P0280ENVIRONMENTAL AND OCCUPATIONAL EXPOSURES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS. A CASE-CONTROL STUDY

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.

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.


2017 ◽  
Vol 44 (8) ◽  
pp. 1206-1215 ◽  
Author(s):  
Naomi Tsurikisawa ◽  
Chiyako Oshikata ◽  
Arisa Kinoshita ◽  
Takahiro Tsuburai ◽  
Hiroshi Saito

Objective.We investigated the risk factors for relapse or prognosis of eosinophilic granulomatosis with polyangiitis (EGPA) in Japanese patients presenting to our hospital.Methods.From June 1999 through March 2015, we retrospectively recruited 121 patients with EGPA according to the American College of Rheumatology criteria. Frequent relapse was defined as disease occurrence at least once every 2 years after a period of initial remission. The study endpoint was the last examination performed. We used multiple logistic regression to analyze risk factors for relapse or survival in EGPA.Results.Gastrointestinal (GI) involvement with both abnormalities on endoscopy and biopsy (p < 0.01) and symptoms; myocardial involvement with both abnormalities on 1 or more cardiac investigations and symptoms (p < 0.01); and treatment at initial or maintenance with immunosuppressants (p < 0.01) or administration of intravenous immunoglobulin (IVIG; p < 0.01) were associated significantly more often with frequent relapse than with infrequent. Overall 5-, 10-, and 20-year survival rates were 91.1%, 83.7%, and 68.6%, respectively. Survival in EGPA was associated with age of onset < 65 years. Age at onset of EGPA was the only significant predictor of survival (p < 0.01). Myocardial or GI tract involvement did not affect mortality risk.Conclusion.Patients with myocardial or GI tract involvement had frequent relapses, but these conditions were not reflected in increased mortality. Treatment with immunosuppressants or IVIG in addition to corticosteroids might have improved the prognosis in Japanese patients with EGPA.


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.


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