Vasculitis

BACKGROUND: Vasculitides comprise a group of rare diseases which affect less than 5 in 10.000 individuals. Most types of vasculitis can become organ- and life-threatening and are characterized by chronicity, high morbidity and relapses, altogether resulting in significant morbidity and mortality. Previous studies have been either monocentric or mainly retrospective - studies with a prospective design mostly consisted of rather small cohorts of 100 to 200 patients. The aim of the Joint Vasculitis Registry in German-speaking countries (GeVas) is to record all patients who have been recently diagnosed with vasculitis or who have changed their treatment due to a relapse (inception cohort)...

OBJECTIVE: To investigate the incidence and risk factors for hypogammaglobulinemia and infectious complications associated with rituximab treatment in childhood-onset rheumatic diseases. METHODS: We performed a single-center retrospective study of patients (n = 85) treated at Boston Children's Hospital (BCH) from 2009 to 2019. Study subjects included patients (ages 6 to 24) who received rituximab for the treatment of a childhood-onset rheumatic disease. RESULTS: New-onset hypogammaglobulinemia developed in 23 (27...

CASE: An 81-year-old man from the northeastern United States presented with an acute Borrelia burgdorferi prosthetic joint infection (PJI) 15 years after undergoing a right total knee arthroplasty. He had no complications until his recent presentation. He was treated with irrigation and debridement with implant retention. Synovial fluid Lyme polymerase chain reaction and serological tests were positive. Postoperatively, he received oral doxycycline and at 6 weeks was asymptomatic. Four months later, he died of neuroborreliosis vasculitis, a complication of Lyme...

The physiologic role of the kidneys is dependent on the normal structure and functioning of the renal vasculature. Knowledge and understanding of the embryologic basis of the renal vasculature are necessary for the radiologist. Common anatomic variants involving the renal artery (supernumerary arteries and prehilar branching) and renal vein (supernumerary veins, delayed venous confluence, retroaortic or circumaortic vein) may affect procedures like renal transplantation, percutaneous biopsy, and aortic aneurysm repair...

INTRODUCTION: Henoch-Schönlein purpura nephritis (HSN) is defined as Henoch-Schönlein purpura with kidney involvement, including hematuria and/or proteinuria. The aim of this study was to evaluate the data of HSN patients who underwent renal biopsy, and compare the main clinical and laboratory parameters that may affect renal biopsy findings, treatment protocols, and short- and long-term outcome of those patients. METHODS: Biopsies performed in 72 HSN patients between January 2007 to January 2017 were retrospectively evaluated...

BACKGROUND: With increased use of immune checkpoint inhibitors (ICIs) among cancer patients, there is substantial interest in understanding clinical, economics outcomes and management of immune-related adverse events (irAEs). PATIENTS AND METHODS: A retrospective study was conducted using Premier Healthcare Database, a US national hospital discharge database, from March 1, 2015 through December 31, 2017. The database comprises more than 880 million inpatient and hospital-based outpatient encounters with more than 200 million unique patients reported by 966 hospitals...

Antineutrophil cytoplasmic autoantibody (ANCA) vasculitis has occasionally been associated with other systemic glomerulonephritis, such as anti-glomerular basement membrane disease. Here, we report the first clinical case of ANCA-associated crescentic glomerulonephritis with AL amyloidosis. An 81-years-old gentleman presented to the hospital with acute kidney injury (serum creatinine 4.7 mg/dL) on a background of chronic kidney disease and volume overload. Autoimmune serology was remarkable for p-ANCA and myeloperoxidase positivity...

Omalizumab is an anti-IgE monoclonal antibody used in severe allergic asthma. Herein, we describe a case of eosinophilic granulomatosis with polyangiitis, which manifested 3 months after initiation of omalizumab therapy, while maintenance corticosteroid therapy dose was unchanged.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides with pulmonary involvement include granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis, and can present with life-threatening pulmonary hemorrhage in up to 40% of patients. Mortality in those patients who require intubation and mechanical ventilation can reach 77%. Extracorporeal membrane oxygenation (ECMO) can be used to support these patients through definitive diagnosis and treatment, although minimizing the risk of ventilator-induced lung injury...

The relationship between migraine and vision is complex. Besides migraine aura status and persistent visual aura without infarction, recognized by International Headaches Classification 3, cases of persistent monocular alterations have been described in migraineurs. To discuss the role of migraine as a risk factor for persistent monocular visual loss. We present five new cases of persistent monocular visual loss in migraineurs, discuss the differential diagnosis and possible relationship with migraine. Five young healthy patients are reported (three women) with a history of migraine (four with visual aura), that developed persistent monocular visual defects, four during an episode of migraine with aura...

BACKGROUND: Currently, the range of copper (II) oxide nanoparticles' (CuO NPs) applications is expanding and the global production of CuO NPs is increasing. In this regard, the risk of exposure of the population to this nanomaterial increases. OBJECTIVE: The aim of the study is to investigate the patterns of bioaccumulation and toxic effects of CuO NPs after multiple oral exposures. METHODS: The particle size was determined by scanning electron microscopy and dynamic laser light scattering...

Systemic lupus erythematosus (SLE) is a connective tissue disease that is chronic, recurrent, and multisystem with unknown etiology. There is still no single biomarker that is pathognomonic for the disease. We know that platelets are the main part of hemostasis and thrombosis. We aimed to investigate whether there is a connection between MPV with SLE and inflammatory markers. We have included 39 female patients with SLE and 45 controls in this study. In both groups, erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP) levels, and MPV levels were investigated...

[Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis] (AAV) is an autoimmune disease characterized by systemic vascular inflammation. We present a case of a 76-year-old man who presented with shortness of breath, fatigue, and weakness. He was eventually diagnosed with hydralazine-induced ANCA-associated renal limited glomerulonephritis. The presentation of this case was unique for a few reasons; the patient showed an initial improvement in kidney function, was non-oliguric, and had no systemic signs of vasculitis...

In case of AAV with kidney involvement, physicians should explore anti-GBM antibodies and be aware of the possible sequential development of AAV, especially with MPO-ANCA, and anti-GBM glomerulonephritis. This sequential disease history is associated with a poor renal outcome, highlighting the need for urgent diagnosis and management.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of chronic multisystem autoimmune diseases with substantial mortality and morbidity and frequent relapses. The complexity of the disease condition and treatment-related adverse reactions as well as infections play important roles in the poor outcomes. Unfortunately, the subjective symptoms and objective indicators are not fully parallel, and manifestations between disease activity and treatment-related adverse reactions are often similar...

Behçet syndrome (BS) is a multisystem vasculitis with variable vessel involvement that shows significant heterogeneity among patients in terms of clinical manifestations and disease course. Treatment choice and response are both influenced by this heterogeneity. BS treatments' main goals are to quickly suppress inflammatory exacerbations and prevent relapses in order to protect organ functions and provide good quality of life. Besides the long-term experience with steroids and traditional immunosuppressives, biologic drugs, especially TNF inhibitors, have gained increasing importance in the treatment of BS over the years...

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BACKGROUND: Treatment for interstitial lung disease (ILD) patients with acute respiratory failure (ARF) is challenging, and literature to guide such treatment is scarce. The reported in-hospital mortality rates of ILD patients with ARF are high (62-66%). Cyclophosphamide is considered a second-line treatment in steroid-refractory ILD-associated ARF. The first aim of this study was to evaluate the in-hospital mortality in patients with ILD-associated ARF treated with cyclophosphamide. The second aim was to compare computed tomographic (CT) patterns and physiological and ventilator parameters between survivors and non-survivors...

OBJECTIVE: To investigate the significance of rheumatoid factor (RF) positivity in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients. METHODS: AAV patients were divided into groups as follows: RF (+)/ANCA (+) (n = 94), RF (-)/ANCA (+) (n = 80), RF (+)/ANCA (-) (n = 15), and RF (-)/ANCA (-) (n = 25). Their clinical data, organ involvement patterns, laboratory data, and patient outcomes were assessed. Kaplan-Meier analysis and propensity score matching (PSM) were performed to compare outcomes and analyse differences between the groups...

Aim: In this study, we aimed to investigate the possible role of endotrophin, a profibrotic byproduct of collagen VI, in the complex process of fibrosis development in the disease group with pulmonary fibrosis among interstitial lung diseases. Material and Method: When the patients' participation in the study were completed, smoking or alcohol drinking conditions, and family history were recorded. Their weights and heights were recorded and body mass index (BMI) was calculated...