radiological features of pulmonary vasculitis (i.e., solitary or multiple nodules, cavitary lesions, nodules with centrilobular or peribronchial distribution, airspace consolidations, “crazy paving” appearance, interstitial disease), including cases with interstitial lung alterations. Therefore, the aim of this review is to describe the typical clinical manifestations of vasculitides and their main radiologic features (especially AAV).

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