Vasculitis

BACKGROUND: In chronic spontaneous urticaria (CSU), the guidelines recommend very limited diagnostic procedures during the routine workup, although additional investigations might be indicated in some CSU patients. For physicians treating CSU patients, it is often difficult to decide which diagnostic tests are useful. OBJECTIVE: To provide recommendations on what diagnostic tests should be performed on which CSU patient. METHODS: We performed an extensive literature search on the respective topics and identified relevant questions that should prompt diagnostic procedures based on the published evidence and expert consensus among all authors...

Hydralazine, a widely used therapy for hypertension and heart failure, can elicit autoimmune disease, including anti-neutrophil cytoplasmic antibody associated glomerulonephritis (ANCA-GN). We identified 80 cases of ANCA-GN complicating treatment with hydralazine, accounting for 4.3% (80/1858 biopsies) of ANCA-GN diagnosed between 2006 and 2019. Over three-fourths of patients were on hydralazine for at least one year, with mean daily dose of approximately 250 mg/day. ANCA testing revealed p-ANCA/myeloperoxidase-ANCA seropositivity in 98%, including 39% with dual p-ANCA/myeloperoxidase-ANCA and cANCA/anti-protinase 3-ANCA positivity, often accompanied by anti-nuclear antibody (89%), anti-histone antibody (98%), and hypocomplementemia (58%)...

BACKGROUND: Lymphopenia is commonly observed in autoimmune diseases, where it has been associated with disease activity or prognosis. However, in ANCA-associated vasculitis (AAV) only few, small-scale studies have been targeted to this issue. Research has not yet focused on AAV with renal involvement (AAV-RI) patients. Thus, the aim of this study was to analyze the association between lymphocyte counts and outcomes in a large cohort of AAV-RI patients. METHODS: We used the Maine-Anjou AAV registry that retrospectively gathers data on consecutive patients affected by AAV in four French Nephrology Centers, recorded since January 2000...

A 64-year-old male with a history of transverse myelitis presented to the hospital with a decreased level of consciousness of one day's duration. CT of the head revealed intracranial hemorrhage measuring 2 x 1.2 cm in the right temporal lobe and multiple small hemorrhages in the left hemisphere, suggestive of vasculitis. Initial vasculitis workup was negative for antinuclear antibody (ANA), complement component 3 (C3), and antineutrophil cytoplasmic antibodies: P-ANCA, C-ANCA. Syphilis, hepatitis B and C, West Nile virus antibody [immunoglobulin G (IgG) and immunoglobulin M (IgM)], herpes simplex virus (HSV) polymerase chain reaction (PCR), and HIV 1 and 2 were also negative...

No abstract text is available yet for this article.

Eosinophilic granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis with cardiac involvement in more than 60% of cases. Authors describe the case of a 48-year-old woman who presented with progressively worsening asthenia, dyspnoea and macular, non-painful, non-itchy cutaneous lesions. She had signs of congestion on clinical examination and a history of asthma and nasal polyps. Blood tests showed eosinophilia (11.2%), positive troponin I (9698 μg/L), elevated B-type natriuretic peptide (2047 pg/mL) and positive C reactive protein (6...

No abstract text is available yet for this article.

OBJECTIVES: Antineutrophil cyto plasmic antibody-associated vasculitis (AAV) is a fatal disease. Currently, predictors of mortality due to AAV are based on the distribution of organ involvement. The novel fibrosis index (NFI) is an index composed of laboratory results that reflect the degree of liver fibrosis. This study aimed to evaluate whether NFI can predict poor outcomes in patients with AAV without substantial liver disease. METHODS: A total of 210 patients with immunosuppressive drug-naïve AAV were retrospectively reviewed...

Adverse reactions to metal debris in relation to metal-on-metal hip arthroplasty has been heavily discussed in the literature. In contrast, few cases have been reported in the context of total knee arthroplasty. A 77-year-old woman presented with a painful total knee arthroplasty. At the time of revision surgery, intra-articular cream-coloured fluid and material was found in association with a well-fixed prosthesis. Synovial and capsular samples were obtained for histological assessment and a diagnosis of aseptic lymphocytic vasculitis associated lesion was confirmed...

A very rare inflammatory disease of CNS, CLIPPERS syndrome, was recently described and only a few sporadic cases are reported in the medical literature. Its etiology and pathogenesis are unknown, that together with the polymorphic and sometimes confounding neurological manifestations, and radiological findings represent a real diagnostic and therapeutic challenge for clinicians. Aim: To highlight the importance of clear and specific diagnostic assessment. Here we present the case of a 40-year-old male with a subacute lymphocytic midbrain inflammation accompanied by vasculitis...

In antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV), the two major target antigens of ANCA are proteinase 3 (PR3) and myeloperoxidase (MPO). Evidence is accumulating that there are distinct differences between patients with PR3-AAV and those with MPO-AAV. In the present study, the clinicopathological features and prognosis of patients with PR3-AAV and MPO-AAV from a single center in China were retrospectively analyzed. A total of 212 Chinese patients with AAV were recruited in the present study; 189/212 (89...

An 80-year-old man presented with new-onset pain in the shoulders and lower extremities and elevated serum inflammatory markers. A clinical diagnosis of polymyalgia rheumatica (PMR) was made, but there was a suboptimal response to glucocorticoid therapy, prompting further evaluation. 18 F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) revealed intense FDG uptake in the arteries of the bilateral lower extremities, head, and neck, but sparing the aorta, suggestive of an uncommon pattern of giant cell arteritis (GCA)...

A 73-year-old man presented with unsteadiness and general malaise and later had problems with cognition. This was initially diagnosed as benign paroxysmal positional vertigo, but he was later found to have giant cell arteritis. Neurologists and physicians should be aware that giant cell arteritis can present with encephalopathy rather than the more typical features of headache, jaw pain and visual disturbance.

A previously healthy 16-year-old adolescent was admitted to our hospital with fever, sore throat, diarrhoea, strawberry tongue, rashes on lower extremities, and pain in the neck, abdomen and joints. He was initially diagnosed with IgA vasculitis triggered by acute pharyngitis. Despite antibiotic treatment, he gradually developed multiorgan failure and cardiac shock. Cardiac catheterisation did not show any signs of coronary artery disease. Subsequently, he developed serious rhabdomyolysis and peripheral extremity necrosis, suggesting peripheral arteritis...

AIM: Diagnosing and monitoring vascular activity in giant cell arteritis (GCA) is difficult due to the paucity of specific serological biomarkers. We assessed the utility of 8 novel biomarkers in an inception cohort of newly suspected GCA patients. METHOD: Consecutive patients were enrolled between May 2016 and December 2017. Serum was collected within 72 hours of commencing corticosteroids and at 6 months. It was analyzed for levels of intra-cellular adhesion molecule 1, vascular endothelial growth factor (VEGF), pentraxin 3, von Willebrand factor and procalcitonin (5-plex R&D Systems multiplex assay) and interleukin (IL)6, IL12 and interferon-γ (high-sensitivity 3-plex ProcartaPlex multiplex assay)...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

We describe a case of a 48-year-old woman who presented with acute respiratory failure due to diffuse alveolar haemorrhage and acute renal failure due to pauci-immune glomerulonephritis consistent with a new diagnosis of microscopic polyangiitis (MPA). The patient had a recent SARS-CoV-2 infection 6 weeks before MPA diagnosis and had stopped immunosuppression for her rheumatoid arthritis (RA) at that time. The patient was treated with pulse intravenous steroids, plasma exchange therapy and rituximab, which induced remission of her illness...

BACKGROUND: Brolucizumab has recently been approved in Europe as a novel treatment for patients with neovascular age-related macular degeneration (nAMD). We report on early experiences with real-world outcomes of switch to brolucizumab therapy in previously anti-vascular endothelial growth factor (anti-VEGF)-treated patients. METHODS: Patients with recalcitrant nAMD were switched to brolucizumab therapy. Functional and structural parameters 4 weeks after first brolucizumab injection were evaluated including best-corrected visual acuity (BCVA (logMAR)), foveal centre point (FCP (µm)), central subfield retinal thickness (CSRT (µm)) and macular volume (mm³)...

INTRODUCTION: Propylthiouracil (PTU) is a synthetic antithyroid drug that can induce ANCA-associated vasculitis. OBSERVATION: A 27-year-old woman diagnosed with Graves' disease was on PTU for the past 10 years. She developed purpuric lesions of the legs and on the tip of the nose diagnosed as vasculitis. ANCAs were positive, with anti-MPO and anti-PR3 on blood ELISA. After discontinuation of PTU, she was able to fully recover. CONCLUSION: All synthetic antithyroid drugs can induce ANCA-associated vasculitis, more often PTU...