ANCA Are Detectable in Nearly All Patients with Active Severe Wegener’s Granulomatosis

Finkielman, Javier D.; Lee, Augustine S.; Hummel, Amber M.; Viss, Margaret A.; Jacob, G.L.; Homburger, Henry A.; Peikert, Tobias; Hoffman, Gary S.; Merkel, Peter A.; Spiera, Robert; Clair, E. William St.; Davis, John C.; McCune, W. Joseph; Tibbs, Andrea K.; Ytterberg, Steven R.; Stone, John H.; Specks, Ulrich

doi:10.1016/j.amjmed.2006.08.016

Cited by 245 publications

(170 citation statements)

References 19 publications

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“…Although the ANCA levels usually decrease from the start of induction therapy to the moment of sustained remission, this is not always the case [58,62]. A reduction or complete disappearance of ANCA is therefore not required to achieve sustained remission.…”

Section: Persistence Of Anca Positivitymentioning

confidence: 99%

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Kemna¹,

Paassen²,

Damoiseaux³

et al. 2017

Expert Opinion on Orphan Drugs

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Introduction: Granulomatosis with Polyangiitis (GPA; formerly Wegener's) and Microscopic Polyangiitis (MPA) are inflammatory disease entities affecting small to medium vessels. They are characterized by the presence of anti-neutrophil cytoplasmic antibodies (ANCA) and are frequently grouped together with Eosinophilic Granulomatosis with Polyangiitis (EGPA; formerly Churg Strauss Syndrome) under the term ANCA-associated Vasculitis (AAV). Due to effective immunosuppressive therapy that was introduced more than 45 years ago, AAV has become a chronic disease in which relapses occur frequently during maintenance treatment or later on after all immunosuppressive therapy is stopped. Since relapses are associated with morbidity and mortality, early detection and prevention of relapses is of great importance. Areas covered: This review focuses on the potential strategies to guide maintenance therapy in patients with GPA or MPA, with an emphasis on patient classifications and the role of serial ANCA measurements. Expert opinion: Risk factors for relapses, e.g., genetic background of the patient, have been studied during the last three decades. Only few of these risk factors directly influence the management of patients. One potentially important factor that may influence therapeutic decisions in AAV patients is serial measurement of ANCA. Recently, it became clear that serial ANCA measurement is an attractive approach in AAV patients with (a history of) capillaritis, e.g., renal involvement or alveolar haemorrhage, but not in patients with more limited disease. Whether ANCA rises can be used to guide therapy andhence-to prevent relapses has been a great debate during the last three decades. At present, we conclude that small studies do support such an approach but that these findings require further validation in larger randomized clinical trials. ARTICLE HISTORY

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Section: Persistence Of Anca Positivitymentioning

confidence: 99%

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Kemna¹,

Paassen²,

Damoiseaux³

et al. 2017

Expert Opinion on Orphan Drugs

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“…AAV comprises three heterogeneous syndromes: Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), and the Churg-Strauss syndrome (CSS) (84). These three multisystem disorders are characterized by necrotizing small-vessel vasculitis with predilection for the kidneys, lungs, and peripheral nervous system that share the occurrence of ANCA in most patients at the time of initial presentation (85)(86)(87). The use of CYC has substantially improved the prognosis of these patients, turning a previously universally fatal illness into a chronically relapsing disease process (88); however, approximately 10% of patients fail to respond to CYC therapy.…”

Section: New Therapiesmentioning

confidence: 99%

Idiopathic Membranous Nephropathy

Fervenza

Sethi

Specks

2008

Clinical Journal of the American Society of Nephrology

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131

123

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Idiopathic membranous nephropathy is still the most common glomerular disease associated with nephrotic syndrome. The greater the proteinuria, the greater the long-term risk for renal failure. In addition, patients who have membranous nephropathy with nephrotic syndrome have significant morbidity and mortality, in particular related to thromboembolic and cardiovascular complications. There is no specific treatment for membranous nephropathy. Supportive care with the use of diuretics and angiotensin-converting enzyme inhibitors in combination with angiotensin II receptor blocker is recommended, but these agents have only a limited effect. Immunosuppressive treatment options include the use of corticosteroids, alkylating agents, cyclosporin A, tacrolimus, and mycophenolate mofetil, but their use is controversial, not all have been shown to be effective, and their use can be associated with significant adverse effects. This has resulted in relatively small improvement in the prognosis of membranous nephropathy in the past 30 yr, with up to 40% of patients eventually reaching end-stage renal failure. Agents that offer more complete response rates with lower adverse effects are truly needed. Recent data suggest that B cells play a key role in the pathogenesis of a number of autoimmune diseases including membranous nephropathy and that selective depletion of B cells in humans may be beneficial in preventing the production of pathogenic immunoglobulins and subsequent renal injury.Clin Case PresentationA 32-yr-old man underwent a general medical examination at his primary care physician's office in February 2004. At that time, he was noted to have elevated total cholesterol of 353 mg/dl and triglycerides of 417 mg/dl and was started on simvastatin 40 mg/d. During the course of the next 2 mo, he started developing swelling in his feet and ankles that proceeded to involve his calves. He also developed tenderness on his left calf. A diagnosis of a deep venous thrombosis was made, and he was started on oral anticoagulant therapy. Given the increased lower extremity edema, a urinalysis was conducted, and the patient was found to have significant proteinuria, with a 24-h urine collection showing proteinuria of 9.3 g. The patient had no history of diabetes, macroscopic hematuria, or hypertension. The patient underwent extensive serologic workup including monoclonal protein studies, serum complement levels, anti-neutrophil cytoplasmic antibodies, hepatitis B and C and HIV serology, and anti-nuclear antibody, all of which were negative or in the normal range, and in July 2004, he was referred to our institution for further evaluation. At presentation at the Mayo Clinic, the physical examination was of a healthy-appearing young man. His BP was 136/87 mmHg, pulse rate was 69, weight was 89.8 kg, and body mass index was 29 kg/m 2 . Eyes, nose, and throat examination was normal.There was no lymphadenopathy. Heart examination showed regular rate and rhythm with no murmurs. Lungs were clear to auscultation. Abdomen was soft, with no ...

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“…ANCA is an autoantibody mainly recognizing antigens on azurophilic granules in the neutrophil cytoplasm and is classified, from the staining pattern, as PR3-ANCA or MPO-ANCA. Approximately 82-94 % of patients with either GPA or microscopic polyangiitis (MPA) are ANCA positive, depending on severity of disease [1,2]. GPA is primarily associated with PR3-ANCA, while MPA is primarily associated with MPO-ANCA.…”

Section: Discussionmentioning

confidence: 99%

“…A part of this response is directed against previously shielded epitopes of neutrophil granule proteins, leading to high titers of autoantibodies known as anti-neutrophil cytoplasmic antibodies (ANCAs). Approximately 82-94 % of patients with either GPA or microscopic polyangiitis (MPA) are ANCA positive, depending on severity of the diseases [1,2]. GPA is primarily associated with proteinase 3 (PR3)-ANCA, while MPA is primarily associated with myeloperoxidase (MPO)-ANCA.…”

Section: Introductionmentioning

confidence: 99%

A case of (double) ANCA-negative granulomatosis with polyangiitis (Wegener’s)

et al. 2012

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A 60-year-old man had experienced cough, bloody sputum, and a 38°C fever for 1.5 months. He visited an outpatient clinic and received antibiotics and nonsteroidal anti-inflammatory drugs. However, because the symptoms continued, he visited our hospital. The past medical history included chronic sinusitis, hypertension, and diabetes mellitus. A chest x-ray film and computed tomography showed multiple pulmonary nodules with cavities.

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ANCA Are Detectable in Nearly All Patients with Active Severe Wegener’s Granulomatosis

Cited by 245 publications

References 19 publications

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Idiopathic Membranous Nephropathy

A case of (double) ANCA-negative granulomatosis with polyangiitis (Wegener’s)

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