Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis

Connective Tissue Diseases ◽

Assessment Tools ◽

Key Points ◽

Strauss Syndrome ◽

This chapter covers the connective tissue diseases including systemic lupus erythematosus, Sjögren’s syndrome, scleroderma, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (including granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis (formerly known as Churg–Strauss syndrome), and microscopic polyangiitis), polyarteritis nodosa, and Behçet’s disease. For each example of a connective tissue disease it provides an overview of the condition and classification criteria, alongside the prognosis. Techniques and tricks for diagnosis, clinical features, assessment tools, and treatment are all covered. Key points of nursing care are described, including the nurse’s role in treatment with thalidomide and cyclophosphamide, and any particular organs that can be affected is detailed.

Pulmonary Vasculitis

Chest Imaging ◽

10.1093/med/9780199858064.003.0061 ◽

2019 ◽

pp. 355-359

Author(s):

Felipe Martínez

Keyword(s):

Vascular Wall ◽

Small Vessel Vasculitis ◽

Goodpasture Syndrome ◽

Work Up ◽

Eosinophilic Granulomatosis ◽

Common Manifestation

Vasculitis refers to inflammation of blood vessel walls that results in vascular wall destruction and ischemic injury to affected organs. Common vasculitides discussed herein include Takayasu arteritis (TAK), giant cell arteritis (GCA), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides such as granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), and anti-glomerular basement membrane (anti-GBM) disease or Goodpasture syndrome. Vasculitides are further subcategorized depending of the size of the predominantly affected vessels: large, medium and small vessel vasculitis. The affected vessel size strongly influences the clinical and imaging manifestations of the disease. Intrathoracic involvement is more common in small and large vessel vasculitides. Diffuse alveolar hemorrhage (DAH), a common manifestation of vasculitis, is considered a syndrome rather than a specific entity and will be discussed in this chapter. However, it should be noted that DAH may also result from non-vasculitic etiologies. The work up and diagnosis of patients with primary vasculitides is challenging and requires close collaboration between the clinician, the radiologist and the pathologist. Radiographic abnormalities are non specific or may be absent. CT and MRI are the imaging modalities of choice for the evaluation and follow up of these patients, and should be considered despite normal radiographics.

Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000615 ◽

2019 ◽

Vol 6 (6) ◽

pp. e615 ◽

Cited By ~ 4

Author(s):

Antje Bischof ◽

Veronika K. Jaeger ◽

Robert D. M. Hadden ◽

Raashid A. Luqmani ◽

Anne-Katrin Pröbstel ◽

...

Keyword(s):

Systemic Vasculitis ◽

Nerve Biopsy ◽

Vasculitic Neuropathy ◽

Diagnostic Certainty ◽

Primary Systemic Vasculitis ◽

Organ Manifestations ◽

ObjectiveReported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN.MethodsData of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis study were analyzed cross-sectionally. VN was categorized as definite (histology proven), probable (multiple mononeuropathy or nerve biopsy consistent with vasculitis), or possible (all others). Associations with other organ manifestations were compared in patients with and without VN.ResultsNine hundred fifty-five patients (mean age 57 years, range 18–91 years, 51% female) were identified. Of these, 572 had granulomatosis with polyangiitis (GPA), 218 microscopic polyangiitis (MPA), and 165 eosinophilic granulomatosis with polyangiitis (EGPA). The prevalence of VN was 65% in EGPA, 23% in MPA, and 19% in GPA. Nerve biopsy was performed in 32/269 (12%) patients, demonstrating definite vasculitis in 17/32 (53%) of patients. VN was associated with myeloperoxidase-ANCA positivity (p = 0.004) and skin (p < 0.001), musculoskeletal, (p < 0.001) and cardiovascular (p = 0.005) involvement. Patients with VN were less likely to have renal (p < 0.001), eye (p < 0.001), and gastrointestinal (p = 0.023) involvement.ConclusionsOur study provides comprehensive insights into the prevalence and organ associations of VN in a large, systematically collected AAV cohort. VN is most commonly associated with skin, musculoskeletal, and cardiovascular manifestations. In routine clinical practice, diagnosis of VN is infrequently confirmed by the gold standard of nerve biopsy but rather supported by the clinical setting of active systemic AAV.

Immunopathogenesis of ANCA-Associated Vasculitis

International Journal of Molecular Sciences ◽

10.3390/ijms21197319 ◽

2020 ◽

Vol 21 (19) ◽

pp. 7319

Author(s):

Andreas Kronbichler ◽

Keum Hwa Lee ◽

Sara Denicolo ◽

Daeun Choi ◽

Hyojeong Lee ◽

...

Keyword(s):

Microscopic Polyangiitis ◽

Autoimmune Disorder ◽

Proteinase 3 ◽

Disease Biomarkers ◽

Disease Phenotypes ◽

Anca Associated Vasculitis ◽

Clinical Presentations ◽

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small- and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.

Development of Hypertrophic Pachymeningitis in a Patient With Antineutrophil Cytoplasmic Antibody–Negative Eosinophilic Granulomatosis With Polyangiitis

JCR Journal of Clinical Rheumatology ◽

10.1097/00124743-900000000-99332 ◽

2018 ◽

pp. 1

Author(s):

Yasuhiro Nakano ◽

Yoshia Miyawaki ◽

Ken-Ei Sada ◽

Yuriko Yamamura ◽

Yuzuki Kano ◽

...

Keyword(s):

Hypertrophic Pachymeningitis ◽

A new index for distinguishing hypereosinophilic syndrome and antineutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis

Asian Pacific Journal of Allergy and Immunology ◽

10.12932/ap-080420-0805 ◽

2020 ◽

Keyword(s):

Hypereosinophilic Syndrome ◽

C-Reactive protein as a diagnostic tool in differential diagnosis of hypereosinophilic syndrome and antineutrophil cytoplasmic antibody–negative eosinophilic granulomatosis with polyangiitis

The Journal of Allergy and Clinical Immunology In Practice ◽

10.1016/j.jaip.2018.10.002 ◽

2019 ◽

Vol 7 (4) ◽

pp. 1347-1351.e3 ◽

Cited By ~ 7

Author(s):

Amélie Leurs ◽

Cécile Chenivesse ◽

Benjamin Lopez ◽

Jean-Baptiste Gibier ◽

Guillaume Clément ◽

...

Keyword(s):

Differential Diagnosis ◽

Diagnostic Tool ◽

Hypereosinophilic Syndrome ◽

C Reactive Protein ◽

Reactive Protein ◽

A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature

Case Reports in Rheumatology ◽

10.1155/2017/2421760 ◽

2017 ◽

Vol 2017 ◽

pp. 1-7 ◽

Cited By ~ 4

Author(s):

Richard A. Lau ◽

Ramandeep Bains ◽

Duminda Suraweera ◽

Jane Ma ◽

Emil R. Heinze ◽

...

Keyword(s):

English Literature ◽

Renal Involvement ◽

High Dose ◽

Proteinase 3 ◽

Anca Associated Vasculitis ◽

Digital Ischemia

This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al., 2016). The patient responded well to therapy including high dose steroids and cyclophosphamide, with improvement of all organs involved and had no further digital ischemia or gangrene on follow-up. We include a review of the English literature summarizing presentation, management, and outcome of 16 similar cases.

Proposal for a more practical classification of antineutrophil cytoplasmic antibody-associated vasculitis

Clinical Kidney Journal ◽

10.1093/ckj/sfaa255 ◽

2020 ◽

Author(s):

Nestor Oliva-Damaso ◽

Andrew S Bomback

Keyword(s):

Clinical Information ◽

Consensus Conference ◽

Chapel Hill ◽

Chapel Hill Consensus Conference ◽

Eosinophilic Granulomatosis ◽

Relapse Rates

Abstract The nomenclature for antineutrophil cytoplasmic antibody (ANCA)-associated kidney disease has evolved from honorific eponyms to a descriptive-based classification scheme (Chapel Hill Consensus Conference 2012). Microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis do not correlate with presentation, response rates and relapse rates as when comparing myeloperoxidase versus leukocyte proteinase 3. Here we discuss the limitations of the currently used classification and propose an alternative, simple classification according to (i) ANCA type and (ii) organ involvement, which provides important clinical information of prognosis and outcomes.