Sputum Antineutrophil Cytoplasmic Antibodies in Serum Antineutrophil Cytoplasmic Antibody–Negative Eosinophilic Granulomatosis with Polyangiitis

We report a case of eosinophilic cholecystitis associated with eosinophilic granulomatosis with polyangiitis (EGPA) complicated by cerebral hemorrhage. A 60-year-old man presented to a local hospital with a diagnosis of acute cholecystitis, with persistent fever and epigastric pain for 2 weeks. His symptoms persisted despite 3-week hospitalization; therefore, he was transferred to our hospital for further evaluation. Laboratory investigations upon admission showed white blood cells 26,300/µL and significant eosinophilia (eosinophils 61%). Abdominal computed tomography revealed no gallbladder enlargement but a circumferentially edematous gallbladder wall. Additional blood test results were negative for antineutrophil cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies; however, immunoglobulin (Ig)G and IgE levels were high at 1,953 mg/dL and 3,040/IU/mL, respectively. He improved following endoscopic transnasal gallbladder drainage for cholecystitis and was diagnosed with EGPA and received corticosteroid and immunosuppressant combination therapy. The eosinophil count decreased immediately after treatment, and abdominal pain and numbness resolved. He returned with left-sided suboccipital hemorrhage likely attributed to EGPA 6 months after discharge. EGPA is characterized by inflammation of small blood vessels and clinically manifests with an allergic presentation of bronchial asthma, as well as renal dysfunction, interstitial pneumonia, enteritis, and cerebral hemorrhage. Few reports have described cholecystitis as a presenting symptom of EGPA. We report a rare case of such a presentation with added considerations.

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Connective tissue diseases

Oxford Handbook of Musculoskeletal Nursing ◽

10.1093/med/9780198831426.003.0005 ◽

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.

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Pulmonary Vasculitis

Chest Imaging ◽

10.1093/med/9780199858064.003.0061 ◽

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.

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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):

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.

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Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis

RMD Open ◽

10.1136/rmdopen-2019-000905 ◽

2019 ◽

Vol 5 (1) ◽

pp. e000905 ◽

Cited By ~ 13

Author(s):

Vítor Teixeira ◽

Aladdin J Mohammad ◽

Rachel B Jones ◽

Rona Smith ◽

David Jayne

Keyword(s):

Partial Response ◽

Granulomatosis With Polyangiitis ◽

Antineutrophil Cytoplasmic Antibodies ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Free Survival ◽

Prednisolone Dose ◽

Tertiary Centre ◽

Limited Experience ◽

Treatment Responses ◽

Eosinophilic Granulomatosis

IntroductionEosinophilic granulomatosis with polyangiitis (EGPA) is a subset of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis with distinct pathophysiological mechanisms, clinical features and treatment responses. Rituximab is a licensed therapy for granulomatosis with polyangiitis and microscopic polyangiitis but there is limited experience of rituximab in EGPA.MethodsEGPA patients from a tertiary centre who received rituximab for mostly refractory EGPA or in whom cyclophosphamide was contra indicated were studied. A standardised dataset was collected at time of initial treatment and every 3 months for 24 months. Response was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 and partial response as ≥50% reduction in BVAS from baseline. Remission was defined as a BVAS of 0 on prednisolone dose ≤5 mg.ResultsSixty-nine patients (44 female) received rituximab between 2003 and 2017. Improvement (response and partial response) was observed in 76.8% of patients at 6 months, 82.8% at 12 months and in 93.2% by 24 months, while relapses occurred in 54% by 24 months, with asthma being the most frequent manifestation. The median BVAS decreased from 6 at baseline to 1 at 6 months, and 0 at 12 and 24 months. Prednisolone dose (mg/day, median) decreased from 12.5 to 7, 7.5 and 5 at 6, 12 and 24 months, respectively. ANCA positive patients had a longer asthma/ear, nose and throat (ENT) relapse-free survival time and a shorter time to remission.DiscussionRituximab demonstrated some efficacy in EGPA and led to a reduction in prednisolone requirement, but asthma and ENT relapse rates were high despite continued treatment. The ANCA positive subset appeared to have a more sustained response on isolated asthma/ENT exacerbations.

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Eosinophilic granulomatosis with polyangiitis in a Nigerian woman

BMJ Case Reports ◽

10.1136/bcr-2018-228901 ◽

2019 ◽

Vol 12 (6) ◽

pp. e228901

Author(s):

Ngozi Lina Ekeigwe ◽

Olufemi Adelowo ◽

Ehiaghe Lonia Anaba ◽

Hakeem Olaosebikan

Keyword(s):

Granulomatosis With Polyangiitis ◽

Rare Condition ◽

Antineutrophil Cytoplasmic Antibodies ◽

Systemic Autoimmune Disease ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

American College Of Rheumatology ◽

Nigerian Woman ◽

History Of ◽

Perinuclear Anca ◽

Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss Syndrome, is a systemic autoimmune disease that is usually associated with asthma and eosinophilia. It is a rare condition associated with antineutrophil cytoplasmic antibodies (ANCA). We report a case of a 52-year-old Nigerian woman who presented with bilateral leg swelling with multiple ulcers, background history of allergic rhinitis and chronic sinusitis since adolescence. There were also associated symptoms of peripheral neuropathy, weight loss, peripheral blood eosinophilia and positive perinuclear ANCA and she was previously managed for rheumatoid arthritis and asthma. She fulfilled the American College of Rheumatology criteria for EGPA and was treated with glucocorticoids and methotrexate which led to significant improvement of her symptoms.

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Non-infectious aortic and mitral valve vegetations in a patient with eosinophilic granulomatosis with polyangiitis

BMJ Case Reports ◽

10.1136/bcr-2018-225947 ◽

2019 ◽

Vol 12 (5) ◽

pp. e225947 ◽

Cited By ~ 3

Author(s):

Kamalesh Karthikeyan ◽

Sudarshan Balla ◽

Martin A Alpert

Keyword(s):

Mitral Valve ◽

Granulomatosis With Polyangiitis ◽

Antineutrophil Cytoplasmic Antibodies ◽

Cardiac Complications ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Rare Form ◽

Coronary Arteritis ◽

Endocardial Fibrosis ◽

Arteries And Veins ◽

Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis characterised by atopic manifestations, inflammation of small-sized to medium-sized arteries and veins, hypereosinophilia and tissue infiltration with eosinophils. Cardiac complications occur most commonly in the absence of antineutrophil cytoplasmic antibodies. Cardiac complications include coronary arteritis (rare), pericarditis, myocarditis, endocardial fibrosis (Loeffler’s endocarditis) and intracavitary thrombosis of the left or right ventricle. This is the first reported case of large non-infectious valvular vegetations associated with EGPA.

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