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

2019 ◽  
Vol 7 (4) ◽  
pp. 1347-1351.e3 ◽  
Author(s):  
Amélie Leurs ◽  
Cécile Chenivesse ◽  
Benjamin Lopez ◽  
Jean-Baptiste Gibier ◽  
Guillaume Clément ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Diagnostic Tool ◽  
Granulomatosis With Polyangiitis ◽  
Hypereosinophilic Syndrome ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Eosinophilic Granulomatosis

Connective tissue diseases

2020 ◽  
pp. 127-184
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Granulomatosis With Polyangiitis ◽  
Connective Tissue Diseases ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Assessment Tools ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Key Points ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis

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 ◽  
2019 ◽  
pp. 355-359
Author(s):  
Felipe Martínez
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Vascular Wall ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
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.

scholarly journals Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides

2019 ◽  
Vol 6 (6) ◽  
pp. e615 ◽  
Author(s):  
Antje Bischof ◽  
Veronika K. Jaeger ◽  
Robert D. M. Hadden ◽  
Raashid A. Luqmani ◽  
Anne-Katrin Pröbstel ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Vasculitic Neuropathy ◽  
Diagnostic Certainty ◽  
Primary Systemic Vasculitis ◽  
Organ Manifestations ◽  
Eosinophilic Granulomatosis

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.

scholarly journals Eosinophilic granulomatosis with polyangiitis with an unusual presentation

2021 ◽  
Vol 35 (2) ◽  
Author(s):  
Rui Nogueira ◽  
◽  
Ana Belmira ◽  
Vitor Sousa ◽  
Rui Alves ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Central Retinal Artery Occlusion ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Peripheral Eosinophilia ◽  
Reactive Protein ◽  
Necrotizing Crescentic Glomerulonephritis ◽  
The Right ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis is an ANCA vasculitis characterized by asthma, rhinosinusitis and peripheral eosinophilia. The kidney is infrequently involved, usually in the form of necrotizing crescentic glomerulonephritis. We present the case of a 60-year-old man who presented with painless sudden loss of visual acuity, purpuric exanthem in his legs, asthenia and myalgia. CT-scan ruled out acute vascular and intracranial space occupant lesions. Optical coherence tomography showed signs of left central retinal artery occlusion and perfusion deficits in the right arterial retinal blood supply. Complementary study showed prominent peripheral eosinophilia (24.500 cel/uL), increased serum IgE (1260U/L) and increased C-reactive protein (10.6mg/ dl). During admission, the patient presented with acute kidney failure (serum creatinine of 4.7mg/dl) and an exceptionally high p-ANCA MPO titer (>600U/L). Eosinophilic granulomatosis with polyangiitis was diagnosed and plasmapheresis, pulse steroid therapy and intravenous cyclophosphamide were provided. Kidney biopsy showed interstitial nephritis with high eosinophil content while the glomerulus was relatively spared, with only mild endocapillary proliferation. The patient didn’t require dialysis. Kidney function was normal at discharge, although the visual deficit did not improve.

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

2020 ◽  
Author(s):  
Nestor Oliva-Damaso ◽  
Andrew S Bomback
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Information ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Consensus Conference ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
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.

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