scholarly journals Programmed cell death-1 blockade in kidney carcinoma may induce eosinophilic granulomatosis with polyangiitis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Masanori Harada ◽  
Hyogo Naoi ◽  
Kazuyo Yasuda ◽  
Yutaro Ito ◽  
Namio Kagoo ◽  
...  
Keyword(s):  
Cell Death ◽  
Programmed Cell Death ◽  
Bronchial Asthma ◽  
Nerve Conduction ◽  
Granulomatosis With Polyangiitis ◽  
Allergic Inflammation ◽  
Eosinophil Infiltration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Kidney Carcinoma ◽  
Eosinophilic Granulomatosis

Abstract Background Immune checkpoint inhibitors have potential applications in treating various cancers but are associated with immune-related adverse events, such as inflammation, in a wide range of organs; however, allergic inflammation caused by these agents has not been extensively studied. Case presentation A 65-year-old man was diagnosed with a kidney neuroendocrine carcinoma. Three months after kidney resection surgery, the tumor cells had metastasized to his liver and lymph nodes. Subsequently, the patient started chemotherapy; however, regardless of treatment, the tumor grew, and the patient experienced a series of adverse effects, such as taste disorder, anorexia, and general fatigue. Finally, he was administered a programmed cell death (PD)-1 inhibitor, nivolumab (biweekly, toal 200 mg/body), which was effective against kidney carcinoma. However, the patient had a bronchial asthma attack at 22 cycles of nivolumab treatment and chest computed tomography (CT) revealed an abnormal bilateral shadow after 37 cycles of nivolumab treatment. Bronchoscopy findings revealed eosinophil infiltration in the lungs along with severe alveolar hemorrhage. Paranasal sinus CT scanning indicated sinusitis and nerve conduction analysis indicated a decrease in his right ulnar nerve conduction velocity. Based on these findings, the patient was diagnosed with eosinophilic granulomatosis with polyangiitis; he was treated with prednisolone, which alleviated his bronchial asthma. To restart nivolumab treatment, the dose of prednisolone was gradually tapered, and the patient was administered a monthly dose of mepolizumab and biweekly dose of nivolumab. To date, there have been no bronchial attacks or CT scan abnormalities upon follow up. Conclusions We present a rare case in which a patient with cancer was diagnosed with eosinophilic granulomatosis with polyangiitis following treatment with a PD-1 inhibitor. Blockade of PD-1 and the programmed cell death ligand (PD-L) 1/PD-1 and PD-L2/PD-1 signaling cascade may cause allergic inflammation. Further studies are needed to identify the specific mechanisms underlying allergic inflammation after PD-1 blockade.

scholarly journals Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous

2020 ◽  
Vol 22 (2) ◽  
pp. 383-392
Author(s):  
V. N. Marchenko ◽  
E. A. Bruchkus ◽  
A. A. Lebedeva ◽  
D. A. Davydov ◽  
N. L. Shaporova ◽  
...  
Keyword(s):  
Bronchial Asthma ◽  
Granulomatosis With Polyangiitis ◽  
Smoking History ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Occupational Hazards ◽  
Clinical Pattern ◽  
Pulmonary Infiltrates ◽  
Eosinophilic Granulomatosis ◽  
Necrotising Vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg—Strauss syndrome, represents a rare form of ANCA-associated necrotising vasculitis which affects small vessels. This disease is characterized by typical combination of immunological disturbances, hypereosinophilia, severe bronchial asthma, transient pulmonary infiltrates, and kidney injury which is less frequent than in other forms of necrotising vasculitis.Verification of the diagnosis is often hampered by comorbidities, such as chronic obstructive pulmonary disease (COPD) in the patients with long-term smoking history and occupational hazards.In this article, we report a clinical case of EGPA in elderly patient with preceding COPD which caused diagnostic difficulties for this eosinophilic syndrome. Clinical pattern at the beginning of disease was presented by moderate inspiratory dyspnea and cough with small amounts of mucus sputum, which appeared after longterm exposure to chlorine-containing substances. COPD diagnosis in this patient was based on clinical pattern, long smoking experience, and occupational hazards. However, persistence of the symptoms during the ongoing therapy, as well as multidirectional dynamics of transient pulmonary infiltrates found on repeated CT-scans, prompted us to intensify diagnostic search for a systemic disease. Clinical, laboratory and instrumental signs of bronchial asthma were revealed, as well as hypereosinophilia and sensory polyneuropathy, which, if combined with CT-scan data, allowed us to prove the EGPA diagnosis.This case shows that, despite great value of immune diagnostics, with negative blood tests for ANCA, it is necessary to detect mutually complicating comorbid pathology. EGPA was considered the basic diagnosis, and COPD as accompanying disorder, taking into account such reasons as an unfavorable prognosis for EGPA and the need for long-term chemotherapy with systemic corticosteroids and monoclonal antibodies. ANCA-negative testing in the patient, absence of severe kidney and skin lesions allows to suggest better clinical prognosis in this patient.

scholarly journals Multiple cerebral infarction diagnosed as Eosinophilic Granulomatosis with Polyangiitis by autopsy

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Kenichiro Hira ◽  
Hideki Shimura ◽  
Riyu Kamata ◽  
Masashi Takanashi ◽  
Akane Hashizume ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Brain Infarction ◽  
Eosinophil Infiltration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Consciousness Disturbance ◽  
The Right ◽  
The Brain ◽  
Eosinophilic Granulomatosis

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism. Case presentation A 53-year-old man presenting with abulia (consciousness disturbance) and left upper limb paralysis was admitted to our hospital. His case was complicated by penetrating branches, small vessel infarcts, and endocardial thrombosis in the right and left ventricle. Cardiomyopathy was also observed. Sixteen days after admission, the patient died from intracranial hemorrhage. Brain autopsy revealed two major findings: 1) large hemorrhagic infarction caused by cardiac embolism; and 2) granuloma and eosinophil infiltration. Vasculitis was accompanied by eosinophil infiltration in the cortical blood vessels and granuloma. Conclusions In this case study, we report autopsy findings of brain infarction in a patient with EGPA and endocardial thrombosis. The brain infarction was caused by the cardiac embolisms and vasculitis.

scholarly journals AB0394 CLINICAL CHARACTERISTICS OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: A SINGLE-CENTER RETROSPECTIVE ANALYSIS ON 52 CASES OF CHINESE PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1225.1-1225
Author(s):  
L. F. Chen ◽  
Y. Mo ◽  
Q. H. Li ◽  
D. H. Zheng ◽  
L. Dai
Keyword(s):  
Paranasal Sinuses ◽  
Clinical Characteristics ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Disease Process ◽  
Chinese Patients ◽  
Eosinophil Infiltration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis

Background:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and heterogeneous systemic vasculitis. Different patients or the same patient in different stages show different manifestations, which may lead to misdiagnosis and delay treatment.Objectives:To analyze the clinical characteristics in Chinese patients with EGPA.Methods:EGPA patients who fulfilled the 1990 ACR classification criteria were included between December 2003 and April 2020. The demographic and clinical characteristics were collected and analyzed retrospectively.Results:There were 52 EGPA patients recruited, 34 (65.4%) patients were males and the median age at onset was 47(38~55) years. The median duration from disease onset to diagnosis was 30(4~96) months. For initial symptoms, respiratory manifestations (61.5%) were the most common, including 42.3% patients beginning with asthma, followed by 21.2% with nose/paranasal sinuses manifestations. Respiratory medicine (53.8%) were the most common department at first visit, followed by rheumatology medicine (11.5%, Figure 1A). There were 44.2% EGPA patients definitely diagnosed at the department of rheumatology or after consultation by rheumatologists.During the whole disease process, the most common clinical manifestations were asthma (88.5%), then nose/ paranasal sinuses (84.6%), pulmonary (76.9%) and nervous system (61.5%) manifestations, followed by constitutional symptom (44.2%), heart (36.5%) and skin (23.1%) involvement. Only 9.6% patients had gastrointestinal tract involvement and 3.8% had renal involvement (Figure 1B).There were 46 (88.5%) patients showing the ratio of peripheral blood eosinophils >10% at diagnosis. Among the rest 6 patients, 3 had higher eosinophil ratio before diagnosis, while the other 3 patients had been treated with glucocorticoid before diagnosis, of whom 2 patients showed pathological eosinophil infiltration in lung or paranasal sinuses mucosa, respectively. There were 8(15.4%) patients with positive ANCA. Compared with EGPA patients with negative ANCA, they had lower incidence of asthma (62.5% vs. 93.2%), but higher incidence of constitutional symptoms (87.5% vs. 36.4%), arthralgia (50.0% vs. 6.8%) and renal involvement (25.0% vs. 0.0%), higher peripheral eosinophil count [2.06(0.80~4.51) ×109/L vs. 1.81(0.93~3.32) ×109/L], ESR [20(7~77) mm/h vs. 18(9~42) mm/h] and CRP [18.5(3.2~65.9) mg/L vs. 3.3(3.2~13.0) mg/L], higher Birmingham vasculitis activity score [17(10~22) vs. 13(9~15)] and vasculitis damage index [3(1~3) vs. 2(1~2), all P<0.05]. There were 21.2% EGPA patients showing poor prognostic factors according to five-factor score (FFS) and 34.6% patients according to the revised FFS.Conclusion:EGPA patients may have no asthma especially those with positive ANCA. Multi-disciplinary collaboration especially based on rheumatologists and pulmonologists should be emphasized for early identification and prompt treatment.Figure 1.Department at first visit (A) and clinical manifestations during the whole disease process of 52 Chinese EGPA patients(B).Disclosure of Interests:None declared

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.

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