scholarly journals Digital infarct and mononeuritis in a middle aged female: always suspect Antineutrophil cytoplasmic antibodies associated vasculitis

2020 ◽  
Vol 7 (4) ◽  
pp. 707
Author(s):  
Avirup Majumdar ◽  
Virendra Atam ◽  
Saurabh Pandey ◽  
Prashant Singh ◽  
Himanshu Chauhan
Keyword(s):  
Antineutrophil Cytoplasmic Antibody ◽  
Organ Damage ◽  
Small Vessel ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
Multisystem Disease ◽  
Cutaneous Manifestations ◽  
Similar Treatment ◽  
Process Testing ◽  
Anca Associated Vasculitis

Vasculitis is a process caused by inflammation of blood vessel walls and results in a variety of disorders.  Small-vessel vasculitis (vasculitis involving arteries, venules and capillaries) should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Testing for Antineutrophil cytoplasmic antibody (ANCA) is the basis of classification of small vessel vasculitis into ANCA associated and non - ANCA associated vasculitis. Apart from cutaneous manifestations like palpable purpura and vasculitic urticaria, digital gangrene in a patient with evidence of mononeuritis multiplex is highly suggestive of ANCA associated vasculitis (AAV). Clinically most of these vasculitides have overlapping clinical presentations and similar treatment. Early diagnosis and rapid initiation of treatment of AAV is recommended rather than ordering for definitive tests (e.g. histopathology or angiograms) since delay in treatment can result in serious end organ damage (pulmonary or renal).

scholarly journals Vasculitis and Breast Cancer: Mind the Hint

2021 ◽  
pp. 550-560
Author(s):  
Miguel Esperança-Martins ◽  
Vasco Evangelista ◽  
Salomão Fernandes ◽  
Raquel Almeida
Keyword(s):  
Breast Cancer ◽  
Ductal Carcinoma ◽  
Small Vessel ◽  
Alveolar Haemorrhage ◽  
Small Vessel Vasculitis ◽  
Diffuse Alveolar Haemorrhage ◽  
Anca Associated Vasculitis ◽  
First Case ◽  
Circulating Antibodies ◽  
Perinuclear Anca

Diffuse alveolar haemorrhage related to an anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis is an extremely rare form of presentation of breast cancer. Here we report the case of a 77-year-old woman with a histological diagnosis of a papillary ductal carcinoma of the breast presenting with a diffuse alveolar haemorrhage secondary to a perinuclear ANCA-associated vasculitis. To our knowledge, this is the first case ever reported of a diffuse alveolar haemorrhage related to an ANCA-associated small vessel vasculitis as a form of presentation of breast cancer. The therapeutic approach of this paraneoplastic vasculitis included the use of corticosteroids and plasmapheresis, a very useful technique to remove endothelial aggressors (circulating antibodies) as a strategy to earn time for a proper therapeutic decision specifically directed for disease modification, but that can also be associated with several severe adverse effects, which are illustrated in our case.

Small vessel vasculitis

2020 ◽  
pp. 4573-4579
Author(s):  
Richard A. Watts
Keyword(s):  
Antineutrophil Cytoplasmic Antibody ◽  
Consensus Conference ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Iga Vasculitis ◽  
Small Vessels ◽  
Chapel Hill ◽  
Familial Association ◽  
Chapel Hill Consensus Conference ◽  
Inflammatory Drugs

Small vessel vasculitis is vasculitis affecting predominately small intraparenchymal arteries, arterioles, capillaries, and venules. There are two main types: antineutrophil cytoplasmic antibody associated and immune complex mediated. The ANCA associated vasculitides are discussed in chapter 19.3 IgA vasculitis (IgAV) was formerly known as Henoch Schönlein purpura. The revised nomenclature reflects the importance of IgA vasculitis in pathogenesis. The Chapel Hill Consensus Conference defined IgA vasculitis as ‘vasculitis with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles)’. IgA vasculitis often involves skin and gut, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur. Its aetiology is unknown, but it frequently occurs after an infection several days to weeks before. The most frequently isolated organism is beta-haemolytic streptococcus. Drugs such as a penicillin, ampicillin, erythromycin, and non-steroidal anti-inflammatory drugs have been reported as precipitating agents. There is an association with HLA-DRB1*01 in Caucasians and there appears to be a familial association.

scholarly journals Antineutrophil Cytoplasmic Antibody–associated Glomerulonephritis Complicated by Pneumatosis Intestinalis

2015 ◽  
Vol 8 ◽  
pp. CCRep.S26155 ◽  
Author(s):  
Saki Nakagawa ◽  
Tetsu Akimoto ◽  
Shin-ichi Takeda ◽  
Mari Okada ◽  
Atsushi Miki ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Bowel Wall ◽  
Pneumatosis Intestinalis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Current Case ◽  
Anca Associated Vasculitis ◽  
Characteristic Imaging

Pneumatosis intestinalis is a characteristic imaging phenomenon indicating the presence of gas in the bowel wall. The link between pneumatosis intestinalis and various kinds of autoimmune diseases has been reported anecdotally, while information regarding the cases with antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis complicated by concurrent pneumatosis intestinalis is lacking. In this report, we describe our serendipitous experience with one such case of pneumatosis intestinalis in a patient with ANCA-associated glomerulonephritis. We also discuss several therapeutic concerns that arose in the current case, which had an impact on the pathogenesis of the disease.

scholarly journals Increase of Antimyeloperoxidase Antineutrophil Cytoplasmic Antibody (ANCA) in Patients with Renal ANCA-associated Vasculitis: Association with Risk to Relapse

2015 ◽  
Vol 42 (10) ◽  
pp. 1853-1860 ◽  
Author(s):  
Makoto Yamaguchi ◽  
Masahiko Ando ◽  
Sawako Kato ◽  
Takayuki Katsuno ◽  
Noritoshi Kato ◽  
...  
Keyword(s):  
Regression Models ◽  
Cox Regression ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Time Dependent ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Anca Associated Vasculitis ◽  
Level Increase ◽  
Diagnostic Values ◽  
Renal Vasculitis ◽  
Risk Of Disease

Objective.The diagnostic values of antiproteinase 3 and antimyeloperoxidase tests using antineutrophil cytoplasmic antibodies (ANCA) are well established. Our study determined whether an increase in ANCA level was a predictor of disease flareup.Methods.Our study included 126 patients with ANCA-associated renal vasculitis treated at 9 nephrology centers in Japan. The relationship between increased ANCA levels and relapse was assessed using time-dependent multivariate Cox regression models adjusted for clinically relevant factors. The outcome of interest was the time from remission to first relapse.Results.During the observation period [median 41 mos, interquartile range (IQR) 23–66 mos], 118 patients (95.8%) achieved remission at least once. After achieving remission, 34 patients relapsed (21.7%). Time-dependent multivariate Cox regression models revealed that lung involvement (adjusted HR 2.29, 95% CI 1.13–4.65, p = 0.022) and increased ANCA levels (adjusted HR 17.4, 95% CI 8.42–36.0, p < 0.001) were significantly associated with relapse. The median time from ANCA level increase to relapse was 0.6 months (IQR 0–2.1 mos).Conclusion.In our study, an increase in ANCA level during remission was associated with a risk of disease relapse. A rise in ANCA level may be useful for guiding treatment decisions in appropriate subsets of patients with ANCA-associated vasculitis.

scholarly journals Development and initial evaluation of a measure of self-management for adults with antineutrophil cytoplasmic antibody-associated small-vessel vasculitis

2007 ◽  
Vol 57 (7) ◽  
pp. 1296-1302 ◽  
Author(s):  
Carolyn T. Thorpe ◽  
Robert F. Devellis ◽  
Megan A. Lewis ◽  
Susan J. Blalock ◽  
Susan L. Hogan ◽  
...  
Keyword(s):  
Antineutrophil Cytoplasmic Antibody ◽  
Initial Evaluation ◽  
Small Vessel ◽  
Self Management ◽  
Small Vessel Vasculitis

scholarly journals Case Report: Systemic Small-Vessel Vasculitis in an Adolescent With Active Ulcerative Colitis

2021 ◽  
Vol 9 ◽  
Author(s):  
Marleen Bouhuys ◽  
Wineke Armbrust ◽  
Patrick F. van Rheenen
Keyword(s):  
Ulcerative Colitis ◽  
Abdominal Pain ◽  
Gastrointestinal Tract ◽  
Leukocytoclastic Vasculitis ◽  
Foot Drop ◽  
Small Vessel ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
Induction Treatment ◽  
High Dose

Introduction: Small-vessel vasculitis (SVV) is a rare immunological disease that affects arterioles, capillaries and venules. It causes purpura, but can also manifest in other organs, including the gastrointestinal tract. SVV and inflammatory bowel disease (IBD) co-occur more frequently than would be expected by chance.Case description: A 16-year-old girl, who had been diagnosed with ulcerative colitis (UC) 2 years earlier at a general hospital, developed purpura, progressive abdominal pain with frequent bloody diarrhea and frontotemporal headache and swelling while on azathioprine and mesalamine maintenance therapy. Serology was positive for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) without antiprotease- or myeloperoixidase antibodies. Endoscopy revealed active left-sided UC and atypical ulcerations in the ascending colon. Biopsies of these ulcerations and of affected skin revealed leukocytoclastic vasculitis. Initially this was interpreted as an extraintestinal manifestation of UC that would subside when remission was induced, consequently infliximab was started. Over the next 3 weeks she developed severe burning pain in her right lower leg that progressed to a foot drop with numbness and the purpura progressed to bullous lesions. The diagnosis was adjusted to ANCA-associated vasculitis with involvement of skin, bowel and peripheral nerves. Infliximab was discontinued and induction treatment with high-dose prednisolone and cyclophosphamide was given until remission of SVV and UC was achieved. Subsequently, infliximab induction and maintenance was re-introduced in combination with methotrexate. Remission has been maintained successfully for over 2 years now. The foot drop only partly resolved and necessitated the use of an orthosis.Conclusion: Pediatric patients with IBD who present with purpuric skin lesions and abdominal pain should be evaluated for systemic involvement of SVV, which includes endoscopic evaluation of the gastrointestinal tract. We discuss a practical approach to the diagnosis, evaluation and management of systemic SVV with a focus on prompt recognition and early aggressive therapy to improve outcome.

scholarly journals The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis

Antibodies ◽  
2019 ◽  
Vol 8 (2) ◽  
pp. 31 ◽  
Author(s):  
Elena Csernok
Keyword(s):  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Urticarial Vasculitis ◽  
International Consensus ◽  
Anca Associated Vasculitis ◽  
Distinct Disease ◽  
A Current ◽  
Made In

Considerable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the diagnosis, prognosis, and monitoring of small-vessel vasculitis. ANCA-directed proteinase 3 (PR3-) or myeloperoxidase (MPO-) are sensitive and specific serologic markers for ANCA-associated vasculitides (AAV), anti-GBM antibodies are highly specific for the patients with anti-GBM antibody disease (formerly Goodpasture’s syndrome), and autoantibodies to C1q are characteristic of hypocomlementemic urticarial vasculitis syndrome (HUVS; anti-C1q vasculitis). The results of a current EUVAS study have led to changes in the established strategy for the ANCA testing in small-vessel vasculitis. The revised 2017 international consensus recommendations for ANCA detection support the primary use PR3- and MPO-ANCA immunoassays without the categorical need for additional indirect immunofluorescence (IIF). Interestingly, the presence of PR3- and MPO-ANCA have led to the differentiation of distinct disease phenotype of AAV: PR3-ANCA-associated vasculitis (PR3-AAV), MPO-ANCA-associated vasculitis (MPO-AAV), and ANCA-negative vasculitis. Further studies on the role of these autoantibodies are required to better categorize and manage appropriately the patients with small-vessel vasculitis and to develop more targeted therapy.

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