scholarly journals Clinical Characteristics of Peripheral Neuropathy in Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Single-Center Study in China

2020 ◽  
Vol 2020 ◽  
pp. 1-10
Author(s):  
Zhaocui Zhang ◽  
Suying Liu ◽  
Ling Guo ◽  
Li Wang ◽  
Qingjun Wu ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Joint Pain ◽  
Associated Factors ◽  
Protective Factor ◽  
Granulomatosis With Polyangiitis ◽  
Lower Limbs ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
College Hospital ◽  
Eosinophilic Granulomatosis

Objective. To investigate clinical features, independent associated factors, treatment, and outcome of patients with peripheral neuropathy (PN) in eosinophilic granulomatosis with polyangiitis (EGPA). Methods. We retrospectively analyzed clinical data of 110 EGPA patients from 2007 to 2019 in Peking Union Medical College Hospital. The independent factors associated with PN in EGPA were analyzed with univariate and multivariate logistic regressions. Results. In EGPA with PN, paresthesia and muscle weakness were observed in 82% and 33% of patients, respectively. Both the upper and lower limbs were involved in 51% of patients. 30% of EGPA patients had symmetrical multiple peripheral neuropathy, whereas only 16.4% presented with mononeuritis multiplex. Compared to patients without PN, patients with PN had a higher erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, Birmingham vasculitis activity score (BVAS), and positivity of myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA). Regarding manifestations, patients with PN tended to develop weight loss and arthritis or joint pain. Notably, ANCA positivity, arthritis or joint pain, and higher BVAS were found to be independent associated factors for PN in EGPA. Patients with PN more frequently need glucocorticoid pulses and intravenous infusion of cyclophosphamide. With the longest follow-up of 11.0 years, we found that age and cardiac involvement were risk factors for survival, and female was the protective factor. Conclusion. PN in EGPA frequently displays with symmetrical multiple peripheral neuropathy in China. Positive ANCA, arthritis or joint pain, and higher BVAS are the independent associated factors of PN in EGPA. Glucocorticoids with immunosuppressants are vital therapeutic strategy.

scholarly journals Peripheral Neuropathy in ANCA Vasculitis

2021 ◽  
Author(s):  
Mouna Snoussi ◽  
Faten Frikha ◽  
Zouhir Bahloul
Keyword(s):  
Peripheral Neuropathy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Vasculitis ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Antineutrophil cytoplasmic antibodies (ANCA)-associated diseases are necrotizing systemic vasculitides that affect small blood vessels (arterioles, capillaries and venules). This entity represents three main systemic vasculitides: granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss’ syndrome). Their clinical manifestations are polymorphous, being the most frequent respiratory, oto-laryngo-pharyngeal and renal involvement. Peripheral neuropathy (PN) is reported in almost 50% of the patients. The aim of this chapter is to discuss the prevalence, clinical presentation, treatment and prognosis of PN in ANCA-associated vasculitis.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis Presenting with Myocarditis as an Initial Symptom: A Case Report and Review of the Literature

2021 ◽  
pp. 329-333
Author(s):  
Kanako Kurihara ◽  
Jun Tsugawa ◽  
Shinji Ouma ◽  
Toshiyasu Ogata ◽  
Mikiko Aoki ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Nerve Biopsy ◽  
Pulse Therapy ◽  
Sural Nerve Biopsy ◽  
Lower Limbs ◽  
Rapid Progression ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Initial Symptom ◽  
Steroid Pulse ◽  
Eosinophilic Granulomatosis

A 66-year-old woman with a history of bronchial asthma had shortness of breath and fatigue upon mild exercise. She was diagnosed as congestive heart failure. A blood test showed eosinophilia without the presence of anti-neutrophil cytoplasmic antibody (ANCA), and a myocardial biopsy specimen revealed eosinophilic infiltration in the myocardium. Eosinophilia was improved when she was administered short-term methylprednisolone. After that, she had numbness and pain in her lower limbs with re-elevation of eosinophils. She had dysesthesia and hypalgesia in the distal part of the limbs. Sural nerve biopsy revealed axonal degeneration and thickness of the arterial wall, indicating a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Two courses of steroid pulse therapy were performed, resulting in marked improvement of her sensory symptoms. ANCA-negative EGPA might be associated with myocarditis and peripheral neuropathy. A sufficient immunotherapy should have been considered to prevent rapid progression.

scholarly journals Eosinophilic Cholecystitis Associated with Eosinophilic Granulomatosis with Polyangiitis

2020 ◽  
Vol 14 (3) ◽  
pp. 668-674
Author(s):  
Hiroyuki Ito ◽  
Yusuke Mishima ◽  
Tsubomi Cho ◽  
Naoki Ogiwara ◽  
Yoshimasa Shinma ◽  
...  
Keyword(s):  
Cerebral Hemorrhage ◽  
Granulomatosis With Polyangiitis ◽  
Epigastric Pain ◽  
White Blood Cells ◽  
Gallbladder Wall ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Test Results ◽  
Laboratory Investigations ◽  
Eosinophilic Granulomatosis

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.

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

RMD Open ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. e000905 ◽  
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.

scholarly journals Eosinophilic granulomatosis with polyangiitis in a Nigerian woman

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.

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

2019 ◽  
Vol 12 (5) ◽  
pp. e225947 ◽  
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.

scholarly journals Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Suying Liu ◽  
Ling Guo ◽  
Xiaoyuan Fan ◽  
Zhaocui Zhang ◽  
Jiaxin Zhou ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Features ◽  
Associated Factors ◽  
Granulomatosis With Polyangiitis ◽  
Disease Duration ◽  
Gastrointestinal Perforation ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Cns Involvement ◽  
Eosinophilic Granulomatosis

Abstract Background Central nervous system (CNS) involvement is extremely rare in eosinophilic granulomatosis with polyangiitis (EGPA), but is associated with a poor prognosis in the five-factor score. This study aims to elucidate the clinical features and independently associated factors of EGPA with CNS involvement. Results CNS involvement was observed in 17.3% (19/110) of EGPA patients from Peking Union Medical College Hospital between 2007 and 2019. We retrospectively reviewed their clinical data and analyzed the independently associated factors. Their mean age was 51.7 ± 11.56 years with no male/female predominance. Ischemic lesions were the most common manifestations, accounting for 63.2% of the 19 cases, followed by posterior reversible encephalopathy syndrome (36.8%), spinal cord involvement (15.8%), medulla oblongata involvement (15.8%), and intracranial hemorrhages (15.8%). Compared to the control group, patients with CNS involvement were of older age (51.7 ± 11.56 vs. 43.7 ± 13.78 years, p = 0.019) and had a higher ratio in the digestive tract involvement (52.6% vs. 28.6%, p = 0.042). Further multivariate analysis revealed that age, disease duration, and fever were the potential independent risk factors for CNS involvement of EGPA. Glucocorticoids combined with cyclophosphamide were the strategic therapy (94.7%). Intrathecal injections of dexamethasone and methotrexate were administered to 21.1% of the patients. Although seven patients relapsed during glucocorticoid reduction, seventeen patients finally achieved clinical remission. One patient died of acute intracerebral hemorrhage within one month, and another died of gastrointestinal perforation. Outcomes and cumulative survival show no significant differences between the two groups. Conclusions CNS involvement is uncommon in EGPA with various manifestations, and ischemic lesions are the most frequent. Age, disease duration, and fever are independent factors associated with CNS involvement in EGPA. The therapy of glucocorticoids combined with cyclophosphamide and intrathecal injections yields favorable responses. Acute intracranial hemorrhage and gastrointestinal perforation may be the principal causes of death.

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