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

Author(s):  
Mirjana Arsenijević ◽  
Nikola Ivančević ◽  
Dejana Jovanović ◽  
Milan Radović ◽  
Ivana Berisavac

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.

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Suying Liu ◽  
Ling Guo ◽  
Xiaoyuan Fan ◽  
Zhaocui Zhang ◽  
Jiaxin Zhou ◽  
...  

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.


2021 ◽  
Vol 14 (9) ◽  
pp. e243395
Author(s):  
Wataru Shiraishi ◽  
Yoshitaka Tsujimoto ◽  
Tomoko Shiraishi

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.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Federica Maritati ◽  
Francesco Peyronel ◽  
Paride Fenaroli ◽  
Giuseppe Daniele Benigno ◽  
Alessandra Palmisano ◽  
...  

Abstract Background and Aims Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome) is a rare systemic disease, included in the group of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), characterized by adult-onset asthma, blood and tissue eosinophilia with organ involvement, and small-vessel vasculitis. The pathogenesis of the disease is poorly understood, but it is likely that immunogenetic factors play a crucial role in its development. However, it has been suspected that the disease may be triggered by exogenous factors including environmental agents, infections, vaccinations, and drugs. Data about the association between individual, environmental and occupational risk factors and the development of EGPA are scarce. In this study we aimed to investigate the role of occupational agents (such as silica, farming, organic solvents and chemical agents) as well as individual agents (such as smoking habits) and their interactions, as potential risk factors for EGPA. Method The study has a case-control design. We enrolled 111 patients with EGPA and 333 healthy controls, matched for age, sex and geographical origin. Occupational history was obtained using a structured questionnaire administered by blinded specialists in occupational medicine. The exposures to non-occupational risk factors potentially predisposing to EGPA were assessed through the interview and through the examination of medical records. All exposures were considered until the time of EGPA diagnosis. Results At a multivariate logistic regression analysis, EGPA was associated with silica exposure (OR 2.26 [95% CI 1.10-4.62], p=0.026), farming (OR 2.10 [95% CI 1.19-3.73], p=0.011) and with the exposure to organic solvents (OR 2.20 [95% CI 1.14-4.2], p=0.018). There was a positive relationship between the duration of exposure to silica, chemical agents and the risk of developing EGPA (Figure 1). A multiplicative effect on risk was found for the co-exposure of silica and farming (OR 7.49 [95% CI 2.77-20.25], p<0.0001) (Figure 2). The exposure to tobacco smoke appeared protective against EGPA (OR 0.49 [95% CI 0.29-0.70], p<0.0001). Conclusion This is, to our knowledge, the first study investigating the role of environmental, occupational and individual risk factors in the development of EGPA, through the comparison of a group of EGPA patients with a group of healthy controls, matched for age, gender and geographical origin. Our results demonstrate that occupational factors may significantly contribute to the development of the disease. In particular, we found a significative association between exposure to silica, farming, organic solvents and EGPA. In addition, our findings reveal that tobacco smoking has a protective role against the disease. Further studies are necessary to investigate the biological effects of such risk factors on disease pathogenesis.


2020 ◽  
Vol 6 (1) ◽  
pp. 00311-2019 ◽  
Author(s):  
Brian D. Kent ◽  
Grainne d'Ancona ◽  
Mariana Fernandes ◽  
Linda Green ◽  
Cris Roxas ◽  
...  

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