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"Small vessel vasculitis"

Supaporn Suwanchote, Muanpetch Rachayon, Pongsawat Rodsaward, Jongkonnee Wongpiyabovorn, Tawatchai Deekajorndech, Helen L Wright, Steven W Edwards, Michael W Beresford, Pawinee Rerknimitr, Direkrit Chiewchengchol
Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies that cause systemic vascular inflammation by binding to target antigens of neutrophils. These autoantibodies can be found in serum from patients with systemic small-vessel vasculitis and they are considered as a biomarker for ANCA-associated vasculitis (AAV). A conventional screening test to detect ANCA in the serum is indirect immunofluorescence study, and subsequently confirmed by enzyme-linked immunosorbent assay. A positive staining of ANCA can be classified into three main categories based on the staining patterns: cytoplasmic, perinuclear, and atypical...
March 10, 2018: Clinical Rheumatology
James C Wang, Brittany A Leader, Ryan A Crane, Bernadette L Koch, Matthew M Smith, Stacey L Ishman
Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e...
April 2018: International Journal of Pediatric Otorhinolaryngology
Judy Savige, Michelle Trevisin, Wendy Pollock
Testing for antineutrophil cytoplasmic antibodies (ANCA) is performed to diagnose or exclude small vessel vasculitis, and, in treated patients, to monitor disease activity. However testing is also undertaken to assist with the diagnosis of other autoimmune diseases and some infections. Most laboratories use the same assays for all sera regardless of the testing indications. The International Consensus Statement on ANCA Testing and Reporting recommended screening for ANCA by indirect immunofluorescence (IIF) and confirming IIF-positive sera in antigen-specific ELISAs for both proteinase 3 (PR3) and myeloperoxidase (MPO)...
February 24, 2018: Journal of Immunological Methods
Ivka Djakovic, Drazan Butorac, Zeljko Vucicevic, Vesna Kosec, Andrea Tesija Kuna, Liborija Lugović-Mihić
Henoch-Schönlein purpura (HSP) is an IgA-mediated small vessels' vasculitis that affects the skin, intestines and kidneys. Pregnancy has been reported as an exacerbating factor. We present the case of a 24-year-old primigravida with HSP that occurred in the third trimester and lasted up to the end of the successful delivery. She had pruritic maculopapular exanthema on her legs. Biopsy of a cutaneous lesion was performed for histopathologic features and direct immunofluorescence (DIF) for the presence of perivascular IgA deposition...
February 15, 2018: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
Michael L Chen, Agata Chlopik, Maija P Hoang, Gideon P Smith
Erythema elevatum diutinum (EED) is a rare, chronic small-vessel vasculitis that presents as firm, red, violaceous, or brown papules and nodules on the extensor surfaces of the limbs. Oral dapsone is considered first-line therapy for EED; in the current case report, a patient presenting with EED began dapsone treatment and symptoms subsided within two weeks. Seven months later, the patient became pregnant and stopped dapsone owing to her concerns with dapsone use during pregnancy, resulting in recurrence of EED symptoms...
October 15, 2017: Dermatology Online Journal
Therese Woodring, Ronnie Abraham, Stephanie Frisch
Cutaneous leukocytoclastic vasculitis (LCV) can occur as skin-limited disease or as part a systemic vasculitis. Appropriate workup includes the evaluation of antineutrophil cytoplasmic antibodies (ANCAs), with a positive titer raising concern for the associated primary vasculitides including microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), or eosinophilic granulomatosis with polyangiitis (EGPA). In the absence of systemic findings, however, a drug etiology must also be considered. Tumor necrosis factor (TNF) inhibitors, propylthiouracil, levamisole-adulterated cocaine, hydralazine, and minocycline have been previously documented to induce ANCA-positive vasculitis (APV), which may present with conspicuously high ANCA titers...
August 15, 2017: Dermatology Online Journal
David B G Oliveira
The small vessel vasculitides granulomatosis with polyangiitis (GPA) and microscopic polyangiitis are associated with autoantibodies to neutrophil cytoplasm antigens (ANCA), principally proteinase-3 (PR3) and myeloperoxidase (MPO). There is an association between GPA and nasal carriage of Staphylococcus aureus. The recent finding that S. aureus produces proteins that bind tightly to and block the function of both PR3 and MPO suggests a mechanism for ANCA formation. The bacterial protein-autoantigen conjugate is recognised by B cells with ANCA specificity, internalised, and the bacterial protein processed and presented to T cells with specificity for bacterial peptides...
March 2018: Medical Hypotheses
Riley Dean, Alison M Messer, Melanie Pickett, Richard Jahan-Tigh
Cutaneous leukocytoclastic vasculitis (LCV) is type of small vessel vasculitis that commonly presents as palpable purpura involving the lower extremities and buttocks. Approximately half of cases are idiopathic, but the disease may be triggered by infection, drug reaction, inflammatory disease, or other causes. We report a case of leukocytoclastic vasculitis secondary to the novel anticoagulant rivaroxaban (Xarelto®).
November 15, 2017: Dermatology Online Journal
Aliaksandr Trusau, Michael L Brit
Propylthiouracil (PTU) is a commonly used medication for the treatment of hyperthyroidism. PTU is known to cause different adverse reactions including autoimmune syndromes. PTU-induced autoimmune syndromes can be classified into drug-induced lupus or drug-induced vasculitis. Differential diagnoses could be very challenging. PTU-induced vasculitis is more common than PTU-induced lupus, and has a higher risk of morbidity and mortality. Usually it is limited to the skin in a form of cutaneous leukocytoclastic vasculitis, but may also affect organs including kidneys and lungs...
2018: Journal of Community Hospital Internal Medicine Perspectives
Amrita Padda, Elena Schiopu, Justin Sovich, Vincent Ma, Ajjai Alva, Leslie Fecher
BACKGROUND: Immune check point inhibitors (ICIs) have emerged as a new therapeutic paradigm for a variety of malignancies including metastatic melanoma. As the use of ICIs expand, immune-mediated adverse events are becoming a common occurrence. CASE PRESENTATION: We describe the first reported patient with small vessel vasculitis, manifested by digital ischemia, following treatment with high dose Ipilimumab for resected stage IIIB/C melanoma. This patient received high dose steroids, five-day intravenous (IV) Epoprostenol protocol, botulinum toxin injections, and Rituximab 375 mg/m2 weekly for four cycles...
February 12, 2018: Journal for Immunotherapy of Cancer
Dorota Dąbrowska, Ewa Jabłońska, Marzena Garley, Jolanta Sawicka-Powierza, Karolina Nowak
Vascular diseases constitute a global health issue due to the increasing number of cases of patients with these diseases. The pathogenesis of the majority of these diseases, including atherosclerosis and thrombosis, is complex and not yet fully understood. One of the major causes for their occurrence can be immune disorders resulting in the development of a chronic inflammation within the vessels. In recent years, studies have placed emphasis on the role of neutrophils in the development of these diseases, i...
February 5, 2018: Archivum Immunologiae et Therapiae Experimentalis
Elena Csernok, Juliane Mahrhold, Bernhard Hellmich
The current practice for detection of anti-neutrophil cytoplasm antibodies (ANCA) directed against proteinase 3 (PR3) and myeloperoxidase (MPO) has been screening by indirect immunofluorescence (IIF) followed by an antigen specific tests for PR3- and MPO-ANCA. However, ANCA diagnostics have undergone many technical developments that have affected the 1999 international consensus recommendations, and lead to a revision of the existing ANCA detection strategy. Recent European multicentre studies have compared the diagnostic performance of various ANCA detection methods and demonstrated that PR3- and MPO-ANCA immunoassays yielded the highest diagnostic accuracy...
January 27, 2018: Journal of Immunological Methods
Ujjwal Rastogi, Ziad Sergie, Sean Pinney, Noah Moss
Eosinophilic granulomatosis with polyangitis (EGPA), previously referred to as Churg-Strauss syndrome, is a necrotizing small vessel vasculitis associated with eosinophilic infiltrates and extravascular granulomas. We report a case of a Caucasian woman successfully bridged to heart transplantation with a continuous flow left ventricular assist device (LVAD) who survived recurrence of EGPA in the allograft. This article is protected by copyright. All rights reserved.
February 2, 2018: American Journal of Transplantation
H Elsayed Mansour, S Gamal Arafa, W Abdelfatah Shehata
A 30-year-old female presented to the rheumatology outpatient clinic of the Internal Medicine Department, Ain Shams University Hospital, Cairo, Egypt, complaining of a large right leg ulcer consistent with pyoderma gangrenosum. There was history of recurrent attacks of bleeding per rectum of one-year duration. During hospitalization she noticed blurring of vision in the left eye with diffuse blackish discoloration of the feet and toes, consistent with small-vessel vasculitis. Colonoscopy with biopsy and histopathology confirmed the diagnosis of inflammatory bowel disease-ulcerative colitis (IBD-UC)...
January 1, 2018: Lupus
Percy Herrera-Añazco, Percy Velásquez-Castillo, Josmel Pacheco-Mendoza, Germán Valenzuela-Rodriguez, Carmen Asato-Higa
The pauci-immune crescentic glomerulonephritis (PICGN) is generally associated with small-vessel vasculitis with a few reported cases associated with other autoimmune diseases such as Systemic Lupus Erythematosus (SLE). We present the case of a female 34-year-old patient with acute kidney injury symptoms with indication for renal replacement therapy in the context of clinical SLE diagnosis. A kidney biopsy was conducted and it was found that most glomeruli showed some segmental sclerosis with synechia to the Bowman's capsule...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Neda Zarghami Esfahani, Daniel M Anderson, Connie Pieper, Harold P Adams
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare diseases characterized by a necrotizing small-vessel vasculitis and circulating ANCA that comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Acute ischemic stroke (AIS) can be a manifestation of central nervous system (CNS) involvement in these diseases. Furthermore, intracerebral hemorrhage (ICH) is a potential complication of these necrotizing vasculitides...
December 2017: ENeurologicalSci
M Poornimambaa, N Asokan, Joy Augustine
No abstract text is available yet for this article.
November 2017: Indian Dermatology Online Journal
Kamble J Harsha, Sujit A Jagtap, Tirur R Kapilamoorthy, Chandrasekharan Kesavadas, Bejoy Thomas, Neelima Radhakrishnan
BACKGROUND: Central nervous system (CNS) vasculitis is an uncommon disease, which is a diagnostic and therapeutic challenge for physicians. Large and medium vessel vasculitis is relatively easy to diagnose by angiogram compared to small vessel vasculitis, where angiograms are often normal; imaging features described till date are sensitive but not specific. PURPOSE: Here, we describe distinct magnetic resonance (MR) imaging features of CNS small vessel vasculitis...
November 2017: Neurology India
Anna Masiak, Zbigniew Zdrojewski
Granulomatosis with polyangiitis (GPA) is a chronic, relapsing, primary systemic small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies. The disease is characterised by the involvement of various organs. The relapse rate rises from about 20% at 12 months to about 60% at 5 years. The combination of glucocorticoids and cyclophosphamide remains the standard therapy for patients with generalised GPA; nevertheless, some patients do not respond satisfactorily to this treatment. According to EULAR-EDTA recommendations for the management of ANCA-associated vasculitis, RTX should be considered for remission-induction of new-onset as well as major relapse of organ-threatening or life-threatening GPA...
2017: Reumatologia
Anawin Sanguankeo, Natanong Thamcharoen, Sikarin Upala
BACKGROUND: Calciphylaxis in a nondialysis patient is a rare condition and is characterized by calcific deposition in tissue. We present a case of calciphylaxis in a nondialysis patient who was diagnosed by clinical presentation and skin biopsy and was treated with sodium thiosulfate with improvement of skin lesions. CASE: A 43-year-old female with type 2 diabetes and atrial fibrillation taking oral anticoagulation medication presented with reddish drainage from the right buttock...
2017: Clinical Nephrology. Case Studies
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