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Cardiogenic shock as the first manifestation of large vessel vasculitis in a young patient: case report.

ABSTRACT:

Background

Cardiogenic shock secondary to coronary involvement in large vessel vasculitis (LVV) is an unsuspected finding, even more, when no other vascular territories are compromised and when it constitutes the initial clinical manifestation. This case report illustrates a case in which a complete diagnostic study uncovered this aetiology.

Case summary

A 33-year-old woman with cough and chest pain who was diagnosed with acute bronchitis returned with worsening dyspnoea, chest pain, and developed cardiogenic shock. The initial differential diagnoses included myocarditis and takotsubo cardiomyopathy (TCM) owing to a positive troponin I, and echocardiogram with left ventricular dilation, dyskinesia in mid-ventricular and apical segments, systolic dysfunction, and functional mitral regurgitation. A cardiac magnetic resonance showed contractility abnormalities resembling the pattern of TCM but lacked the characteristic myocardial oedema. Subsequently, a coronary angiography expected to result without obstructions showed a critical narrowing of the left main coronary artery. Surgical management consisted of a pericardium patch grafted in the stenotic ostium to restore adequate perfusion. The surgical specimens were sent to the pathology laboratory that reported findings compatible with LVV. Four days after the surgical intervention the patient was discharged alive with a complete recovery of left ventricular systolic function.

Discussion

Chest pain symptoms in a young woman, could be caused by multiple entities, and an ischaemic aetiology from a non-atherosclerotic origin should be kept in mind. A complete study with coronary angiography is crucial to rule out an ischaemic cause even in low-risk groups for atherosclerotic coronary heart disease.

SUBMITTER: Isaza N

PROVIDER: S-EPMC6176963 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:ObjectiveTo assess the relationship between measures of disease assessment in patients with large vessel vasculitis.MethodsPatients with giant cell arteritis (GCA) or Takayasu arteritis (TAK) were recruited into a prospective, observational cohort. Assessments within the following outcomes were independently recorded: 1) patient-reported outcomes (Multidimensional Fatigue Inventory, patient global assessment of disease activity [PtGA], Short Form 36 health survey [SF-36], Brief Illness Perception Questionnaire), 2) physician global assessment of disease activity (PhGA), 3) laboratory outcomes (C-reactive protein [CRP] level, erythrocyte sedimentation rate [ESR]), and 4) imaging outcomes (PETVAS, a qualitative score of vascular 18 F-fluorodeoxyglucose-positron emission tomography activity).ResultsAnalyses were performed on 112 patients (GCA = 56, TAK = 56), over 296 visits, with a median follow-up of 6 months. Correlation network analysis revealed assessment measures clustered independently by type of outcome. PhGA was centrally linked to all other outcome types, but correlations were modest (ρ = 0.12-0.32; P < 0.05). PETVAS, CRP level, and PtGA were independently associated with clinically active disease. All 4 patient-reported outcomes strongly correlated with each other (ρ = 0.35-0.60; P < 0.0001). Patient-reported outcomes were not correlated with PETVAS, and only PtGA correlated with CRP level (ρ = 0.16; P < 0.01). Patients whose clinical assessment changed from active disease to remission (n = 29) had a corresponding significant decrease in ESR, CRP level, and PETVAS at the remission visit. Patients whose clinical assessment changed from remission to active disease (n = 11) had a corresponding significant increase in CRP level and PtGA at the active visit.ConclusionMeasures of disease assessment in large vessel vasculitis consist of independent, yet complementary, outcomes, supporting the need to develop composite outcome measures or a standard set of measures covering multiple types of outcomes.