scholarly journals Pathological findings of hypertrophic pachymeningitis associated with eosinophilic granulomatosis with polyangiitis

2021 ◽  
Vol 14 (9) ◽  
pp. e243395
Author(s):  
Wataru Shiraishi ◽  
Yoshitaka Tsujimoto ◽  
Tomoko Shiraishi
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Granulomatosis With Polyangiitis ◽  
Central Nervous System Involvement ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Pathological Findings ◽  
Hypertrophic Pachymeningitis ◽  
First Case ◽  
Eosinophilic Granulomatosis

The most common neurological manifestation of eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is mononeuritis multiplex caused by small-vessel vasculitis. In contrast, central nervous system involvement is rare. Among EGPA-associated central nervous system disorders, there are only a few reported cases of hypertrophic pachymeningitis (HP). Here, we report a patient with EGPA with headache and ophthalmoplegia who presented with HP and had a dural biopsy. The biopsy specimen showed lymphocytic inflammatory cell infiltration without EGPA-specific findings, that is, eosinophilic infiltration, granuloma or angiitis. To the best of our knowledge, there are no previous reports of EGPA-associated HP pathology. Here, we report the first case presentation of a patient with EGPA-associated HP with pathological findings.

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.

scholarly journals Rare case of recurrent stroke in a patient with eosinophilic granulomatosis with polyangiitis: a case report

2021 ◽  
Vol 57 (1) ◽  
Author(s):  
Mirjana Arsenijević ◽  
Nikola Ivančević ◽  
Dejana Jovanović ◽  
Milan Radović ◽  
Ivana Berisavac
Keyword(s):  
Risk Factors ◽  
Central Nervous System ◽  
Nervous System ◽  
Ischemic Stroke ◽  
Granulomatosis With Polyangiitis ◽  
Oral Corticosteroid ◽  
Recurrent Stroke ◽  
Cardiac Risk ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis

Abstract Background Central nervous system involvement is rarely described in eosinophilic granulomatosis with polyangiitis (EGPA) and occurs in 5–9% of patients. Among central nervous system manifestations, cerebral infarctions are the most common. To the best of our knowledge, a recurrent stroke in patients with EGPA without cardiac risk factors during maintenance therapy so far has not been described. Case presentation A previously healthy 57-year-old female during the course of 1 year developed asthma, sinusitis, polyneuropathy, muscle weakness, and rash followed by fatigue, myalgia, arthralgia, and fever. After an initial diagnostic evaluation, elevated values of eosinophils, liver enzymes, creatine kinase, lactate dehydrogenase, and inflammatory markers (sedimentation rate and C-reactive protein) were found, and renal impairment was detected. On the third day of hospitalization, she developed left-sided hemiparesis due to an ischemic stroke in the right basal ganglia. She has been diagnosed with EGPA, and oral corticosteroid, immunosuppressive, and antiplatelet therapy were applied. Despite potent treatment and initial recovery, a few weeks later, she developed recurrent ischemic stroke in the left hemisphere and pulmonary embolism as rare and potentially severe complications of EGPA. Assuming that complete disease remission had not been established previously, oral prednisone was initially substituted with intravenous methylprednisolone pulses. During follow-up, immunosuppressive therapy was slowly discontinued, oral corticosteroid therapy was reduced to a maintenance dose, and thromboembolic events were well controlled by oral anticoagulant therapy. Conclusion Anticoagulant therapy, in addition to immunosuppressive maintenance therapy, should be considered in any EGPA patient who has had an ischemic stroke even without cardiac risk factors.

Rituximab and Dupilumab Improve Eosinophilic Granulomatosis With Polyangiitis With Multiple Pulmonary Thrombi

2021 ◽  
Author(s):  
Sei Adachi ◽  
Chiyako Oshikata ◽  
Takeshi Kaneko ◽  
Naomi Tsurikisawa
Keyword(s):  
Heart Failure ◽  
Granulomatosis With Polyangiitis ◽  
Disease Onset ◽  
Gastrointestinal Symptoms ◽  
Patient Treatment ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Oxygen Inhalation ◽  
First Case ◽  
Venous Thromboembolic Events ◽  
Eosinophilic Granulomatosis

Abstract BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is characterized by a necrotizing vasculitis with tissue and peripheral blood eosinophilia affecting small and medium-sized arteries, capillaries, and veins. Venous thromboembolic events have occurred in 19 of 232 (8.2%) patients with EGPA. However, there are only a few reported cases of EGPA complicated by pulmonary embolism or infarction. Case presentationWe report the case of a 43-year-old woman with eosinophilic granulomatosis with polyangiitis patient with acute respiratory and heart failure due to bilateral pulmonary artery thrombosis and left femoral vein thrombosis in addition to cardiac involvement as myocarditis, pericardial effusion, and diastolic dysfunction, gastrointestinal symptoms and peripheral neuropathy 12 years after disease onset. The condition was refractory to treatment with systemic corticosteroids, intravenous cyclophosphamide, and mepolizumab, but the acute cardiac failure associated with the thrombosis, cardiac and gastrointestinal symptoms, and multiple polyneuropathy improved after a switch to rituximab. But her heart failure did not improve sufficiently, she continued to need oxygen inhalation at 1 L/min and asthma exacerbations occurred. We changed the patient’s treatment with mepolizumab to dupilumab. Not only did she have no asthma attacks after switching to dupilumab, but also her vasculitis symptoms improved. Oxygen therapy was discontinued as heart failure improved five months after starting the dupilumab. ConclusionsThis may be the first case report of the successful treatment of pulmonary thromboembolism associated with EGPA by rituximab. In addition, in this patient, treatment with dupilumab was effective not only for the asthma symptoms but also for the symptoms of vasculitis.

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.

Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii84-iii94 ◽  
Author(s):  
Giorgio Trivioli ◽  
Benjamin Terrier ◽  
Augusto Vaglio
Keyword(s):  
B Cells ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Presentation ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Mononeuritis Multiplex ◽  
Persistent Symptoms ◽  
Tissue Eosinophilia ◽  
Eosinophilic Granulomatosis

Abstract Eosinophilic granulomatosis with polyangiitis is characterized by asthma, blood and tissue eosinophilia and small-vessel vasculitis. The clinical presentation is variable, but two main clinic-pathologic subsets can be distinguished: one hallmarked by positive ANCA and predominant ‘vasculitic’ manifestations (e.g. glomerulonephritis, purpura and mononeuritis multiplex) and the other by negative ANCA and prominent ‘eosinophilic’ manifestations (e.g. lung infiltrates and cardiomyopathy). The pathogenesis is not fully understood but probably results from the interplay between T and B cells and eosinophils. Eosinophilic granulomatosis with polyangiitis must be differentiated from several conditions, including hypereosinophilic syndromes and other small-vessel vasculitides. The overall survival is good; however, patients frequently relapse and have persistent symptoms. The recently developed monoclonal antibodies targeting B cells and eosinophilopoietic cytokines such as IL-5 are emerging as valid alternatives to conventional immunosuppressive therapies. In this review, we discuss the essential features of eosinophilic granulomatosis with polyangiitis, with particular respect to the most relevant issues concerning clinical presentation and management.

P0373MEPOLIZUMAB THERAPY FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA) - ONE YEAR FOLLOW-UP STUDY USING ANTI-IL5 AS A STEROID SPARING THERAPEUTIC APPROACH

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Allyson Egan ◽  
Pasupathy Sivasothy ◽  
Robin Gore ◽  
Marcos MartinezDel-Pero ◽  
Lisa Willcocks ◽  
...  
Keyword(s):  
Adjuvant Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Care Pathway ◽  
Response To Therapy ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Positive Serology ◽  
Eosinophilic Asthma ◽  
Asthma Control Questionnaire ◽  
Eosinophilic Granulomatosis

Abstract Background and Aims EGPA is a small vessel vasculitis characterised by the presence of tissue eosinophilia, necrotising vasculitis and granulomatous inflammation1. Typically, a prodromal asthmatic phase, leads to an eosinophilic stage, which can evolve to include the presence of vasculitis with renal manifestations. In the recent randomised, placebo-controlled MIRRA trial for relapsing and refractory EGPA, adjuvant therapy with anti-IL5 mAB Mepolizumab [MEPO] at 300mg s/c monthly, accrued longer times in remission, reduced steroid exposure and reduced relapse rates2. The aim of our study was to analyse the response and outcome for EGPA patients who received 100mg s/c of MEPO monthly for a minimum of 52 weeks, with particular focus on the steroid minimisation benefits. Method This retrospective, descriptive study analysed 13 patients with EGPA, who received 100mg s/m monthly MEPO therapy under the eosinophilic asthma care-pathway. Time points of assessment included MEPO commencement [M0] and 12 [M12] months. Results One patient had MEPO switched to Rituximab to treat both EGPA and new onset rheumatoid arthritis Conclusion The relapsing nature of EGPA places a potential dependency of therapy on steroids for asthmatic and vasculitic flares. This underscores the importance of targeted pathway specific biologic therapy to minimise steroid exposure, prevent tissue damage and ensure early response to therapy. This study demonstrates that anti-IL5 serves as a favourable model with steroid minimisation, improvement in asthma control questionnaire, reduction in BVAS and eosinophil counts at the 100mg s/c dosage. ANCA positive serology normalised in all four patients, independent of subtype. Well tolerated, it demonstrated considerable clinical benefit, with 12 patients [92.3%] continuing anti-IL5 therapy beyond 12 months. Adjuvant therapy with conventional immunosuppressants was well tolerated and renal function was preserved. ADDIN Mendeley Bibliography CSL_BIBLIOGRAPHY 1. J.C.Jenette, et al Revised International Chapel Hil Consensus Conference Nomenclature of Vasculitides. 65, 1–11 (2013). 2. Wechsler, M. E. et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N. Engl. J. Med. 376, 1921–1932 (2017).

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