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Acute coronary vasospasm in a patient with eosinophilic granulomatosis with polyangiitis following NSAID administration

View Article: PubMed Central - PubMed

ABSTRACT

Eosinophilic 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.

No MeSH data available.


Related in: MedlinePlus

Emergency coronary arteriogram following sudden cardiac arrest. Very tight spasms of the left anterior descending artery (70–90%, 10–20 mm length) (A) and right coronary artery (50%, 10–20 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).
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Figure 2: Emergency coronary arteriogram following sudden cardiac arrest. Very tight spasms of the left anterior descending artery (70–90%, 10–20 mm length) (A) and right coronary artery (50%, 10–20 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).

Mentions: 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. Nasofibroscopy revealed no laryngeal cause but noticed nasal polyps. CT-scan revealed peribronchial infiltrates and submucosal edema of the esophagus and the stomach with a small peritoneal effusion. Moderate hypereosinophilia was found (1.29 × 109/L). Antihistaminic drugs were ineffective. The recurrence of abdominal pain, mucosal edema, and atypical dyspnea with laryngeal component suggested a diagnosis of nonhistaminergic angioedema. Quantitative and functional assay of C1-inhibitor and complement assay were normal. Thus, bradykinin-mediated angioedema was suspected. 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. Icatibant was administered during attacks as a rescue medication. C1-esterase inhibitor treatment was stopped 1 month later after another abdominal attack. Moreover, 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, the patient was admitted to the Emergency department with localized abdominal pain. Corticosteroid therapy was stopped 12 days before as the patient was out of medication. One hour after NSAID (ketoprofen) administration, he presented with angina pectoris followed by ventricular fibrillation and cardiac arrest. One defibrillation biphasic shock of 150 J enabled to restore sinusal cardiac activity. Active-life support lasted about one minute. Electrocardiogram (ECG) displayed significant antero-septo-lateral ST elevation with an inferior mirror image (Fig. 1). Troponin was initially elevated at 2.33 μg/L (N < 0.1 μg/L). Eosinophils were 1.34 × 109/L. Coronary angiography revealed a tight spasm of the anterior interventricular branch of the left anterior descending artery and of the right coronary artery (Fig. 2). Spasms were reversed after intracoronary injection of glycerin trinitrate. 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. Nasal mucosa biopsy of polyps revealed eosinophilic infiltrates. 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. An implantable cardioverter defibrillator was implanted 15 days after cardiac arrest. High dose intravenous corticosteroids (1 mg/kg/day) and immunosuppressive therapy by intravenous cyclophosphamide (600 mg/m2 monthly during 3 months, then 700 mg/m2) were initiated. After 6 months, cyclophosphamide was switched to azathioprine (2.5 mg/kg). General symptoms markedly improved. Daily oral prednisone (10 mg) and azathioprine therapy was pursued 30 months without relapse. The patient fully recovered neurologically after cardiac arrest. He was discharged 23 days after entering the hospital.


Acute coronary vasospasm in a patient with eosinophilic granulomatosis with polyangiitis following NSAID administration
Emergency coronary arteriogram following sudden cardiac arrest. Very tight spasms of the left anterior descending artery (70–90%, 10–20 mm length) (A) and right coronary artery (50%, 10–20 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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5134854&req=5

Figure 2: Emergency coronary arteriogram following sudden cardiac arrest. Very tight spasms of the left anterior descending artery (70–90%, 10–20 mm length) (A) and right coronary artery (50%, 10–20 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).
Mentions: 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. Nasofibroscopy revealed no laryngeal cause but noticed nasal polyps. CT-scan revealed peribronchial infiltrates and submucosal edema of the esophagus and the stomach with a small peritoneal effusion. Moderate hypereosinophilia was found (1.29 × 109/L). Antihistaminic drugs were ineffective. The recurrence of abdominal pain, mucosal edema, and atypical dyspnea with laryngeal component suggested a diagnosis of nonhistaminergic angioedema. Quantitative and functional assay of C1-inhibitor and complement assay were normal. Thus, bradykinin-mediated angioedema was suspected. 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. Icatibant was administered during attacks as a rescue medication. C1-esterase inhibitor treatment was stopped 1 month later after another abdominal attack. Moreover, 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, the patient was admitted to the Emergency department with localized abdominal pain. Corticosteroid therapy was stopped 12 days before as the patient was out of medication. One hour after NSAID (ketoprofen) administration, he presented with angina pectoris followed by ventricular fibrillation and cardiac arrest. One defibrillation biphasic shock of 150 J enabled to restore sinusal cardiac activity. Active-life support lasted about one minute. Electrocardiogram (ECG) displayed significant antero-septo-lateral ST elevation with an inferior mirror image (Fig. 1). Troponin was initially elevated at 2.33 μg/L (N < 0.1 μg/L). Eosinophils were 1.34 × 109/L. Coronary angiography revealed a tight spasm of the anterior interventricular branch of the left anterior descending artery and of the right coronary artery (Fig. 2). Spasms were reversed after intracoronary injection of glycerin trinitrate. 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. Nasal mucosa biopsy of polyps revealed eosinophilic infiltrates. 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. An implantable cardioverter defibrillator was implanted 15 days after cardiac arrest. High dose intravenous corticosteroids (1 mg/kg/day) and immunosuppressive therapy by intravenous cyclophosphamide (600 mg/m2 monthly during 3 months, then 700 mg/m2) were initiated. After 6 months, cyclophosphamide was switched to azathioprine (2.5 mg/kg). General symptoms markedly improved. Daily oral prednisone (10 mg) and azathioprine therapy was pursued 30 months without relapse. The patient fully recovered neurologically after cardiac arrest. He was discharged 23 days after entering the hospital.

View Article: PubMed Central - PubMed

ABSTRACT

Eosinophilic 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.

No MeSH data available.


Related in: MedlinePlus