61,822 research outputs found
Implications of Antiphospholipid and Antineutrophilic Cytoplasmic Antibodies in the Context of Postinfectious Glomerulonephritis.
While antineutrophil cytoplasmic antibody (ANCA) positivity has been documented in some patients with postinfectious glomerulonephritis (PIGN) and is associated with more severe disease, antiphospholipid antibodies (APA) are not known to be a common occurrence. We describe a child with severe acute kidney injury who was noted to have prolonged positivity of both ANCA and APA; a renal biopsy showed noncrescentic immune complex mediated glomerulonephritis with subepithelial deposits compatible with PIGN. He recovered without maintenance immunosuppressive therapy and at last follow-up had normal renal function. We discuss the cooccurrence and implications of ANCA and APA in children with PIGN
Familial vasculitides: granulomatosis with polyangitis and microscopic polyangitis in two brothers with differing anti-neutrophil cytoplasm antibody specificity
Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a group of rare autoimmune diseases. Although the aetiology of AAV is uncertain, it is likely that genetic and environmental factors contribute. We report the unusual case of two brothers presenting with AAV with differing clinical pictures and differing ANCA specificity. There is a recently identified difference in genetic risk factors associated with ANCA specificity, making it surprising that first-degree relatives develop AAV with differing clinical and serological features. Our report illustrates the complex aetiology of AAV and suggests that further research on the interaction of genetic and environmental factors is needed
Pathologic manifestations of levamisole-adulterated cocaine exposure.
UnlabelledRheumatic manifestations of cocaine have been well described, but more recently, a dramatic increase in the levamisole-adulterated cocaine supply in the United States has disclosed unique pathologic consequences that are distinct from pure cocaine use. Most notably, patients show skin lesions and renal dysfunction in the setting of extremely high perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA). Unexpectedly, antibodies to myeloperoxidase, the typical target of p-ANCA, are relatively low if at all present. This discrepancy is due to the fact that p-ANCA seen in association with levamisole-adulterated cocaine exposure is often directed against atypical p-ANCA-associated antigens within the neutrophil granules such as human neutrophil elastase, lactoferrin, and cathepsin G. Biopsies of the skin lesions reveal leukocytoclastic vasculitis often involving both superficial and deep dermal vessels. Renal injury most typically manifests as crescentic and necrotizing pauci-immune glomerulonephritis. In this review, the manifestations of levamisole-adulterated cocaine-induced vasculitis are discussed with an emphasis on the typical histomorphologic findings seen on biopsy.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1764738711370019
Nasal manifestations in granulomatosis with polyangiitis: a case report and review of the literature
Granulomatosis with polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides of medium and small arteries, characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract with coexist- ing glomerulonephritis. We report a case of GPA in a patient presenting with a six-month history of spontaneous epistaxis, nasal obstruction and frontal headache. Nasal endoscopy showed a large nasal septum perforation and an anterior translucid mass in the right nasal fossa. Findings were confirmed by computed tomography (CT) scan with contrast. The patient underwent func- tional transnasal endoscopic removal of the mass; histological examination showed tissue features suggestive of GPA; dosage of c-ANCA e p-ANCA antibodies confirmed GPA diagnosis. Nasal septum perforation has long been recognized as a feature of GPA, in which granulomatous destruction of nasal cartilage can result in perforation and saddle-nose deformity. Prompt diagno- sis of GPA is important to initiate therapy which may be life-saving and organ sparing
ANCA-positive vasculitis: Clinical implications of ANCA types and titers
INTRODUCTION:
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease that can affect multiple organs, the kidney being one of the most affected. Apart from the diagnostics value of ANCA, they have also been advocated as biomarkers of the disease activity. Recently, the genetic changes found in polyangiitis associated with serine-protease proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA raised the possibility of immune-pathogenic and therapeutic differences.
OBJECTIVE:
To identify differences in the number of relapses, inflammatory markers, outcomes and renal histology related to the types of ANCA. To analyze the implications of ANCA titers in prognosis.
METHOD:
A retrospective observational study in a Portuguese tertiary hospital.
RESULTS:
There were no differences in the progression of renal function, histological pattern and initial treatment with regard to ANCA subtypes. As for the evaluated parameters, there were no significant differences according to the types of ANCA, except for mean CRP values within the normal range, which was 6.3±1.3 mg/L for MPO-ANCA and 12.4±10.14 mg/L for PR3-ANCA (p=0.04). We found that 66.7% of the MPO-ANCA-positive showed no relapses versus 40% in the case of PR3-ANCA-positive. There was no correlation between the ANCA titers at presentation, during remission, and in the last evaluation, and the number of relapses.
CONCLUSION:
PR3-ANCA patients have a mean CRP value within the normal range significantly higher than that of MPO-ANCA patients (p=0.04), which seems to reveal greater inflammatory activity in the first.info:eu-repo/semantics/publishedVersio
Genetic loci of Staphylococcus aureus associated with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides
The proteinase 3 (PR3)-positive anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) granulomatosis with polyangiitis (GPA) has been associated with chronic nasal S. aureus carriage, which is a risk factor for disease relapse. The present study was aimed at comparing the genetic make-up of S. aureus isolates from PR3-ANCA-positive GPA patients with that of isolates from patients suffering from myeloperoxidase (MPO)-ANCA-positive AAV, and isolates from healthy controls. Based on a DNA microarray-based approach, we show that not only PR3-ANCA-positive GPA patients, but also MPO-ANCA-positive AAV patients mainly carried S. aureus types that are prevalent in the general population. Nonetheless, our data suggests that MPO-ANCA-associated S. aureus isolates may be distinct from healthy control- and PR3-ANCA-associated isolates. Furthermore, several genetic loci of S. aureus are associated with either PR3-ANCA- or MPO-ANCA-positive AAV, indicating a possible role for pore-forming toxins, such as leukocidins, in PR3-ANCA-positive GPA. Contrary to previous studies, no association between AAV and superantigens was detected. Our findings also show that a lowered humoral immune response to S. aureus is common for PR3-ANCA- and MPO-ANCA-positive AAV. Altogether, our observations imply that the presence or absence of particular virulence genes of S. aureus isolates from AAV patients contributes to disease progression and/or relapse
Anti-neutrofila cytoplasmatiska antikroppar (ANCA) hos hund
Anti-neutrofila cytoplasmatiska antikroppar (ANCA) är en specifik typ av autoantikroppar som
riktar sig mot innehållet i cytoplasmatiska granula hos neutrofila granulocyter. På humansidan
är de i hög grad associerade med sjukdomar som förlöper med vaskulit. Få studier har dock
gjorts om dess relevans inom veterinärmedicin. Målet med studien var att utvärdera förekomsten av ANCA hos hundar med steroid-responsiv meningit-arterit (SRMA) samt immunemediated rheumatic disease (IMRD) för att undersöka om dessa kan användas som en ny
diagnostisk markör för sjukdomar som förlöper med vaskulit. Serum samlades in från 17 hundar
med SRMA och 6 hundar med IMRD och analyserades via indirekt immunofluorescens samt
enzyme-linked immunosorent assay. Via indirekt immunofluorescens analyserades varje prov
med två olika fixeringar: etanolfixerade granulocyter samt formalinfixerade granulocyter. Det
gjordes för att enklare kunna skilja mellan ANCA och antinukleära antikroppar (ANA).
Majoriteten av alla prover (28 av 30) var åtminstone svagt positiva för antingen c-ANCA eller
p-ANCA vid etanolfixering. En cutoff på 1:100 valdes för etanolfixering, samt 1:10 för formalinfixering.
Av hundarna med SRMA var 2 positiva för c-ANCA vid formalinfixering. Av hundarna med
IMRD var 4 positiva för p-ANCA vid etanolfixering samt 3 positiva för c-ANCA vid formalinfixering. Endast en av dessa hundar, tillhörande gruppen med IMRD, var positiv både vid
formalin och etanolfixering. Samtliga ANCA-positiva prover var negativa för både MPO
och/eller PR3 med ELISA.
Tre av fyra p-ANCA-positiva hundar var positiva även för ANA. Från studier av ANCA hos
människor har det visat sig att det kan vara svårt att skilja ANA från p-ANCA.
Mycket är fortfarande oklart angående ANCA hos hund. Eventuell interferens av ANA vid
immunofluorescens-teknik vid samtidig närvaro av p-ANCA gör att vidare studier krävs för att
fastställa relevansen och de diagnostiska möjligheterna för ANCA hos hundar.Anti-neutrophil cytoplasmic antibodies (ANCA) is a specific type of autoantibody directed
towards the contents of cytoplasmic granules in neutrophil granulocytes. In humans the
antibodies are highly associated with diseases progressing with vasculitis. Few studies have
been carried out on the relevance of ANCA in veterinary medicine. The aim of the study was
to evaluate the presence of ANCA in dogs with steroid-responsive meningitis-arteritis (SRMA)
and immune-mediated rheumatic disease (IMRD) to evaluate whether ANCA can be used as a
new diagnostic marker in dogs in diseases progressing with small vessel vasculitis. Serum was
collected from 17 dogs with SRMA and 6 with IMRD and analyzed through indirect immunefluorescence as well as enzyme-linked immunosorbent assay.
The majority of all samples (28 out of 30) were at the very least slightly positive for either cANCA or p-ANCA on ethanolfixation. A cutoff of 1:100 was chosen for ethanolfixation and
1:10 for formalinefixation.
Out of the dogs suffering from SRMA, 2 were positive for c-ANCA with formalinefixation.
Out of the dogs suffering from IMRD, 4 were positive for p-ANCA with ethanolfixation and 3
were positive with formalinefixation. Only one of these dogs, belonging to the IMRD-group,
was positive both with formaline and ethanolfixation. All the ANCA-positive dogs were
negative for PR3 as well as MPO through ELISA.
Three out of four p-ANCA-positive dogs were also positive to ANA. Studies on ANCA in
humans have shown that it can be difficult to distinguish ANA from p-ANCA.
Much is still unknown concerning ANCA in dogs. Possible interference of ANA during
immunofluorescence-analysis of p-ANCA leads to more research needing to be done in order
to determine the relevance and diagnostic possibilities of ANCA in dogs
Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis.
Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH
Child-rearing With Minimal Domination: A Republican Account
Parenting involves an extraordinary degree of power over children. Republicans are concerned about domination, which, on one view, is the holding of power that fails to track the interests of those over whom it is exercised. On this account, parenting as we know it is dominating due to the low standards necessary for acquiring and retaining parental rights and the extent of parental power. Domination cannot be fully eliminated from child-rearing without unacceptable loss of value. Most likely, republicanism requires that we minimise children’s domination. I examine alternative models of child-rearing that are immune to republican criticism
Antineutrophil Cytoplasmic Antibodies Testing in a Large Cohort of Unselected Greek Patients
Objective. To retrospectively evaluate ANCA testing in a cohort of unselected Greek in- and outpatients. Methods. In 10803 consecutive serum samples, ANCA were tested by indirect immunofluorescence (IIF) and ELISA. ELISA in inpatients was performed only on IIF positive sera. Results. Low prevalence (6.0%) of IIF positive samples was observed. Among these samples, 63.5% presented perinuclear (p-ANCA), 9.3% cytoplasmic (c-ANCA) and 27.2% atypical (x-ANCA) pattern. 16.1% of p-ANCA were antimyeloperoxidase (anti-MPO) positive, whereas 68.3% of c-ANCA were antiproteinase-3 (anti-PR3) positive. Only 17 IIF negative outpatients' samples were ELISA positive. ANCA-associated vasculitides (AAV), connective tissue disorders and gastrointestinal disorders represented 20.5%, 23.9%, and 21.2% of positive results, respectively. AAV patients exhibited higher rates of MPO/PR3 specificity compared to non-AAV (93.8% versus 8%). Conclusions. This first paper on Greek patients supports that screening for ANCA by IIF and confirming positive results by ELISA minimize laboratory charges without sacrificing diagnostic accuracy
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