The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis

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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Henoch-Schonlein Purpura in Children: The Role of Corticosteroids

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2019.538 ◽

2019 ◽

Vol 7 (11) ◽

pp. 1812-1814

Author(s):

Bella Kurnia

Keyword(s):

Abdominal Pain ◽

Renal Disease ◽

Systemic Vasculitis ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Henoch Schonlein Purpura ◽

Progression Of Renal Disease ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

BACKGROUND: Henoch- schonlein purpura (HSP) is an IgA- mediated systemic small vessel vasculitis. It is the most common form of systemic vasculitis in children.CASE REPORT: A 9 years old girl admitted to the hospital with chief complain of purplish red rash on both legs since approximately 1 week with painful knees and ankles that make the patient unable to walk. The patient was diagnosed with HSP and was treated with corticosteroid and analgesics. The patients only stayed for 2 nights at the hospital and discharged from the hospital with the ability to walk and experience no pain. CONCLUSION: The role of corticosteroids in the treatment of HSP is still controversial. But from various research, we can conclude that the role of corticosteroid in HSP is as a symptom reliever (reduce abdominal pain and arthritis), but does not slow the progression of renal disease.

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Atypical Bacterial Pathogens and Small-Vessel Leukocytoclastic Vasculitis of the Skin in Children: Systematic Literature Review

Pathogens ◽

10.3390/pathogens10010031 ◽

2021 ◽

Vol 10 (1) ◽

pp. 31

Author(s):

Céline Betti ◽

Pietro Camozzi ◽

Viola Gennaro ◽

Mario G. Bianchetti ◽

Martin Scoglio ◽

...

Keyword(s):

Mycoplasma Pneumoniae ◽

Legionella Pneumophila ◽

Leukocytoclastic Vasculitis ◽

Systemic Vasculitis ◽

Bacterial Pathogen ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Systemic Involvement ◽

Symptomatic Disease ◽

Atypical Pathogen

Leukocytoclastic small-vessel vasculitis of the skin (with or without systemic involvement) is often preceded by infections such as common cold, tonsillopharyngitis, or otitis media. Our purpose was to document pediatric (≤18 years) cases preceded by a symptomatic disease caused by an atypical bacterial pathogen. We performed a literature search following the Preferred Reporting of Systematic Reviews and Meta-Analyses guidelines. We retained 19 reports including 22 cases (13 females and 9 males, 1.0 to 17, median 6.3 years of age) associated with a Mycoplasma pneumoniae infection. We did not find any case linked to Chlamydophila pneumoniae, Chlamydophila psittaci, Coxiella burnetii, Francisella tularensis, or Legionella pneumophila. Patients with a systemic vasculitis (N = 14) and with a skin-limited (N = 8) vasculitis did not significantly differ with respect to gender and age. The time to recovery was ≤12 weeks in all patients with this information. In conclusion, a cutaneous small-vessel vasculitis with or without systemic involvement may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae. The clinical picture and the course of cases preceded by recognized triggers and by this atypical pathogen are indistinguishable.

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Vasculitis and Breast Cancer: Mind the Hint

Case Reports in Oncology ◽

10.1159/000514729 ◽

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.

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Systemic Vasculitis Syndromes

10.2310/fm.1050 ◽

2017 ◽

Author(s):

Alexandra Villa-Forte ◽

Brian F Mandell

Keyword(s):

Kawasaki Disease ◽

Blood Vessels ◽

Granulomatosis With Polyangiitis ◽

Systemic Vasculitis ◽

Plain Radiograph ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Saddle Nose ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Saddle Nose Deformity

Vasculitis is defined by histologic evidence of inflammation that involves the blood vessels. The diagnosis of a specific primary vasculitic disorder depends on the pattern of organ involvement, the histopathology, the size of affected blood vessels, and the exclusion of diseases that can cause “secondary” vasculitis. This review presents an approach to the patient suspected of having vasculitis, and goes on to discuss small vessel vasculitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa, Kawasaki disease, large vessel arteritis, and Behçet disease. Figures show classification of the systemic vasculitis syndromes, the relationships among the causes of small vessel (“hypersensitivity”) vasculitis, palpable purpura of the distal extremities, saddle nose deformity, the nodular infiltrates of the lung in granulomatosis with polyangiitis shown on plain radiograph as well as computed tomography, necrotizing scleritis, livedo reticularis, and angiograms of a patient with Takayasu arteritis. Tables list selected laboratory tests for patients with multisystem disease and possible vasculitis, practical comments on immunosuppressive therapies for vasculitis, features of vasculitis, diagnostic criteria for Kawasaki disease, and giant cell arteritis. This review contains 8 highly rendered figures, 5 tables, and 59 references.

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P186 Efficacy and safety of rituximab in the treatment of systemic vasculitis: experience at a single centre

Rheumatology ◽

10.1093/rheumatology/keaa111.181 ◽

2020 ◽

Vol 59 (Supplement_2) ◽

Cited By ~ 1

Author(s):

Carl Orr ◽

Ayna Verdiyeva ◽

Nadia Ahmad ◽

Hairulhadi Ariff ◽

Raashid Luqmani

Keyword(s):

Systemic Vasculitis ◽

Demographic Data ◽

Underlying Disease ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Chimeric Antibody ◽

Efficacy And Safety ◽

Electronic Patient Records ◽

Patient Demographic Data ◽

Age Profile

Abstract Background Rituximab is a monoclonal chimeric antibody targeting the mature B cell molecule CD20, expressed on the cell surface. It is licenced and widely used in the treatment of autoimmune diseases, including the ANCA associated vasculitides and rheumatoid arthritis. The efficacy and safety of rituximab in rare diseases requires ongoing appraisal. Methods We interrogated the records of 62 consecutive patients treated with rituximab for small vessel systemic vasculitis at our unit over the last 3 years, using a contemporaneously maintained central database, as well as individual electronic patient records. Results The patient demographic data, and main results are presented in Table 1. 31 (50%) of the patients treated with rituximab were female, and the indication for treatment in most cases was ANCA associated vasculitis (77.4%). After a mean follow up period of 2.04 years, the mean reduction in BVAS was 1.64 (3.83). 5 severe adverse events were recorded, defined as death or hospitalisation potentially related to treatment with rituximab. 3 patients died of respiratory sepsis, 1 experienced a wound infection, and 1 experienced an ischaemic colon. Four patients were noted to have low immunoglobulins. Conclusion Rituximab is an effective treatment for many patients with systemic, small vessel vasculitis. Understanding the safety profile of this drug in the context of our cohort is challenging, given the significant morbidity associated with the underlying disease, as well as the age profile and concomitant immunosuppressive medications. Continued surveillance regarding efficacy and safety of rituximab in the small vessel systemic vasculitides remains invaluable. Disclosures C. Orr None. A. Verdiyeva None. N. Ahmad None. H. Ariff None. R. Luqmani None.

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Perfect Timing: Mobile Brain/Body Imaging scaffolds the 4E-cognition research program

10.31234/osf.io/wru4j ◽

2020 ◽

Author(s):

Francisco J. Parada ◽

Alejandra Rossi

Keyword(s):

Real Life ◽

Body Imaging ◽

The Core ◽

4E Cognition ◽

Body Dynamics ◽

Brain And Behavior ◽

And Behavior ◽

A Current ◽

Made In

Recent technological advancements encompassed under the Mobile Brain/Body Imaging (MoBI) framework, have produced exciting new experimental results linking mind, brain, and behavior. The main goal of the MoBI approach is to model brain and body dynamics during every-day, natural, real-life situations. However, even though considerable advances have been made in both hardware and software, technical and analytical conditions are not yet optimal. The MoBI approach is based on attaching synchronized, small, and lightweight neurobehavioral sensors to and around participants during behaviorally-measured structured, semi-structured, and unstructured settings. These sensors have yet to become fully unobtrusive or transparent. Even though a considerable technical and analytical gap still exists, acquisition of brain/body dynamics during real-world situations as well as in virtual, modified, and/or extended laboratory settings has been -in many cases- successful. Nevertheless, even if the technical/analytical gap is breached, novel hypotheses, measures, and experimental paradigms are needed in order to tackle MoBI’s ultimate goal: to model and understand cognition, behavior, and experience as it emerges and unfolds unto and from the world. Such a goal is not completely novel or unique to the MoBI framework; it is at the core of a long-standing scientific and philosophical challenge. The present work starts by briefly reviewing the historical origins of complexity in order to identify three “waves and ripples of complexity” derived from naturalist accounts to the historical brain/body problem. We furthermore argue for a current 4th wave. Finally, we offer the reader what we consider to be the main objective for the MoBI+4E framework in its quest for understanding the functional role of brain/body/world couplings in the emergence of cognition.

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