A043 ORAL CORTICOSTEROID–SPARING EFFECT OF MEPOLIZUMAB IN PATIENTS WITH EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

2021 ◽  
Vol 127 (5) ◽  
pp. S7
Author(s):  
M. Wechsler ◽  
D. Jayne ◽  
B. Terrier ◽  
B. Hellmich ◽  
J. Bentley ◽  
...  
2020 ◽  
Vol 6 (1) ◽  
pp. 00311-2019 ◽  
Author(s):  
Brian D. Kent ◽  
Grainne d'Ancona ◽  
Mariana Fernandes ◽  
Linda Green ◽  
Cris Roxas ◽  
...  

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 ◽  
pp. 329-333
Author(s):  
Kanako Kurihara ◽  
Jun Tsugawa ◽  
Shinji Ouma ◽  
Toshiyasu Ogata ◽  
Mikiko Aoki ◽  
...  

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.


2020 ◽  
Vol 14 (3) ◽  
pp. 668-674
Author(s):  
Hiroyuki Ito ◽  
Yusuke Mishima ◽  
Tsubomi Cho ◽  
Naoki Ogiwara ◽  
Yoshimasa Shinma ◽  
...  

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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