eosinophilic granulomatosis with polyangiitis ( egpa ) is a rare systemic small - vessel necrotizing vasculitis . its most frequent manifestations are late - onset asthma , chronic rhinitis , and sinus polyposis associated with eosinophilia . cardiac manifestations are very diverse but coronary vasospasm is uncommon . here , we report a case of an acute coronary vasospasm in a patient with egpa after corticosteroids withdrawal and nonsteroidal antiinflammatory drug ( nsaid ) introduction . few coronary vasospasms have been reported in patients with symptomatic egpa during both active and passive phases of the disease . to our knowledge , this is the first case describing acute coronary vasospasm leading to cardiac arrest during corticosteroid interruption and following nsaid administration in egpa . a 30-year - old man without significant medical history presented with a 5-month history of recurrent abdominal pain and dyspnea . the patient used nsaid occasionally in the early symptom phase as pain killers without adverse effect . from april to august 2013 , he suffered from intense crisis of acute abdominal pain localized on the upper and lower left quadrants , followed by hypoxemic acute dyspnea with cough and sometimes wheezing . ct - scan revealed peribronchial infiltrates and submucosal edema of the esophagus and the stomach with a small peritoneal effusion . the recurrence of abdominal pain , mucosal edema , and atypical dyspnea with laryngeal component suggested a diagnosis of nonhistaminergic angioedema . oral tranexamic acid and prophylactic treatment with human c1-esterase inhibitor twice a week was initiated as it seemed to be effective on abdominal symptoms . assay of kinin metabolism was normal thus dismissing the diagnosis of bradykinin - mediated angioedema . asthma was diagnosed as the patient sometimes experienced wheezing and inhaled salbutamol was slightly effective on symptoms . oral corticosteroid therapy was initiated as the diagnosis of vasculitis was suspected . on september 2013 one hour after nsaid ( ketoprofen ) administration , he presented with angina pectoris followed by ventricular fibrillation and cardiac arrest . electrocardiogram ( ecg ) displayed significant antero - septo - lateral st elevation with an inferior mirror image ( fig . coronary angiography revealed a tight spasm of the anterior interventricular branch of the left anterior descending artery and of the right coronary artery ( fig . nine days after cardiac arrest , cardiac mri disclosed a small - scale anteromedial cardiac infarction without any ventricular dysfunction or without vasculitis , myocarditis , and pericarditis . repetitive anti - neutrophil cytoplasmic antibodies ( anca ) measurements were negative and complement assay was normal . serum tryptase levels and screening for parasites were normal . neither fip1l1-pdgfra myeloid mutant nor lymphoid clone was found . histological analysis of esophagus , stomach , colon tissue , and temporal artery did not show evidence of vasculitis . the patient met 4 of the 6 american college of rheumatology diagnostic criteria required for egpa : asthma , parasinusal abnormalities , neuropathy , extravascular eosinophils . blood eosinophilia could not be taken in consideration as it was below the threshold of 10% of total white blood cell count . high dose intravenous corticosteroids ( 1  mg / kg / day ) and immunosuppressive therapy by intravenous cyclophosphamide ( 600  mg / m monthly during 3 months , then 700  mg / m ) were initiated . after 6 months , cyclophosphamide was switched to azathioprine ( 2.5  mg / kg ) . daily oral prednisone ( 10  mg ) and azathioprine therapy was pursued 30 months without relapse . typical st elevation in leads avl , i , v1 , v2 , v5 , v6 and mirror st depression in leads ii , iii , avf . . very tight spasms of the left anterior descending artery ( 7090% , 1020  mm length ) ( a ) and right coronary artery ( 50% , 1020  mm length ) with a distal spasm ( b ) are observed ( showed with arrows ) . both spasms are progressively reversed after intracoronary injection of trinitrine ( from left to right ) . existence of anca - negative egpa hypothesizes the role of eosinophilic infiltrates in some organ - specific symptoms , such as cardiac , gastrointestinal , and pulmonary manifestations , associated with a low frequency of vasculitis histology as compared to anca positive patients . previous episode of left trigeminal neuralgia could have been the first manifestation of egpa in our patient . to date eosinophilic infiltration of the arterial wall is suspected to drive a segmental vasoconstriction through the release of chemokines and eicosanoid inflammatory mediators . human coronary arteries express several subtypes of leukotriene receptors , mostly blt1 which binds leukotriene b4 ( ltb4 ) but also cysteinyl leukotriene ( cyslt ) receptor 1 . cyslt receptor 1 and blt1 are upregulated in smooth - muscle cell by proinflammatory stimuli diseases . in a vasculitis like egpa , we could consider cyslt and leukotriene b4 ( ltb4 ) to be responsible of vessel smooth muscle cells constriction through eosinophil chemoattraction and local inflammation . besides , local eosinophil accumulation has been reported to be enhanced with leukotriene through paracrine activation . thus , nsaid might exacerbate this segmental eosinophilic - mediated vasoconstriction process . nevertheless they decrease the synthesis of prostaglandin e2 which inhibits the production and the release of cyslt and ltb4 by eosinophils and mast cells . by analogy , in aspirin - exacerbated respiratory disease , basal low expression level of cox-2 make subjects very sensitive to non selective cox inhibitors . indeed , the removal of constitutive cox-1 activity by drugs permits eosinophil activation which leads to bronchospasm . in addition , discontinuation of systemic corticosteroids has been presumed to trigger egpa flare . in spite of nasal polyps and asthma , the diagnosis of aspirin - induced asthma kounis syndrome is defined as allergic reactions accompanied by cardiac symptomatology . in our case , the patient used ketoprofen and ibuprofen a few times as self - medication when he experienced acute trigeminal neuralgia pain at the beginning of the medical history but he stopped because of their ineffectiveness . he had never suffered from chest pain or dyspnea when he used nsaid which was not in favor of a kounis syndrome . moreover , he had not experienced other symptom than angina pectoris after ketoprofen administration before cardiac arrest . in summary moreover , corticosteroid compliance must be emphasized among patients who display egpa with high cardiac risk to prevent fatal issues .
abstracteosinophilic with polyangiitis ( egpa , formerly known as churg - strauss syndrome ) is a rare systemic disease characterized by a small - vessel necrotizing vasculitis . 
 cardiac manifestations are broad - ranging and are associated with a poor prognosis . 
 coronary vasospasm is uncommon.here , we report a case of an acute coronary vasospasm in a patient with egpa after corticosteroids withdrawal and nonsteroidal antiinflammatory drug ( nsaid ) introduction . 
 this patient was initially misdiagnosed as bradykinin - mediated angioedema . a 30-year - old man presented with recurrence of abdominal pain and acute dyspnea . 
 nsaid administration for pain during a flare was followed by coronary vasospasms leading to cardiac arrest . 
 corticosteroid treatment was recently interrupted by the patient.this case reports a rare cardiac complication of egpa . 
 nsaid might contribute to coronary vasospasm by eosinophilic degranulation in egpa . 
 moreover , corticosteroid compliance must be emphasized among patients who display egpa with high cardiac risk to prevent fatal issues .