Effect of Disease Activity, Glucocorticoid Exposure, and Rituximab on Body Composition During Induction Treatment of Antineutrophil Cytoplasmic Antibody –Associated Vasculitis
ConclusionOur findings suggest that changes in BMI, as well as glucocorticoid exposure, are independently associated with improvements in disease activity in AAV. Rituximab may also have effects on BMI independent of its impact on disease activity. (Source: Arthritis Care and Research)
Source: Arthritis Care and Research - June 27, 2017 Category: Rheumatology Authors: Zachary S. Wallace, Eli M. Miloslavsky, Matthew Cascino, Sebastian H. Unizony, Na Lu, Gary S. Hoffman, Cees G. M. Kallenberg, Carol A. Langford, Peter A. Merkel, Paul A. Monach, Philip Seo, Robert Spiera, E. William St.Clair, Ulrich Specks, Paul Brunetta, Tags: Vasculitis Source Type: research

Rituximab Induction and Maintenance Treatment in Patients with Scleritis and Granulomatosis with Polyangiitis (Wegener's).
CONCLUSIONS: RTX was effective as an induction and maintenance treatment in our small cohort of patients with GPA-associated scleritis. PMID: 28628344 [PubMed - as supplied by publisher] (Source: Ocular Immunology and Inflammation)
Source: Ocular Immunology and Inflammation - June 20, 2017 Category: Allergy & Immunology Tags: Ocul Immunol Inflamm Source Type: research

[Granulomatous reaction pattern of the skin : Interstitial granulomatous dermatitis - lymphoma - vasculitis].
Authors: Rose C, Holl-Ulrich K Abstract A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Differentiating between interstitial granulomatous dermatitis and the interstitial form of granuloma ...
Source: Der Hautarzt: Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete - June 15, 2017 Category: Dermatology Tags: Hautarzt Source Type: research

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody –Associated Vasculitis
ConclusionThis is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one‐half of this patient cohort experienced damage to various organ systems early in their disease course. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - June 9, 2017 Category: Rheumatology Authors: Kimberly A. Morishita, Lakshmi N. Moorthy, Joanna M. Lubieniecka, Marinka Twilt, Rae S. M. Yeung, Mary B. Toth, Susan Shenoi, Goran Ristic, Susan M. Nielsen, Raashid A. Luqmani, Suzanne C. Li, Tzielan Lee, Erica F. Lawson, Mikhail M. Kostik, Marisa Klein Tags: Full Length Source Type: research

Imaging diagnosis of orbital Wegener granulomatosis: A rare case report
Conclusion: WG is a serious, fatal disease. Early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients. (Source: Medicine)
Source: Medicine - June 1, 2017 Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research

Rituximab and Fc γ receptors in granulomatosis with polyangiitis (Wegener's): comment on the article by Cartin‐Ceba et al
(Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - May 31, 2017 Category: Rheumatology Authors: Wolfgang Merkt, Hanns ‐Martin Lorenz, Carsten Watzl Tags: Letters Source Type: research

ANCA-associated vasculitis with renal involvement.
Abstract Systemic vasculitis is a rare but severe group of diseases characterized by inflammation and necrosis of blood vessels. The size of the vessel affected varies among the different forms of vasculitis and there are three main subgroups: large, medium and small vessel vasculitis. Among small vessel vasculitis, the antineutrophil cytoplasmic antibody (ANCA)-associated forms are of particular importance. This subgroup includes: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and the form limited to the kidney. ANCA ar...
Source: Journal of Nephrology - May 30, 2017 Category: Urology & Nephrology Authors: Binda V, Moroni G, Messa P Tags: J Nephrol Source Type: research

A pragmatic approach to vasculitis in the gastrointestinal tract
Although vasculitis involving the gastrointestinal tract (GIT) is an uncommon occurrence, occasionally vasculitis can present as haemorrhagic infarction or ischaemia for which a length of bowel is removed. Invariably, the appropriate clinical history is not forthcoming, or vasculitis is not clinically suspected. The purpose of this overview is to provide the practising gastrointestinal (GI) pathologist with a framework to recognise and diagnose vasculitides within the GIT. The classification may be approached by aetiological agent or size of vessel involved; an international consensus group now favours the latter approach....
Source: Journal of Clinical Pathology - May 22, 2017 Category: Pathology Authors: Chetty, R., Serra, S. Tags: Editor's choice Review Source Type: research

Rare Manifestations of Wegener ’s Granulomatosis
  To the Editor: Wegener’s granulomatosis (WG) is a necrotizing granulomatous small-vessel vasculitis.1 The frequency of cardiac involvement in WG varies from 6 to 12 percent.2 (Source: Annals of Saudi Medicine)
Source: Annals of Saudi Medicine - May 12, 2017 Category: General Medicine Tags: ISSUE 3 Source Type: research

Granulomatosis with polyangiitis (Wegener granulomatosis): a proteinase-3 driven disease?
Publication date: Available online 8 May 2017 Source:Joint Bone Spine Author(s): Véronique Witko-Sarsat, Nathalie Thieblemont Granulomatosis with polyangiitis (GPA, Wegener granulomatosis) is a systemic autoimmune vasculitis that affects small arteries, arterioles, and capillaries, most notably in the kidneys and lungs. In this disease, proteinase 3 (PR3), produced by neutrophils, is targeted by antineutrophil cytoplasmic antibodies (ANCA). Recent work by our group has shown how PR3 impairs the resolution of inflammation and deregulates the immune system. Normally, the clearance of activated neutrophils triggers an an...
Source: Joint Bone Spine - May 9, 2017 Category: Orthopaedics Source Type: research

ANCA-associated vasculitis in childhood: recent advances
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener ’s), microscopic poly... (Source: Italian Journal of Pediatrics)
Source: Italian Journal of Pediatrics - May 5, 2017 Category: Pediatrics Authors: Marta Calatroni, Elena Oliva, Davide Gianfreda, Gina Gregorini, Marco Allinovi, Giuseppe A. Ramirez, Enrica P. Bozzolo, Sara Monti, Claudia Bracaglia, Giulia Marucci, Monica Bodria, Renato A. Sinico, Federico Pieruzzi, Gabriella Moroni, Serena Pastore, Gi Source Type: research

ANCA-associated vasculitis in childhood: recent advances
AbstractAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener ’s), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in c hildhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of...
Source: Italian Journal of Pediatrics - May 5, 2017 Category: Pediatrics Source Type: research

ANCA-associated vasculitis in childhood: recent advances
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener ’s), microscopic poly... (Source: Italian Journal of Pediatrics)
Source: Italian Journal of Pediatrics - May 5, 2017 Category: Pediatrics Authors: Marta Calatroni, Elena Oliva, Davide Gianfreda, Gina Gregorini, Marco Allinovi, Giuseppe A. Ramirez, Enrica P. Bozzolo, Sara Monti, Claudia Bracaglia, Giulia Marucci, Monica Bodria, Renato A. Sinico, Federico Pieruzzi, Gabriella Moroni, Serena Pastore, Gi Source Type: research

An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis.
We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA) and proteinase (PR-3) antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA). Our patient had ANCA-associated vasculitis (AAV) with features suggestive of both GPA and EGPA. She was treated with methylprednisolone and ...
Source: Saudi Journal of Kidney Diseases and Transplantation - May 1, 2017 Category: Urology & Nephrology Authors: Surendran S, Gundappa C, Gandhi A, Kurien AA, Fernando E Tags: Saudi J Kidney Dis Transpl Source Type: research

Nationwide Trends in Hospitalizations and In ‐Hospital Mortality in Granulomatosis With Polyangiitis (Wegener's)
ConclusionThe findings from these nationally representative, contemporary inpatient data indicate that the in‐hospital mortality of GPA has declined substantially over the past 2 decades, while the overall hospitalization rate for GPA increased slightly. Infection remains a common principal hospitalization diagnosis among GPA patients, including hospitalizations resulting in mortality. (Source: Arthritis Care and Research)
Source: Arthritis Care and Research - April 28, 2017 Category: Rheumatology Authors: Zachary S. Wallace, Na Lu, Eli Miloslavsky, Sebastian Unizony, John H. Stone, Hyon K. Choi Tags: BRIEF REPORT Source Type: research